A healthy nine-year-old Japanese male presented with a history of inferonasal conjunctival mass that was noted to increase in size in a few years' time. Initial examination revealed a 10-mm soft yellowish inferonasal conjunctival mass with a whitish surface. The rest of the examination findings were unremarkable. A computed tomographic scan was done and showed a conjunctival mass that was not connected posteriorly to the orbital fat but had the same density as the orbital fat. Complete surgical excision was performed, and pathologic evaluation of the excised specimen revealed a dermolipoma. The patient was subsequently followed up to monitor for any postoperative complications. Three months following the operation, there was no recurrence of the tumor, and the patient had an excellent cosmetic outcome. Dermolipoma is the second most common conjunctival mass in children and is the most common epibulbar choristoma . It is aThis study was conducted in accordance with the tenets of the Declaration of Helsinki and its later amendments. Written informed consent for the publication of identifiable face photos was obtained from the patient’s mother. A nine-year-old boy consulted with our service for the removal of a conjunctival mass. His parents noticed the mass a few years ago, and the lesion had gradually increased in size. The patient had no history of trauma. Past medical history and family history were also unremarkable.On initial consultation, his best-corrected visual acuity was 1.2 in both eyes. Refractive errors were insignificant. Intraocular pressures were 19 mmHg in the right eye and 17 mmHg in the left. Extraocular muscle motility was normal. Slit-lamp examination of the left eye revealed a 10-mm soft conjunctival mass in the inferonasal quadrant Figure . AlthougThe surgery was done under general anesthesia by one of the authors (YT). The mass was completely excised, and the conjunctival defect was closed using 6-0 polyglycolic acid sutures . Pathological examinations of the excised specimen showed mature fat cells and fibrous connective tissue covered by stratified squamous epithelium with goblet cells Figure . The finSince dermolipoma is typically found in the superotemporal quadrant, the clinician must have a high level of suspicion in order to diagnose rare cases presenting in an unusual anatomic location. The authors have found only three case reports on dermolipomas presenting inferonasally as protruding conjunctival masses, one of which was found to contain ectopic lacrimal gland tissue . AnotherDermolipomas in locations other than the superotemporal and inferonasal quadrants are also rare. A previous report showing 116 ocular dermoids demonstrated that 30% of dermolipomas were found in locations other than the temporal quadrant, but this report did not show further details . AnotherOrbital dermolipoma is usually present at birth but may not be visually detected until later in life -5. As thSmall and asymptomatic lesions are typically observed. Since the parent found the lesion cosmetically bothersome, surgical treatment was opted for. Dermolipoma may exhibit posterior extension into the orbit without connection to the intraconal orbital fat ,11, but The authors report a rare case of dermolipoma found in the inferonasal conjunctival fornix, which is an unusual location for this type of tumor. Dermolipoma must, therefore, be considered in a patient presenting with protruding conjunctival mass with a characteristic appearance, regardless of the anatomic location.


Introduction: Despite the improvement of early diagnosis methods for multiple pathological entities belonging to the digestive tract, bowel obstruction determined by multiple etiologies represents an important percentage of surgical emergencies. General data: Although sometimes obstructive episodes are possible in the early stages of colorectal cancer, the most commonly installed intestinal obstruction has the significance of an advanced evolutionary stage of neoplastic disease. Development of Obstructive Mechanism: The spontaneous evolution of colorectal cancer is always burdened by complications. The most common complication is low bowel obstruction, found in approximately 20% of the cases of colorectal cancer, and it can occur either relatively abruptly, or is preceded by initially discrete premonitory symptoms, non-specific (until advanced evolutionary stages) and generally neglected or incorrectly interpreted. Success in the complex treatment of a low neoplastic obstruction is conditioned by a complete diagnosis, adequate pre-operative preparation, a surgical act adapted to the case , and dynamic postoperative care. The moment of surgery should be chosen with great care and is the result of the experience of the anesthetic-surgical team. The operative act must be adapted to the case and has as its main objective the resolution of intestinal obstruction and only in a secondary way the resolution of the generating disease. Conclusions: The therapeutic measures adopted  must have a dynamic character in accordance with the particular situation of the patient. Except for certain or probably benign etiologies, the possibility of colorectal neoplasia should always be considered, in low obstructions, regardless of the patient’s age. Despite the improvement of early diagnosis methods for multiple pathological entities belonging to the digestive tract, bowel obstruction determined by multiple etiologies represents an important percentage of surgical emergencies, accounting for approximately 20–30% of the cases diagnosed with acute abdomen . EffortsThis research focuses on the determinism, the physiological mechanisms, and the treatment particularities of obstructive colorectal cancer, an entity representing 60–80% of low bowel obstructions ,4. Intestinal obstruction is commonly found in surgical services, as a stand-alone entity , or as an epiphenomenon of other medical or surgical conditions . Although the clinic is generally sufficient for positive and topographic diagnosis, the etiological and pathogenic details cannot be clearly outlined, losing their specificity due to an intricate clinic with a spectacular dynamic of the suggestive elements for certain generating causes. With the exception of obstructions with ischemic mechanism from the beginning , those of the simple obstructive type present a clinical, dynamic mosaic, which frequently fails the attempts of systematization, creating taxonomic controversies, but justifies hydro-electrolytic, acid-base and nutritional rebalancing measures and finally the surgical procedure .The contribution of paraclinical and laboratory investigations is extremely useful, but even in these conditions a lot of cases remain etiologically obscure ,6. CliniA serious clinical entity by itself, intestinal obstruction can also be complicated  which produces an exponential worsening generating mortality rates comparable to severe digestive bleeding, severe pancreatitis, or major sepsis .Distal bowel obstructions  have a simple obstruction (except volvulus) as their established mechanism. Symptoms are more indefinite, and the worsening of the general condition occurs more slowly. These so-called “advantages” are nullified by factors, such as age, etiology , and multiple complications (most commonly septic). The decompressing factor that the small intestine can have in distal obstructions can be canceled by a pressure-competent ileocecal valve, transforming the colon into a closed, under-pressure loop (double obturated) . MassiveIschemic-type rheologic changes have multiple pathogenies. The cumulative effect of colic parietal vessel elongation , the ischemia produced by direct tissue pressure , extensive hemorrhagic intraparietal changes , or hypovolemic parietal hypoxia contribute to the premature alteration of the mucous-epithelial barrier, and then to colic perforation . Massive fluid-electrolyte intersectoral redistributions, with the formation of the IIIrd surgical space, associated with hydro-electrolyte losses through vomiting, contribute to the establishment of the dysvolemic status , which in association with the installed toxic-septic status, represent a powerful pathogenic association .The first pathogen that occurs is the impairment of lumen freedom, with upstream storage of gas and stercoral content. Secondarily, enteral motility disorders are installed as a result of cholic distension and episodic appearance of hyperperistaltism for evacuation purposes (“fighting colic’’). The tertiary element that occurs is the modification of intestinal wall viability with the addition of infectious factor (tumor abscess) or juxtatumoral or diastatic colic perforations. Low digestive malignancy, along with progressive lumen obstruction, can precipitate the obstructive episode through other mechanisms: invagination of pediculate tumors, extrinsic parietal invasions, obstructive carcinomatosis, the association of ischemic colic sufferings, etc. From this perspective, mechanical intestinal occlusion presents an initiator of pathogenic mechanisms (the obstacle in the colic lumen) and a systemic resonator  that worsens itself by dysvolemia and sepsis .Although sometimes obstructive episodes are possible in the early stages of colorectal cancer, the most commonly installed intestinal obstruction has the significance of an advanced evolutionary stage of neoplastic disease. Malignant colorectal obstructions generally evolve with an afebrile state. “Warm” obstructions suggest the appearance of septic, ischemic or co-existence of multiple metastases with hyperpyrexia, accentuated in the context of intersectoral dehydration .A therapeutic attitude in confirmed or intuited non-ischemic cases begins with measures aimed at hydro-electrolyte, acid-base, metabolic rebalancing, and measures to release the intestinal territory proximal to the stenosis through sustained digestive aspiration  and retrograde rectocolic lavage. The rhythm and duration of these measures remain an equation with multiple unknowns and traps, but aim at reconfiguring the general state, correcting the installed imbalances , impregnating with antibiotic and possibly anticoagulant, cardiac tonic, etc. .Depending on the specific situation, these measures can be adopted regulated or ultra-quickly. In the case of a favorable response, this period can be extended, hoping for complete release, which would allow a safer and more comfortable surgical act, in elective conditions. Failure to respond requires emergency surgery. The surgical attitude cannot be standardized considering the multitude of factors involved, but it ranges from large-scale surgical interventions aimed at solving the generating injury (tumor) as well as the complication (obstruction), to minimal surgical gestures that can contribute to the resolution of the obstruction (cecostomy) ,16.Although there is no unity of opinion regarding surgical strategies depending on topography, for the right colon, the right hemicolectomy followed by ileo-transverse anastomosis maintains a leading position, while for tumors located under the splenic flexure of the colon, the interventions in two or three times with different types of stomas upstream are valid, practiced for reasons of safety or necessity.Quality pre-operative preparation, a well-conducted and adapted surgical act and meticulous postoperative care can bring good results with the resolution of the underlying disease (neoplasm) and its complication (obstruction). However, this morbid association represents a severe pathological circumstance, in front of which optimism must remain moderate and circumspect ,18.Although there are embryological, anatomical, histological, and functional differences between colic and rectal locations respectively, we note the existence of some common elements between the neoplasms of these segments.The middle portion of the primitive intestine (mesenteron) generates the upper structures of the digestive tract , but also cecum, appendix vermiform, ascending colon, and right half of the transverse colon. The metenteron (embryonic posterior intestine) will develop the distal half of the transverse colon, descendant, sigmoid, rectum and upper portion of the anal canal. Studies on the different embryological origins of malignant colorectal segments suggest possible connections between embryology and carcinogenesis, this area of research being of high interest and requiring further studies . The involvement of genetic factors with autosomal dominant transmission was confirmed with the identification of hereditary nonpolyposis neoplasm (Lynch Syndrome I and II) and adenomatous familial polyposis ,21.In addition, it is important to mention the adenoma-carcinoma sequence, which is defined by a set of recurrent driver mutations in a series of genes  that accumulate in the process of adenoma formation and progression to sporadic colorectal cancer .The entire colon and rectum above the pectinated line is lined with one layered columnar epithelium. Below this level, up to the Hilton’s white line, the rectal epithelium is a non-keratinized, pluristratified pavement. In the area of interference between the two territories, histologists describe a state of “cellular unrest”, which predisposes to phenomena of metaplasia and even malignant degeneration. Gland structures with different morphology and function can contribute to oncogenesis .The geographical distribution of colorectal cancer is uneven between different countries or continents. The migration of some population groups to regions with high incidence increases the frequency of this pathology, suggesting the influence of some environmental factors .The distribution of colorectal cancer between the two genders is relatively equal, with the prevalence on the right colon in women and on the left colon in men. The maximum incidence is recorded at 60–70 years of age, although it is more and more common at a young age . In the Inflammatory bowel diseases  and diffuse colic polyposis, register a significant percentage of malignant degeneration in the absence of treatment or in conditions of insufficient or incorrectly conducted treatment ,30. The The low intake of vegetables and cellulose fibers and the excess of animal fats, carbohydrates, and alcohol predisposes to the development of colorectal neoplasia .The excessive presence of secondary bile acids (deoxycholic and lithocholic) in the digestive tract promotes carcinogenesis. The effect seems mediated by the excessive fixation of dietary calcium, which causes punctual peeling of the cholic mucosa, favoring the appearance of metaplastic changes. Large bile discharges into the digestive tract post-cholecystectomy raised the suspicion that this surgery predisposes to colorectal cancer ,36.Obesity, sedentary lifestyle, smoking, diabetes, abdominal radiotherapy, racial factor, etc., are other factors that can leave their mark on colorectal carcinogenesis ,39,40,41Vitamins A, C, E, beta carotene, aspirin, and non-steroidal anti-inflammatory drugs seem to have a protective role, noting a higher incidence of colorectal neoplasm in those with vitamin deficiencies ,43.The spontaneous evolution of colorectal cancer is always burdened by complications. Their variety is very large. Among the local complications, the most common are: loco-regional invasion with possible inter-visceral or external fistulas, tumor infection with adjacent sclerolipomatous reaction or abscesses, peritonitis by evacuation of tumor abscesses or intestinal perforations, intestinal obstructions, lower digestive bleeding, etc. General complications are represented by anemia, paraneoplastic venous thrombosis, multiple metastases, hepatic abscesses with systemic sepsis, etc. ,45.The most common complication is low bowel obstruction, found in approximately 20% of the cases of colorectal cancer and can occur either relatively abruptly, or is preceded by initially discrete premonitory symptoms, non-specific (until advanced evolutionary stages), and generally neglected or incorrectly interpreted ,46.The common obstructive mechanism is simple obstruction and is specific to situations with preceding manifestations . For forms with sudden onset may occur intussusceptions , volvuluses of supratumoral mobile segments and the association of peritoneal carcinomatosis or enteral ischemic phenomena. Colic volvulus is favored by the increased weight of endoluminal content and hypermobility, with fixed points of the extremities as close as possible. In intestinal volvuluses, necrosis is not directly correlated with the number of rotations of the loop, but with the degree of “constriction of the affected mesenteries” ,47.Low bowel obstruction due to neoplastic cause  presents anatomical and functional characteristics important in stage, pathogenic, topographic, and etiological diagnosis, orienting therapeutic consequences.The simple obstructive mechanism is installed later in colic segments with large caliber. Subsequently, the ileocecal valve follows the segment with the largest diameter (the cecum and the ascending colon), which explains the lower frequency of right colon occlusions if the Bauhin valve is not interested. The diameter of the colic lumen decreases discreetly to the distal segments . Peritumoral sclerolipomatosis and anemia are more common in the right colon, contributing to the shaping of the characteristics specific to this level of tumor location ,49.2O, compared to the one in the small intestine where it is approximately 200–300 cm H2O) [The thickness of the colic wall has less importance regarding the frequency of colorectal neoplasms on certain topographies but has profound relevance in relation to retrograde diastatic perforations (Laplace’s law) . The lar cm H2O) ,51,52,53Although it enjoys two important arterial sources (upper mesenteric artery for the right colon and lower mesenteric artery for the left colon) and marginal arches Drummond and Riolan-Haller and inconstant central intermesenteric anastomosis Huard, the colon does not benefit through long and short vasa recta arteries of a generous arterial flow comparable to the upper segments of the tract digestive system . Mesenteric arteries are the preferred territories for the phenomenon of atheromatosis contributing to the progressive decrease of blood flow in irrigated territories. Elongation of vasa recta in the distended colic wall favors ischemia and parietal microthromboses, precipitating colic perforation. The closing of the venous network  is an additional factor in the irreversibility of parietal lesions ,55.The colorectal area stretches between the ileocecal valve and the anal sphincter. The latter is of limited importance in low intestinal obstruction, being most commonly located under the obstacle. The ileocecal valve is deeply involved in the pressure play and dynamics of the cholic stasis content in low intestinal obstruction. A “permissive” valve can favor the reflux of stercoral content towards the ileal and then jejunal territory, tempering for the moment the axial and lateral pressures from the obstructed colon. Its increased pressure competence can close the possibility of retrograde reflux, with the exponential increase of intracolic pressures.The functional sphincters of the colon  are limited areas with better represented circular musculature and may have a limited role in colon dynamics in the early stages of the obstruction evolution. It can be concluded that Laplace’s physical law may be influenced by cholic morphofunctional characteristics ,57.The mobility of colic segments can influence the dynamics of the obstructive process. If the fixed segments are the site of tumor development, they will suffer a simple process of obstruction, while for mobile segments other obstructive mechanisms may overlap  .Obstruction of the colic lumen is more common in ulcer-vegetative , exophytColic innervation is vegetative, receptor and effector. The Meissner submucous plexus and axons of neurons in the posterior root node of the spinal nerves T11-L1 are responsible for visceral sensitivity. The secretive and motor activities are modulated by the Auerbach myenteric plexus, unequally represented for the right colon (related to the upper mesenteric plexus) and the left colon (dependent on the lower mesenteric plexus). The poor representation of the myenteric plexus is associated with a diminished peristaltic accompanied by a background distension of the left colon (megadolichocolon), decompensated early in the obstacles of colorectal junction or below this level. Colic hyperdistension makes the action of pharmacological active factors  ineffective on tonus and peristalsis ,61.Other functional factors involved in the pathogenic mechanisms of low intestinal obstruction depend on the individual characteristics of each patient, or derive from anatomical considerations: cholic resorptive function, dramatically extracted dysmicrobisms ,63, nutrSuccess in the complex treatment of a low neoplastic obstruction is conditioned by a complete diagnosis, adequate pre-operative preparation, a surgical act adapted to the case  and dynamic postoperative care. Diagnostic errors consist both in overestimating the case , but especially in the negative . Etiological clarification is only possible sometimes, but this aspect is not a major drawback given the indication of surgical exploration, which will clarify this aspect as well.Preoperative management of patients diagnosed with bowel obstruction involves, even from the emergency room, the placement of a peripheric venous catheter and starting the infusion of crystalloid solutions such as saline or Ringer solution. Acid-base and electrolyte rebalancing is a priority, often requiring repeated assessments of serum ions and pH. Considering the potential for dehydration and the rapid evolution of the disease, a urinary catheter is necessary for monitoring diuresis. In patients with severe cardiac, renal, or pulmonary failure, monitoring of fluid and electrolyte rebalancing using central Swan-Ganz catheters may be helpful. Preoperative hematological imbalances such as severe anemic syndromes can be adjusted by blood transfusions and severe thrombocytopenia by administering platelet masses. In the case of coagulopathy  or in case of changes in coagulation parameters due to treatments for various cardiologic disorders, prompt administration of plasma or vitamin K is necessary. Antibiotherapy, thromboembolic prophylaxis, and treatment of associated diseases are equally important ,68,69,70The moment of surgery should be chosen with great care and is the result of the experience of the anesthetic-surgical team. The operative act must be adapted to the case and has as its main objective the resolution of intestinal obstruction and only in a secondary way the resolution of the generating disease. The resection of the involved colorectal segment is performed , respecting oncological principles. Afterward, anastomosis is taken into account when the proximal segment shows no structural changes. Otherwise, to avoid a digestive fistula, it is recommended to close the distal segment and perform an ostomy at the level of the proximal one .Often, the surgical procedure does not involve the removal of the obstructive process, but only the restoration of the digestive transit. This involves cases in which is intended to shorten the duration of the surgical intervention due to the patient’s comorbidities and the general condition at the time or procedures performed for palliative purposes, for advanced neoplasia. In the case of external digestive derivations without removal of the obstructive process, it is recommended to perform a continuous ostomy, in order to avoid a “closed loop” .“The patient with intestinal obstruction is in the situation of a rescued from drowning. This is not the case for a swimming lesson”—Wangensteen.The postoperative stage can crown the effort made to save the patient or compromise the previous efforts and should be managed by a multidisciplinary team. Analgesic, antibiotic, prokinetic, and anticoagulation therapy in the case of prolonged immobilization should be taken into account. Diuresis and intestinal transit will be monitored, and oral nutrition will be gradually resumed. Local complications  or general complications, such as cardiorespiratory, renal, or hepatic failures, should be closely monitored and treated promptly .○The main cause of low bowel obstruction is colorectal cancer.○Preceded by early or sudden signs, low neoplastic obstruction generally has the meaning of a neoplasm in an advanced evolutionary stage.○The therapeutic measures adopted  must have a dynamic character in accordance with the particular situation of the patient.○Except for certain or probably benign etiologies, the possibility of colorectal neoplasia should always be considered, in low obstructions, regardless of the patient’s age.○A “truce” in the fight against the obstruction can be deceptive.


Hamartoma is a mass formed by the proliferation and disorder of two or more kinds of cells inherent in normal organs or anatomical parts, which can occur in any part of the body. The most common hamartoma are kidney hamartoma, spleen hamartoma, liver hamartoma, and lung hamartoma. Urethral hamartoma is extremely rare in clinical practice.Combined with literature review, the diagnosis and treatment process of a child with posterior urethral hamartoma and hypospadias in our hospital were analyzed. The patient was cured after surgical treatment, the lesion was completely removed, the appearance was satisfactory, and there was no recurrence, urethral stricture, urethral fistula, and other complications. The pathological results of this case support the histological diagnosis of hamartoma, which provides reference for the clinical diagnosis and treatment of congenital malformation and tumor of urogenital in children.When a child has posterior urethral hamartoma, the symptoms may not be very typical, and it is often combined with urethral malformation. Therefore, it is necessary to perform careful physical examination combined with pathological examination to be able to make an accurate diagnosis. Under normal circumstances, the prognosis of urethral hamartoma is good. However, more cases are needed to be observed for verification, and a long-term effective follow-up after surgery is needed. In clinical practice, hamartoma in the urethra is extremely rare, especially in the posterior urethra of children. X-ray imaging, color Doppler ultrasound, CT scan, MRI, and voiding cystourethrography (VCUG) are helpful in the diagnosis of this tumor. Most of the tumors are round solid masses, and generally tend to be benign. A surgical resection is the only effective method for the treatment of urethral hamartoma . UrethraA boy aged 1 year and 5 months was admitted to the hospital because of ectopic urethral opening and perineal vegetation for more than 1 year. After birth, the parents found that the child has an abnormal appearance of the penis, with the penis bending downward, the urethra opening in the perineum, and a red polyp protruding from the perineum urethral orifice. As the child grows, the protrusion gradually increased and the deformity became more serious. The patient did not experience frequent urination, urination pain, urgency, or dysuria. Ultrasound examination in the outpatient department of our hospital showed that “there was no definite echo of the uterus and ovary in the pelvic cavity, and there was no obvious abnormality in the ultrasound images of the bilateral testes and epididymis,” and the chromosome examination showed “46, XY.” The outpatient was admitted to our department with “perineal vegetation unknown, perineal hypospadias.” The results of the physical examination conducted by the admission specialist were the following: the penis body of the child was short, the penis body and glans were poorly developed, the urethral opening was at the perineum of the ventral root of the penis, the penis was severely curved downward and was difficult to extend, and the foreskin was cap-like covering the dorsal side of the penis glans. The red polyp protruded from the perineal urethral orifice and was located approximately parallel to the penile body . The proThe child underwent urethral vegetation resection and cystoscopy under general anesthesia. The urethral opening was found from the middle of the red vegetation protruding from the ventral penile body and perineum, and the cystoscope was inserted. The root of the abnormal vegetation in the perineum was located at the distal end of the verumontanum opening approximately 0.5 cm . The vegThe operation was successful, and postoperative treatments such as prevention of infection, preventive hemostasis, nutritional rehydration, and dressing change were provided. The results of postoperative pathological examination showed that part of the examined tissues were polypoid hyperplasia, covered by the proliferative squamous epithelium, under which were proliferating fibers, small blood vessels, and smooth muscle, and diffuse infiltration of chronic inflammatory cells . Some tiThe child was reexamined in the outpatient department of our hospital since early childhood, and through continuous physical examination comparison, it was known that the urethral vegetation continued to grow. We provided this case through relevant diagnosis and treatment and literature analysis of “urethral hamartoma.”First reported by Albrecht in 1904, “hamartoma” is a tumor-like malformation caused by the wrong combination and arrangement of normal tissues in an organ during development . It is oThe main complaints of the child were the abnormal appearance of the penis and ectopic urethral opening. Since birth, the child was found by his parents to have ectopic urethral opening and a red polyp protruding from the perineal urethral opening, and the protrusion gradually increased with the growth of the child. Whether clinical diagnosis or imaging diagnosis, hamartoma has a high misdiagnosis rate . MRI is A complete surgical resection is the only effective treatment for posterior urethral hamartoma. In this case, the mass was completely removed during operation, and the results of the postoperative pathological examination supported the histological diagnosis of hamartoma. Histologically, the main components of hamartoma include mesenchymal tissues such as cartilage, fibrous tissue, adipose tissue, nerve, and blood vessels . The proPosterior urethral hamartoma complicated with hypospadias is rare in children, and more clinical practice studies are needed. Before surgery, it is necessary to perform conventional series of examinations such as x-ray imaging, color Doppler ultrasound, CT scan, MRI, and VCUG. Before a formal resection, urethrocystoscopy is needed to further clarify the nature and location of the tumor and the condition of the urethra. During the resection, attention should be paid to reduce the damage of urethral mucosa, so as to minimize the influence on the subsequent urethroplasty. Therefore, in patients with urethral hamartoma, more cases to be observed and a longer-term effective follow-up are needed for verification.


Bilateral pneumothorax is a rare occurrence and vigilant clinical examination is mandatory to suspect that during the presentation. This case illustrates a young lady presented with bilateral pneumothorax, new identification of lung cysts and chylous pleural effusion leading to diagnosis of lymphangioleiomyomatosis. This case illustrates a young lady presented with bilateral pneumothorax, new identification of lung cysts and chylous pleural effusion leading to the diagnosis of lymphangioleiomyomatosis. A 27‐year‐old lady presented to our hospital with abrupt severe breathlessness with chest tightness. She was sweating, severely tachypnoeic, tachycardic and hypoxic. Chest examination revealed central trachea, bilaterally reduced breath‐sounds and hyperresonance on percussion. Chest sonography suspected bilateral pneumothorax and chest x‐ray Figure  confirmeThe high‐resolution computed‐tomography Figure  of chestLymphangioleiomyomatosis is a rare cystic lung disease with involvement of lymphatics.Both authors contributed to the conception of the case report. Harshana Bandara wrote the first draft. Dushantha Madegedara revised subsequent versions and approved the final version.None declared.The authors declare that appropriate written informed consent was obtained for the publication of this manuscript and accompanying images.


Localized colorectal amyloidosis has a good prognosis, but cases involving bleeding or perforation may require surgery. However, there are few case reports discussing the differences in the surgical strategy between the segmental and pan-colon types.λ type). We diagnosed localized segmental gastrointestinal amyloidosis, because there was no amyloid protein in the margins, and the tumor was localized. There were no malignant findings.A 69-year-old woman with a history of abdominal pain and melena was diagnosed with amyloidosis localized in the sigmoid colon by colonoscopy. Since preoperative imaging and intraoperative findings could not rule out malignancy, we performed laparoscopic sigmoid colectomy with lymph-node dissection. Histopathological examination and immunohistochemical staining revealed a diagnosis of AL amyloidosis (Unlike systemic amyloidosis, localized amyloidosis has a favorable prognosis. Localized colorectal amyloidosis can be classified into the segmental type, in which amyloid protein is deposited locally, and the pan-colon type, in which amyloid protein is deposited extensively in the colon. Amyloid protein causes ischemia due to vascular deposition, weakening of the intestinal wall due to muscle layer deposition, and decreased peristalsis due to nerve plexus deposition. No amyloid protein should remain outside the resection area. The pan-colon type is often reported to cause complications such as anastomotic leakage, and primary anastomosis should be avoided. On the other hand, if there is no contamination or tumor remnants in the margin, the segmental type may be considered for primary anastomosis. Amyloidosis is characterized by extracellular deposition of amyloid protein in various organs . AmyloidA 69-year-old Japanese woman with a history of hypertension was referred to the hospital for abdominal pain and melena. No shock was evident, and lower gastrointestinal endoscopy revealed a well-defined submucosal tumor-like protruding lesion with a central ulcer in the sigmoid colon Fig. . The lesAt the time of surgical referral, the abdominal pain had improved, and tenderness and peritoneal irritation signs were absent. The gastrointestinal amyloidosis was localized but symptomatic, so the patient underwent elective laparoscopic sigmoid colectomy as well as a total biopsy. There was a soft mass in the sigmoid colon, and the serosal surface was slightly uneven Fig. . There wλ type)  Fig. . As therAL amyloidosis is the most frequent type of amyloidosis compared with other types, and the annual incidence of new cases of amyloidosis is high 78%) . The una8% . The In the literature published to date, 14 cases of surgery for colorectal amyloidosis have been reported . ExcludiThree changes result from amyloid protein deposition in the intestinal wall. The first is the deposition of amyloid protein in the vessel wall, which causes vessel wall thickening and weakening. These changes result in narrowing of the vessel lumen and infarction, leading to ischemia of the intestinal wall. Second, amyloid protein deposited within the intestinal wall destroys the muscle layer, resulting in weakening of the intestinal wall. Third, deposition of amyloid protein in the Auerbach's plexus leads to decreased peristalsis. Decreased peristalsis causes intestinal contents to become stagnant, resulting in increased intestinal pressure . This chIn the Japanese literature, there was a case of three times of colonic perforations that eventually revealed amyloid protein deposition in the entire colon, and the diagnosis was the pan-colon type of localized amyloidosis . Ussia eIn this case, we determined from the preoperative pathology and intraoperative findings that the patient had localized segmental amyloidosis, and primary anastomosis was performed. The postoperative pathology also supported the results. As far as we could find, there are no studies mentioning the safety of anastomosis or resection margins in the segmental type of localized amyloidosis. Therefore, it is important to consider the segmental type or pan-colon type in planning treatment strategies preoperatively. However, unlike cancers arising from the mucosa, in gastrointestinal amyloidosis, it is difficult to determine the exact extent of amyloid deposition preoperatively based on endoscopic findings and tissue biopsy alone. Because of the autofluorescence property of the AL amyloid protein, Kuroha et al. mentioned the possibility that areas of amyloidosis will appear bright green on autofluorescence endoscopy . This meLocalized colorectal amyloidosis has a good prognosis but may require surgery due to perforation or bleeding. The segmental type may be considered for primary anastomosis. On the other hand, sub-total colectomy should be considered as an option, and primary anastomosis should not be performed in the pan-colon type.


Linear lichen planus (LLP), also known as blaschkolinear or blaschkoid lichen planus, is a rare subtype of lichen planus that presents along the lines of Blaschko. While LLP has been associated with vaccinations, neoplasms, medications, and successive pregnancies, we present a case of LLP following a primary pregnancy. A 29-year-old G1P1 female presented to dermatology for the evaluation of an intensely pruritic, whorled rash confined to her left lower leg that appeared shortly after the birth of her first child. A biopsy of the lesion and subsequent histopathology confirmed the diagnosis of LLP. The patient was treated with topical steroids with minimal response to therapy and declined further treatment. Linear lichen planus (LLP), also known as blaschkolinear or blaschkoid lichen planus, is a rare subtype of lichen planus that presents along blaschkoid lines . The blaA 29-year-old G1P1 female presented for the evaluation of an intensely pruritic rash localized to her left lower leg that began three months after giving birth to her first child. A physical examination of the patient’s left lower leg revealed excoriated, violaceous, lichenified papules and plaques in a blaschkoid distribution , linear psoriasis, and drug-induced blaschkitis. While lichen striatus can morphologically mimic LLP, lichen striatus typically presents in children and is histologically characterized by an epidermal lichenoid infiltrate . ILVEN aWhile LLP typically self-resolves slowly over the course of months, medical management is frequently necessary. Topical glucocorticoids are the first-line therapy, but oral glucocorticoids might be indicated in extensive or refractory cases. Other treatment options include phototherapy and oral antihistamines . Post-inWhile LLP has been reported in association with various vaccinations, neoplasms, medications, and successive pregnancies, our case represents the first biopsy-confirmed case of primigravida LLP eruption presenting after delivery. Because LLP may present similarly to other blaschkoid dermatoses, a biopsy often proves useful in confirming the diagnosis and informing the treatment plan. It is important that dermatologists recognize this condition and treat it appropriately as early intervention can greatly improve patient morbidity.


A 68-year-old female was admitted to the emergency department due to headache, mental confusion and a mild hemiparesis on the left side in October 2021. Her medical history revealed dilatative cardiomyopathy, arterial hypertension and nicotine abuse but no known malignancy. Clinical evaluation yielded a hemiparesis on the left side grade 4 according to the Medical Research Council (MRC) scale. Magnetic resonance imaging (MRI) was performed and showed a right temporal mass lesion with perifocal edema. The case was discussed at the interdisciplinary neuro-oncological board and surgery was indicated.At surgery a temporal craniotomy was performed followed by a temporal pole resection with resection of the temporomesial structures. The surgery was uneventful and the patient had no new neurological deficits. A postoperative MRI scan showed a complete resection of the contrast-enhancing portions. The patient was transferred to an oncology ward. Due to renal insufficiency, the chemotherapy could only be started with a reduced dose of cytarabine and without methotrexate. The chemotherapy was well tolerated. No tumour recurrence has been detected in the regular follow-up MRIs to date.The MRI showed a large space-occupying intra-axial lesion of the right temporal lobe Figs. , 2 and 3Glioblastomas  are the most common primary central nervous system (CNS) tumors in adults, mostly arising supratentorially with a preponderance in men in the 7th decade of life . AccordiGliosarcoma (WHO grade 4) is defined as a subtype of IDH wildtype GBM, histologically characterized by a biphasic tissue pattern with glial and mesenchymal differentiation , 5. In MMetastases represent the most common secondary CNS tumor in adults with roughly 1/3 of all intracranial neoplasms, commonly arising from lung or breast carcinoma or malignant melanoma . They maPrimary central nervous system lymphomas (PCNSL) are relatively rare, accounting for only 4% of all intracranial neoplasms, with a peak incidence at about 60 years , 9, 10. Pleomorphic xanthoastrocytomas (PXA) WHO grade 2–3 are rare astrocytic tumors accounting for 1% of primary brain tumors, usually seen in the pediatric and young adult population. PXAs typically occur in the supratentorial brain with a predilection for the temporal lobe, commonly related to temporal lobe epilepsy. With a mostly superficial localization, a cyst with Gd-affinitive nodules is often part of the lesion, which in addition often presents with an adjacent lepromeningeal and/or dural Gd-enhancement . Due to In the hematoxylin and eosin (H&E) stained sections of the formaldehyde-fixed and paraffin-embedded biopsy material, fragments of a highly cellular, diffusely growing tumor with many medium to large sized lymphoid cells were found Fig. . These lIn summary, the histopathological finding of a high cellular, highly proliferative, diffuse growing tumor with large B cells (CD20 + CD79+) primarily appearing within the temporal lobe leads to diagnosing a primary diffuse large B‑cell lymphoma of the CNS.Diffuse large B‑cell lymphoma of the CNS (CNS-DLBCL) is a rare tumor that accounts for 1–3% of primary CNS tumors but is the most common tumor type encountered among primary CNS lymphomas , 18. The


Mycobacterium abscessus. She received five weeks of broad-spectrum IV antibiotic therapy followed by three months of oral azithromycin, tigecycline, and linezolid. A one-year post-operative visit showed appropriate healing without crusting or visible infection. This case contributes to the small handful of documented presentations of NTM rhinosinusitis in immunocompetent patients. NTM should be considered when patients present with refractory rhinosinusitis as they may require extended courses of antibiotic treatment. Familiarity with risk factors can further expedite making a diagnosis, ensuring prompt initiation of treatment and relief of symptoms for patients.Non-tuberculous mycobacteria (NTM) are slow-growing opportunistic pathogens that cause a variety of cutaneous, soft tissue, and pulmonary infections. On rare occasions, NTM causes chronic rhinosinusitis, with the majority of cases presenting in immunocompromised individuals. Other potential risk factors include the presence of foreign bodies, previous sinus surgery or chemoradiation, and use of contaminated water in sinus rinses. We report here a rare case of NTM rhinosinusitis in an otherwise immunocompetent 66-year-old female. The patient underwent functional endoscopic sinus surgery where intraoperative acid-fast bacteria cultures grew  S. aureus and anaerobic organisms  are common bacterial isolates for CRS [Chronic rhinosinusitis (CRS) is a common disease process affecting 5-12% of the global population. Due to the vague nature of defining CRS and overlapping comorbidities , prevalence is likely overestimated [ for CRS . RefractNon-tuberculous mycobacteria (NTM) are opportunistic, rapidly or slow-growing, acid-fast bacilli pervasively found in soil, water, and the natural environment. Pulmonary disease, superficial lymphadenitis, and soft tissue infections are localized clinical manifestations of NTM, though disseminated disease is seen in HIV and immunocompromised patients. Other risk factors for NTM isolates include prior chemoradiation and foreign body presence . NTM sinPatient one is a 66-year-old female with a past medical history notable for CRS, acquired hypothyroidism well controlled with 100mcg levothyroxine daily, blood transfusion, and turbinate hypertrophy that was treated with right sinus surgery 10 years prior to presentation. The procedure was uncomplicated and provided significant relief.She recently presented to an external clinic complaining of a three-month history of chronic, persistent nasal obstruction, sinus and retro-orbital facial pressure, facial pain, colored mucopurulent drainage, headaches, aural fullness, and dizziness. The patient had just undergone a left upper root canal secondary and a recent tooth extraction, during which her tooth shattered and was not completely removed. While awaiting removal, she received multiple courses of antibiotics from her periodontist and developed throbbing, left periorbital pain. Her history is otherwise unremarkable and negative for tobacco, alcohol, or recreational drug use. She is a retired educator and previously worked in a microbiology lab and is devoid of exposure to acid-fast bacilli, fungi, and additional tuberculosis risk factors.After referral to a local ENT, she was deemed refractory to multiple topical and oral antibiotics, steroids, antihistamine (azelastine), and high-volume nasal topical irrigations. She presented to our institution for a second opinion. On assessment, the patient was afebrile and in no acute distress . Her nasal obstruction (left > right) was positional and improved in an upright position and exacerbated by bending down. On physical exam, she was tender on percussion in the left frontal and maxillary sinus. The nasal septum was deviated to the left and no masses. The head and neck exam was otherwise unremarkable. Initial cultures were negative. Initial diagnostic nasal endoscopy demonstrated synechia at the right middle turbinate and lateral wall, and thick, yellow mucus in the maxillary sinus outflow tract. A maxillofacial CT scan demonstrated thickening of the left frontal, ethmoid, and maxillary sinuses, with the left side being more affected than the right. Maximal medical therapy was recommended at this time, and daily antihistamines, saline irrigation, and nasal steroids were initiated.Mycobacterium abscessus without acid-fast bacilli visualized on direct smear. Culture sensitivities were susceptible to amikacin, cefoxitin, clarithromycin, and impinenem with tentative susceptibility to azithromycin, clofazimine, and tigecycline.With unchanged symptoms after three months, functional endoscopic sinus surgery was planned. Intraoperatively bony osteitis, hypertrophy throughout the ethmoid cavity, and a 3mm bony dehiscence on the cranial wall of the right orbit were visualized. Bilateral and endoscopic total ethmoidectomy, frontal sinusotomy, maxillary antrostomy with removal of tissue, concha bullosa resection, and turbinate outfracture were done. Though extensive damage was present, the bone fragments in the pathology sample did not elicit osteomyelitis or pathologic changes. The patient was discharged afebrile and hemodynamically stable. Intraoperative cultures of the biopsy specimen eventually resulted in The patient then had a peripherally inserted central catheter line placed and antibiotics were. Plan was to complete a total of 12 months of antibiotic therapy with the first six to eight weeks being IV therapy . However, five weeks after initiation, all antibiotics were discontinued due to the presence of transaminitis and side effects of ocular migraines, tinnitus, and nausea. She was started on oral antibiotics with azithromycin, tigecycline, and linezolid. A second irrigation six weeks post-operatively at the ENT clinic only grew normal flora. Postoperative physical exam revealed midline septum and absence of previously noted crusting residual tenderness of sinuses. Post-op endoscopy after rigid nasal debridement demonstrated turbinates in the appropriate position and patent air spaces bilaterally. The patient completed three months of oral antibiotic therapy. A one-year post-operative visit showed appropriate healing without crusting or visible infection. M. avium complex, M. chelonae, M. fortuitum, and M. abscessus [M. chelonae, M. fortuitum, and M. abscessus [M. abscessus complex and M. mucogenicum. Of note, the prevalence of NTM extrapulmonary infection was higher in younger black patients, possibly due to higher rates of HIV or other immunocompromised states within this population [Nontuberculous mycobacteria are environmental opportunistic pathogens found ubiquitously in natural water and soils. Common species includebscessus ,6. NTMs bscessus . Rhinosibscessus . AnotherM. avium isolates clonally related to the patient’s isolates.A majority of the cases of NTM CRS in the literature occur in individuals with underlying HIV infection, cystic fibrosis, or other immunocompromising conditions -13. OnlyNTM as an underlying cause of CRS is rare, and as such is difficult to identify. NTM should be suspected in patients with a long history of refractory CRS that has been unresponsive to standard therapies that are aimed toward the more common pathogens. Initial acid-fast bacterial (AFB) tissue staining is not sensitive or specific ,16, as sOnce a definitive diagnosis is established, treatment typically involves long-term antibiotic therapy ,12,16-20In this case, we present an immunocompetent individual with a history of prior sinus surgery who was diagnosed with refractory NTM CRS, and treated with prolonged IV and oral antibiotics. This case adds to a growing body of literature documenting the clinical presentation of NTM CRS in immunocompetent patients. It is important that physicians are aware of these cases in order to promptly diagnose the condition and begin appropriate treatment.


Laryngeal schwannomas are encapsulated tumors composed entirely of benign neoplastic Schwann cells,Laryngeal schwannomas usually present as insidious, slow-growing, submucosal masses and are most commonly seen between the ages of 20 and 50.Most schwannomas were located in the false vocal cords or in the aryepiglottic folds.The treatment of choice is surgical excision with clear margins and the final diagnosis can only be confirmed through histopathology. The overall prognosis and outcome for laryngeal schwannoma is good.We intend to call attention for a very rare case of dysphonia and stridor in a 7-year-old girl that should be included in the differential diagnosis.A 7-year-old girl presented with one-year progressing dysphonia and a recent (1-month) appearing mild stridor. She had no significant medical history. Flexible fiber-optic laryngoscopy revealed a submucosal lesion in the left ventricle region A. The voIn 1987, Stanley et al. described two pediatric cases – 12-year-old female with hoarseness and 17-year-old male with other voice abnormality.5The most common presentation is related to vocalization, with hoarseness or dysphonia experienced by most patients (71.2%), followed by dysphagia (24.7%), dyspnea (23.3%), and foreign body sensation (16.4%). The presented case had one-year progressing dysphonia, seeking medical consultation only when presented mild stridor.Most schwannomas are located in the false vocal cords (45.8%) or the aryepiglottic folds (33.3%) with less frequent involvement of the true vocal folds (16.7%), epiglottis (9.7%), subglottic areas (5.6%), and post cricoid areas (4.1%).The imaging of schwannomas shows typical features of a benign lesion: oval shape, regular margins, and absence of osseocartilaginous erosion and compression without infiltration of surrounding structures,The diagnosis of certainty is given by histopathology, based on the Enger and Weiss histological schwannoma diagnosis criteria: (1) Encapsulated tumor; (2) The presence of Antoni A and/or Antoni B stroma; and (3) A positive S100 immunostaining.Indications for surgical treatment are tumor growth and presentation of symptoms.That was the chosen approach, with elevation of a micro flap and submucous dissection of the mass. Other alternatives of external excision are laryngofissure, lateral pharyngotomy or lateral thyrotomy.The prognosis for laryngeal schwannoma is extremely good.Our last revision fibro laryngoscopy was done with 2-years of follow-up and there is no sign of recurrence.Schwannoma within the larynx is rare and can present with a variety of symptoms ‒ from mild dysphonia to life threatening respiratory distress. We present a case with these both extremes’ symptoms: one-year progressing dysphonia and a one month appearing mild stridor. Diagnosis can only be conﬁrmed through histopathology. Surgical excision with clear margins remained as the treatment of choice, and should be done endoscopically, if possible, like in the presented case. The overall prognosis and outcome for laryngeal schwannoma is good, as demonstrated by the 2-year follow-up with no symptoms and no signs of recurrence.We state that there was no submission of the case report ‒ Unusual cause of progressing dysphonia and stridor in a child ‒ to the Ethics Committee, since there is no demand from the main institution. Both patient and the responsible relative agreed with the discussion and publication of the case.This paper has no funding source.We state that there are no known conflicts of interest associated with this publication and that there was no significant financial support for this work that could have influenced its outcome.We authorize the publication of the article and declare that it is unpublished and has not been submitted in another journal.We confirm that the manuscript has been read and approved by all the named authors and that there are no other people who meet the criteria for authorship who are not listed. We also confirm that the order of the authors listed in the manuscript has been approved by all.We confirm that the appropriate intellectual property protection measures associated with this work have been provided and that there are no impediments to publication regarding intellectual property. In doing so, we confirm that we follow our institutions' regulations on intellectual property.We further confirm that any aspect of the work covered in this manuscript that involved humans was conducted with the ethical approval of all relevant bodies and that these approvals are recognized in the manuscript.


Primary necrosis of the ligamentum teres hepatis (PNLTH) is an extremely rare disease which presents with acute abdominal pain. It has easily been misdiagnosed due to lack of clinical manifestations. Early recognition of PNLTH is crucial for treatment while CT is a good methed to diagnose and differential diagnose PNLTH.Teaching Point: Primary necrosis of the ligamentum teres hepatis (PNLTH) is an extremely rare disease characterized with acute abdominal pain, while computed tomography is the recommended technique to diagnose and differential diagnose this disease. Liver function tests with normal enzymatic profile and bilirubin was plain. The patient was suspected with acute pancreatitis and subsequently received an enhanced computed tomography (CT) scan for further diagnosis. The CT examination of the abdomen showed normal morphology of pancreatic with fewer exudation above the body of pancreas. A hypodensity strip  and WBC 21 × 10CT results suggested the diagnosis of necrosis of the ligamentum teres hepatis and provided a CT volume reproduction of the lesion to show its neighboring relationships (The hepatic round ligament is a remnant of umbilical vein atresia, formed by the embryonic remnants of degenerative atresia of the left umbilical vein, which connects to the left hepatic vein or inferior vena cava through the venous ligament. The clinical incidence of PNLTH is extremely low and the pathogenesis is unclear . In this


Despite its extremely low incidence, intra-abdominal herniation through the lesser omentum is associated with a high mortality rate and must be recognized early and treated urgently. To overcome a lack of data on the management of this condition, we collected and reviewed all the reported cases of operated lesser omental hernia and presented the case of a patient treated by laparoscopy for an isolated lesser omental hernia.According to PRISMA guidelines and using PubMed, Cochrane Library, and Web of Science, a systematic literature review of cases of lesser omental hernia treated by surgery was performed on February 12, 2023.Of 482 articles, 30 were included for analysis and only 9 articles presented an isolated hernia through the lesser omentum. Among these, 4 patients were female and the median age was 38. Upper abdominal pain and vomiting were reported in 7 out of 9 patients. The small bowel was responsible for 78% (7/9) of all lesser omental herniations. All of them were treated by laparotomy. In addition, we describe the case of a 65-year-old woman without prior surgical history who was treated by laparoscopy for a spontaneous closed loop hernia through the lesser omentum without any other associated hernias.Mostly associated with prior surgery or trauma, this type of herniation could sometimes occur spontaneously without any sign of peritonitis. Due to the high mortality rate, internal abdominal hernias should always be ruled out with a CT scan in front of patients presenting with persisting acute abdominal pain and no alternative diagnosis.The online version contains supplementary material available at 10.1007/s00464-023-10279-4. Transomental herniations are rare conditions, mostly reported in patients over the age of 50 years old . An abnoA classification of lesser omental hernia has been proposed by Kitagishi et al. . Two forTo add to the scientific knowledge of this rare manifestation in accordance with the guidelines for surgical case reports (SCARE) , we repoRegarding the case presentation, data was retrieved from patient records and presented according to the SCARE 2020 guidelines. Guidelines do not require IRB approval.https://www.researchrabbit.ai) was used to collect additional articles. Language was restricted to English. The search terms used in the title, abstract, Medical Subject Headings, and keyword fields included combinations as follows: (((lesser omentum) OR ) OR (gastrohepatic omentum)) AND (hernia).We then performed a systematic literature review in accordance with the PRISMA 2020 guidelines . This syAll articles presenting in English with a case of lesser omental herniation were selected for analysis. No limitations were applied on the age of the patients or their ethnicity. Conference abstracts, simulation studies and clinical studies in non-human subjects, unpublished studies, and other studies in a foreign language were not included. Studies involving patients who presented other types of intra-abdominal herniations were also removed.Two authors (ASA and AB) independently identified the relevant studies based on the title and the abstract. Selected articles were then fully read. If the studies met all selection criteria, data were extracted independently by the two authors. Disagreement was resolved after consultation with the senior author (TR).The following variables were extracted: the name of the first author, the publication year, the patient age and gender, types of symptoms, the associated hernias, the herniated organ, surgical history, abdominal trauma history, and the type of surgical treatment. Two authors (ASA and AB) independently identified these parameters.Through collecting all the current evidence of this condition, we hope to gain better insight into this rare phenomenon.A 65-year-old woman was admitted to the emergency department for an upper abdominal pain scaled at 10/10 in the visual analogue scale (VAS) , 15. TheAt follow-up appointment, the patient described persistent mild pain in the upper abdominal region. Laboratory tests were normal except for a mildly elevated C-reactive protein. A complete US did not find any signs of acute cholecystitis, but a moderate quantity of free intraperitoneal fluid was detected. A total abdominal computed tomography (CT) scan showed a “closed loop” small-bowel internal hernia of the lesser omentum and free intraperitoneal fluid along the dilated upward ileal loops, in the pouch of Douglas and in peri-splenic localisation Figs. , 3.Fig. The patient was admitted to the operation room for emergency laparoscopy surgery Fig. . LaparosAmong 482 studies, only 30 available articles of which were case reports, described patients with a lesser omentum hernia in English Fig. . The medOf these 30 studies, only nine cases presented a direct herniation through the small omentum into the lesser sac as in the presented case and was also defined as type 1 according to Kitagishi classification or type 4 according to Chen classification (Supplementary Table 2) –24. FourWe presented the first case of a patient with a lesser omental hernia, treated successfully with laparoscopic surgery alone. A lesser omental hernia diagnosis is challenging as symptoms are variable and can overlap with other abdominal diseases. In this case, the patient had no risk factors for an intra-abdominal hernia. The laboratory tests were reassuring and the diagnosis of a biliary etiology masked the clinical picture of bowel strangulation. While the exact cause of lesser omental defect was not identified, anatomic predisposition was probably responsible of the spontaneous bowel herniation into the omental bursa.Internal herniation describes the protrusion of a visceral content through a normal or an abnormal aperture within the abdominal cavity . IncludiTraditionally, plain abdominal radiography was a basic first-line diagnostic imaging. However, interpretation of a plain abdominal radiograph is challenging and past studies have shown low sensitivity in acute abdominal pain evaluation with this imaging tool . Recent In the reviewed cases most surgeries were performed by laparotomy and for direct lesser omental hernias all cases were performed by laparotomy. This could be related to the emergent character of bowel obstruction but also to the anatomic specificity of this region. Indeed, complex cases typically including strangulation require enlargement of the hernia sac dissection which due to the localization might be more confidently performed by laparotomy. However, depending on the situation, a simple traction on the herniated viscera could be enough to remove the obstruction. Only gentle traction must be applied to avoid loops or portal pedicle injuries. However, as presented in our case, laparoscopic management was feasible and prove to be an effective strategy to confirm the diagnosis and reduce the hernia.The limitations of the study are mainly due to the lack of existing data, mostly explained by how uncommon this type of hernia is. Only the lack of case reports precluded us from performing a meta-analysis. However, we now present for the first time, all the current evidence regarding lesser omental hernias and describe the first laparoscopic case of a spontaneous hernia through the lesser omentum.Mild abdominal pain and vomiting could hide an internal abdominal hernia. Past abdominal trauma or surgery are risk factors for lesser omental hernias. A CT scan should be the first-line imaging of choice for persistent acute abdominal pain without alternative diagnosis when the patient is stable. Laparoscopic surgery offers a safe and reliable approach to confirm the diagnosis and reduce lesser omental hernias.Supplementary file1 (DOCX 12 kb)Supplementary file2 (DOCX 18 kb)Below is the link to the electronic supplementary material.


Dear Editor,Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) is defined to be 6% of primary cutaneous lymphomas.A 25-year-old female patient was referred to the dermatology department with the complaint of slowly enlarging masses on her chest, abdomen, and upper arm for the past year. The lesions were slightly itchy. She had been applying mild topical corticosteroids to the lesions without any improvement. She had no medical history or concomitant disease. On physical examination, there were erythematous firm nodules sized approximately 0.5‒1 cm in diameter located on the left deltoid A, right A punch biopsy was performed on the abdominal nodule. Histopathological examination revealed dense, diffuse infiltration within the papillary dermis, composed of small and medium-sized T-lymphoid cells, with pleomorphic nuclei . There aStainings with PD-1 E and bclDue to the localization and multiplicity of the lesions, and also considering the aesthetic concern, a non-aggressive treatment option was preferred in this case. Oral doxycycline 100 mg per day was administered to our patient. Regression of the lesions started in the third week of the treatment and a total clinical remission was achieved after 4 months. She has been in our follow-up for 6 months after cessation of doxycycline and no recurrence has been observed.PCSM-TCLPD is a provisional entity with a benign prognosis. The classical clinical presentation of PCSM-TCLPD is an asymptomatic solitary erythematous papule, nodule, or plaque. Itching, pain, and ulceration have occasionally been defined. Multiple lesions as in our case have been described in the literature.Pseudolymphoma, primary cutaneous follicle center lymphoma, primary cutaneous marginal zone lymphoma, and tumoral stage of mycosis fungoides (MF) should be considered clinically in the differential diagnosis. Due to the clinical and histopathological overlap, the most difficult one to distinguish from PCSM-TCLPD could be pseudolymphoma. Causes and triggers of pseudolymphoma such as insect bites, infections, drugs and foreign agents  should be questioned. For MF, the existence of patches and plaques belonging to different stages of the disease, and epidermotropism of lymphocytes with atypical cerebriform nuclei in the histopathological examination are used to distinguish the disease from PCSM-TCLPD.There is no standard guideline for the treatment, options can be assessed according to individual features of the cases. Several reports showed that spontaneous resolution is possible after biopsy, therefore conservative approach rather than aggressive treatments is preferable. The modalities found to be successful in the treatment are topical or intralesional steroids, excision, radiotherapy, and systemic doxycycline.None declared.Esra Sarac: Study concept and design, data collection, analysis and interpretation of data, writing of the manuscript, critical review of the literature, final approval of the final version of the manuscript.Cuyan Demirkesen: Study concept and design, data collection, analysis and interpretation of data, writing of the manuscript, final approval of the final version of the manuscript.None declared.


ESA of the lower extremities where complete clinical remission was induced with triple combination medical management with intralesional 5-fluorouracil, field treatment with topical 5-fluorouracil and imiquimod, and oral acitretin. Our case adds to the literature supporting combination medical therapy for challenging cases of ESA.The termeruptive squamous atypia(ESA) is used to describe squamous proliferations that do not present with high-grade histologic features and for which surgical management may exacerbate the condition. Non-surgical management of ESA with radiation, local or systemic chemotherapy, retinoids, or immunotherapy have been reported with variable success. In contrast, combination treatment with retinoids, immunomodulatory or chemotherapeutic agents may result in a more durable response. We report a case of recalcitrant  Keratoacanthomas (KA) are typically considered to be low-to-moderate grade histologic variants of squamous cell carcinoma (SCC). Although classification and management remain contentious, complete surgical excision is considered the current gold standard treatment . HoweverLiterature review of this entity is challenging as cases have been reported as eruptive keratoacanthoma in cases when patients had multiple true SCCs KA-type and also in cases of ESA. Que et al. reported that ESA responded well to intralesional 5-fluorouracil (5-FU) with or without adjunct treatment with topical steroids, topical 5-FU, cryotherapy, or oral acitretin2. Non-surgical management with radiation or local or systemic chemotherapy, retinoids, and immunotherapy have been reported with variable success, however, they have often demonstrated incomplete resolution or recurrence with medical monotherapy -5. In coWe report a case of recalcitrant ESA of the lower extremities where complete clinical remission was induced with triple combination medical management with intralesional 5-fluorouracil, field treatment with topical 5-fluorouracil and imiquimod, and oral acitretin. Our case adds to the literature supporting combination medical therapy for challenging cases of ESA.A 68-year-old female with Fitzpatrick type I skin and past medical history of Grave’s disease status-post radiation, rheumatoid arthritis status-post treatment with rituximab, and IgG immunodeficiency presented for evaluation of numerous lower extremity lesions. She was initially seen at an outside practice six months prior to presentation where biopsies initially showed actinic keratoses with lichenoid inflammation but repeat biopsy was consistent with well-differentiated squamous cell carcinoma. The lesions were repeatedly treated with cryotherapy without improvement, and two lesions were resected, following which new lesions developed immediately post-operatively.On presentation to our clinic, her legs were covered in innumerable exophytic pink papules and plaques with central crust, in the range of 20-30 lesions, clinically consistent with keratoacanthomas or squamous proliferations Figures -1B. We sThe two largest lesions were injected with intralesional 5-fluorouracil (5-FU) and the patient was started on 10 mg of oral acitretin daily. On follow-up, the lesions treated with 5-FU demonstrated notable regression, so twelve more discrete and large lesions were treated with intralesional 5-FU. The patient was also instructed to field treat her legs with alternating courses of 5-FU and imiquimod for four to six weeks. The majority of her lesions had involuted on follow-up after this therapy Figures -2C. AcitPatients with multiple hyperkeratotic proliferations with low-grade histologic features, particularly on the lower extremities in the setting of repeated trauma, are likely presenting with eruptive squamous atypia (ESA) . These lMedical management with topical steroids, topical or systemic retinoids, topical immunomodulators like imiquimod, or chemotherapeutic agents such as methotrexate, cyclophosphamide, and topical 5-FU has been reported for ESA and presumed eruptive KA -6. Our aOur case supports current literature suggesting that a combination of medical therapies may be most effective when managing ESA, and care should be taken to avoid misdiagnosis and mistreatment of these lesions as though they are malignancies such as KA or SCC. Surgical treatments can exacerbate the condition without providing resolution, and we recommend a combination medical approach, as has been supported in the literature. To our knowledge, this is the first report of successful treatment with our triple combination of topical imiquimod, oral acitretin, and intralesional 5-fluorouracil.


Intrathoracic Solitary Fibrous Tumors (SFT) mainly arise from the pleura; however, these tumors may also originate from the mediastinum. We present a rare case of posterior SFT extending to several mediastinal sites and with an unusual large size, successfully treated with surgical resection.A 66-year-old female presented with an initial manifestation of ambiguous pain in the chest and dysphagia and later developed pitting edema in both lower extremities and cachexia five months before admission. Chest imaging confirmed a mediastinal mass (17 × 15 × 8 cm) which was surgically removed. Immunohistochemistry confirmed the diagnosis of a solitary fibrous tumor with positive B-cell lymphoma 2, STAT6, and CD99, negative S100 and smooth muscle actin, and low levels of Ki67 (5–7%). The patient’s follow-up course was unremarkable.Mediastinal SFTs may grow extremely huge, with the potential to invade multiple adjacent sites. Surgical removal of the tumor remains the mainstay of treatment in these cases. Solitary Fibrous Tumors (SFT), is a rare tumor originating from mesenchymal tissue. It is distributed equally between men and women, with a propensity to present in the fifth and sixth decade of life –3. AccorSFTs make up fewer than 2% of all soft tissue masses and are often seen in the pleura; however, these tumors may also be detected in other areas of the body, including skin, nervous system, soft tissue, liver, lung, kidney, and thyroid –8. AlthoA 66-year-old female presented with an initial manifestation of ambiguous pain in the chest and dysphagia and later developed pitting edema in both lower extremities and cachexia five months before admission. Past medical history was unremarkable, and the patient denied exposure to asbestos or other drugs or harmful substances. In the physical examination, vital signs were stable, and nothing significant was found. However, laboratory findings showed hypoalbuminemia .Before hospital admission, the patient underwent echocardiography, in which a mass was seen in the mediastinum. Therefore, a computed tomography (CT) was performed, showing an oval shaped, hypodense, well defined posterior mediastinal mass lesion measuring 160*80 mm in transverse and anteroposterior diameter, and 160mm in craniocaudal extension, causing anterior displacement of the heart, widening of subcarinal angle associated with mild to moderate right side pleural effusion. The adjacent thoracic vertebra was normal, however, linear atelectasis was also seen in basilar segment of right upper lobe  was inserted. The patient also developed sudden tachycardia. The electrocardiogram displayed atrial fibrillation with a rapid ventricular response, which was treated with amiodarone .After stabilization, the patient was scheduled for surgery. The thoracic cavity was entered from the fifth intercostal space . Due to the large size of the tumor and the localization of the tumor and heart, sternotomy could not be performed. We attempted to approach the patient through the left posterolateral position and left thoracotomy. A huge posterior mediastinal mass was detected with extension to both right and left chest cavities with important adhesion to the pericardium, arch of the aorta and descending aorta, inferior vena cava (IVC), esophagus, inferior pulmonary vein, and lung tissue with 300 cc pleural effusion. We considered changing the position and entering the right cavity in case the tumor could not be removed with en bloc resection and left thoracotomy. After pneumonolysis, a meticulous dissection of the lesion was done, and the lesion was excised. An air leak test was done, the chest cavity was irrigated, and the operation ended after inserting two chest tubes. Fortunately, complete removal of the tumor was feasible through the left posterolateral position and right thoracotomy was not required.A semi-ovoid lobulated creamy mass (17 × 15 × 8 cm) was received for histopathological examination. The capsular surface was bosselated, creamy, and vasculated. Cut sections showed homogenous creamy lobulated areas with fish flesh appearance and patchy necrotic areas. Necrosis was present at less than 5%, and one reactive lymph node was identified. Also, the mitosis rate was 1–2 per 10 high power field (HPF). Immunohistochemistry (IHC) confirmed the diagnosis of a solitary fibrous tumor with positive B-cell lymphoma 2 (BCL2), STAT6, CD34, and CD99 and negative S100 and smooth muscle actin (SMA). Furthermore, Ki67 levels were low (5–7%)  is considered an acceptable method in smaller tumors with no external invasion, the preferred surgical method for large tumors close to mediastinal organs, especially those located in the posterior mediastinum, is thoracotomy , 17. In Regarding the role of chemotherapy in SFTs, we did not administer chemotherapy since the tumor was localized and was removed with resection with clear margins. Also, the patient had no signs of recurrence during her 9-month follow-up. Surgical excision with clear margins has been mentioned to be the treatment of choice based on feasibility and improved survival , 27. AltMediastinal SFTs may grow extremely huge, with the potential to invade multiple adjacent sites. Due to several reasons, the diagnosis and treatment of these tumors have remained complex. Firstly, they may present with wide-ranging clinical manifestations. Secondly, even with appropriate clinical suspicion, the radiologic features of mediastinal SFTs on chest CT-scan are not distinctive. Finally, the definitive diagnosis is only established with the help of pathologists and IHC staining methods. Surgical removal is the mainstay of treatment, however, even with the complete resection of the tumors, the latter may reoccur. Therefore, all patients should be closely monitored on a regular basis in order to detect the earliest signs of recurrence.


VIPomas are a type of neuroendocrine tumor that independently produces vasoactive intestinal peptide (VIP). VIPomas causing watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome are not frequently observed in adult patients without pancreatic ailments. However, in children, the occurrence of a VIPoma originating in the pancreas is exceedingly uncommon. Instead, WDHA syndrome is more commonly associated with neurogenic tumors that secrete VIP, often located in the retroperitoneum or mediastinum. Among infants, chronic diarrhea is a prevalent issue that often necessitates the attention of pediatric gastroenterologists. The underlying causes are diverse, and delays in arriving at a definitive diagnosis can give rise to complications affecting the overall well-being of the child. The authors present the case of an infant with chronic watery diarrhea, subocclusion manifestations, mild hypokalemia, and metabolic hyperchloremic acidosis secondary to a VIPoma in the retroperitoneum that was diagnosed via abdominal ultrasound and tomography. The laboratory results revealed lowered potassium levels and an excessive secretion of VIP. Following the surgical removal of the tumor, the diarrhea resolved, and both electrolyte levels and the imbalanced hormone levels returned to normal. Immunohistochemical examination confirmed the diagnosis of ganglioneuroblastoma, with N-MYC negative on molecular biology tests. We present the clinical and histo-genetic aspects of this rare clinical entity, with a literature review. In 1958, Verner and Morrison initially documented the occurrence of intractable aqueous diarrhea and a deficiency in potassium levels , concomitant with noninsulin-secreting neoplasms originating from the islets of the pancreas . The abbPancreatic non-beta-cell hyperplasia is a rare condition, although it is observed in youngsters. The majority of medical training is based on case reports ,12. CurrPediatric patients may experience persistent diarrhea due to a wide variety of gastrointestinal disorders. Secretory diarrhea, which occurs in a tiny proportion of patients, is caused by active intraluminal fluid secretion. The identification of such pathologic cases is essential due to their significant diagnostic implications. A hidden VIPoma, a tumor that secretes VIP, may be the most likely cause of persistent secretory diarrhea. In children, extrapancreatic lesions are predominant; they are represented by pheochromocytoma, ganglioneuroblastoma, ganglioneuroma, and mastocytoma . The diaThe diarrhea is characterized by an extended duration of watery consistency, with a fasting stool volume exceeding 750–1000 mL/day. It is not septic and typically begins slowly, with a gradual increase in frequency over several years. The episodes of diarrhea become more persistent, occurring more than 10 times per day. If the diarrhea becomes severe, it can cause dehydration and an uncontrollable imbalance in electrolytes and acid levels, leading to acidosis. The hyperchlorhydria previously described is exacerbated by the inhibitory impact of VIP on parietal gastric mucosal cells, which potentiates dietary magnesium, zinc, and vitamins C, K, and B deficits linked to malabsorption . Skin raWhile the majority of these tumors cannot be removed completely, a multifaceted strategy can control both the symptoms and the tumor, allowing most patients to live a long life. As a result of the rarity of this neoplasm and the paucity of prospective data, there are no precise guidelines for the management of VIPomas .Healthcare providers need to recognize these additional symptoms when evaluating patients with VIPoma. Early diagnosis and treatment of VIPoma can help alleviate symptoms and prevent potentially life-threatening complications .The authors of this report describe a child diagnosed with chronic watery diarrhea and hypokalemia due to a retroperitoneal VIP-producing tumor, which immunohistochemical and molecular biology tests confirmed to be N-MYC-negative ganglioneuroblastoma. The symptoms were entirely resolved, and the preoperatively increased plasma VIP level returned to normal following surgical removal of the tumor. The case report is followed by a narrative review of the literature on this topic.A female patient, aged 11 months, was directed to our hospital due to a past of watery diarrhea, malnutrition, and significant abdominal distension. Vomiting was not reported. Over a month, she encountered prolonged bouts of watery diarrhea, occurring as frequently as 7–8 times a day, with no presence of blood or mucus.The mother, who is 42 years old, and the father, who is 40 years old, are both from a rural area.There was no evidence of a relevant family history of chronic illnesses. The female infant, who was the eighth to be delivered naturally, had a gestational age of 38 weeks, weighed 3100 g, and received a score of 9 on the APGAR scale. According to the national program, the child received his or her vaccinations. During the first month of life, the infant was naturally fed with her mother’s milk and then she received a milk formula. At the age of four months, supplemental feeding was also properly started. There is no relevant medical history for the infant. Her digestive function was normal for the first 10 months of her life. However, watery diarrhea started at the age of 10 months without a fever or vomiting.Before being admitted to the hospital, a diagnosis of a protein from cow’s milk allergy was made after four weeks of outpatient testing. The infant commenced a lactose-free, protein-hydrolysate formula meal, but the trial of cow’s milk exclusion was unsuccessful. The initial antiparasitic treatment for unproved giardiasis was ineffective. Despite the administration of oral rehydration solution on multiple occasions, the infant’s diarrhea endured without resolution.The pediatric patient was admitted with a stature measuring 70 cm, corresponding to the 5th percentile for their age, and a weight of 8 kg, aligning with the 25th percentile for their age. A physical examination revealed a regular heart rate of 85 beats per minute and normal blood pressure of 80/55 mmHg. The baby had significant abdominal distension, facial flushing, and no palpable abdominal mass or enlargement of the liver or spleen. Laboratory testing revealed mild metabolic acidosis and hypokalemia. The outcomes of a comprehensive analysis of blood cell counts and standard biochemical assessments fell within established normal parameters. We excluded the presence of infectious enterocolitis, intestinal parasitosis, celiac disease, exocrine pancreas insufficiency/mucoviscidosis, and cow’s milk protein allergy. The diarrhea persisted despite the introduction of a modular, elemental amino-acid-based dietary regimen.In light of the ongoing significant abdominal distension, an abdominal X-ray was conducted, revealing bowel dilation characterized by air-fluid levels and the absence of any luminal obstructive lesion .Following an abdominal computed tomography (CT) scan, an abdominal ultrasound revealed a calcified mass approximately 6/4 cm in diameter anterior to the L1–L4 lumbar spine and aorta but not in contact with the left kidney. There were no anomalies in the pancreas .A further endocrinological assessment was conducted to obtain a precise diagnosis. There were no symptoms of thyroid dysfunction. When plasma hormone levels were examined, it was found that VIP concentrations were increased . Additionally, the levels of homovanillic acid and vanillylmandelic acid in the 24 urinary samples were both normal. However, increased levels of neuron-specific enolase  raised concerns about a potential VIP-secreting ganglioneuroblastoma. The Department of Pediatric Surgery received a referral regarding the infant. A 6/4 cm encapsulated retroperitoneal tumor without contact with the left kidney was discovered during surgical exploration, anterior to the L1–L4 lumbar spine and aorta. There were no obvious alterations in the pancreas’ appearance. The next step was to remove the tumor, after which a slide was stained using the hematoxylin and eosin method for histopathologic analysis, and additional immunohistochemical and molecular tests were carried out.The tumor was histologically determined to be an INSS II-localized ganglioneuroblastoma intermixed with a stroma-rich (Shimada Classification) tumor with complete gross excision, positive lymph nodes, and a low MKI (mitosis-karyorrhexis index) of less than 100. The growth pattern of the tumor was solid/lobular. Large, round-to-ovoid nuclei and an abundance of basophilic cytoplasm were features of the tumor cells. A “salt and pepper appearance” with prominent nucleoli and alternate regions of euchromatin and heterochromatin was seen. Other tumor cells were distinguished by rich, eosinophilic cytoplasm, eccentric nuclei, and prominent cell boundaries. The tumor cells contained a small quantity of bleeding and had somewhat calcified dystrophic regions .Additional immunohistochemistry testing was conducted using the following panel of markers to confirm the final diagnosis and rule out other lesions with a similar appearance: Chromogranin, Neurofilament (NF 200), Protein S 100 (S100), Glial Fibrillary Acidic Protein (GFAP), Neutron-Specific Enolase (NSE), Synaptophysin, and T-Cell Surface Glycoprotein E2 (CD99). As an internal control marker, vimentin showed a favorable stromal response. The tumor cells responded moderately to strongly to NSE, Synaptophysin, and NF 200. The distribution of the cytoplasmic response pattern was diffuse and heterogeneous. In the ganglion cells and the stroma, immunohistochemistry for S100 and GFAP was highly reactive. S100 was also present in the cytoplasm of Schwann cells. Chromogranin was found to be isolated positively in neuroblasts. Overall, CD99 was rated as unfavorable .Additional molecular biology analyses were conducted. The status of the N-MYC gene was evaluated using fluorescence in situ hybridization (FISH), yielding a negative result.The patient experienced a smooth postoperative recovery. Diarrhea subsided, and laboratory findings indicated the restoration of plasma potassium levels to normal. Importantly, there was no recurrence of diarrhea during the three-year follow-up period. The plasma VIP concentration consistently remained at low levels in successive measurements after the surgical procedure.The first report on VIPoma dates back to 1970. Since then, about six review articles have been published studying the pediatric population. In these articles, 61 pediatric cases were recovered before primary exclusion. However, the incidence of cases in children has not yet been established. Although the prevalence of the pediatric population is unknown, the estimated annual incidence is roughly 1 in 10 million ,13.The mean diagnostic age for VIPoma varies widely, from childhood to eighty years, according to various studies . In chilThe majority of VIPoma cases are single tumors, but about 5% of people with multiple endocrine neoplasia type 1 (MEN1) syndromes also have VIPoma. VIPoma is a rather rare pathology that represents 1–2% of all pancreatic neoplasms. Every year, one in a million people are found to have VIPomas .A functioning pancreatic neuroendocrine tumor (pNET), known as the VIP-secreting tumor, or VIPoma, is extremely rare and life-threatening .PNETs are regarded as a subset of neuroendocrine tumors and have distinctive biology, natural histories, and treatment options. Depending on whether they emit peptide hormones that cause certain hormone-related symptoms, typically in predictable patterns based on tumor subtype, these tumors are categorized as “functional” or “non-functional” . BecauseVIP is secreted by this particular form of endocrine tumor. In 50 to 75% of patients, it affects the D1 cells of the pancreatic islets of Langerhans, and, at one out of every two cases at the time of diagnosis, lymph node metastases and/or liver are present . UsuallyChronic diarrhea in childhood is a fairly common pathology and can be the consequence of many gastrointestinal disorders. Four different pathophysiological mechanisms underlie the etiology of chronic diarrhea, and they often overlap: osmotic, secretory, associated with dysmotility, and inflammatory diarrhea. Only a small percentage of these cases involve true secretory diarrhea, which is the only type of diarrhea that lasts through extended fasting.Low stool osmotic gap  is a key indicator of secretory diarrhea, whereas high stool osmolarity and ending diarrhea with the removal of the trigger from the meal are specific to osmotic diarrhea. Inflammatory diarrhea is characterized by pathological feces that contain blood, mucus, and bacteria. Diarrhea accompanied by dysmotility, such as toddler diarrhea, is more challenging to diagnose and frequently remains one of exclusion .In most cases, there was a long time between the onset of diarrhea and the diagnosis of the tumor. This is supported by the results of two retrospective studies performed on cases of VIPoma in childhood. Murphy et al. conducted an investigation involving a cohort of six patients, elucidating an average hospitalization duration of four weeks for diagnostic evaluations. Notably, the period between the initial recommendation for hospitalization and the subsequent establishment of a diagnosis exhibited variability, spanning from one to ten months; the mean duration of persistent diarrhea was 5 months, and at the time of diagnosis, five children were severely malnourished . BourdeaAn early diagnosis of childhood VIPomas has been made possible using a diagnostic algorithm . Age is the initial clinical trait. Although WDHA has been described in people of various ages, it seems to be more prevalent in babies and young children. The average age of presentation was 21 months in the Iida study from 1980 , 18 montChronic diarrhea is the second symptom. It is reasonable to suspect secretory diarrhea and a VIP secretory tumor when there is persistent watery diarrhea that is resistant to fasting food and total parenteral nutrition over 48–72 hours. Bourdeaut et al.  observedThe third clinical sign is blood pressure. Blood pressure above the 99th percentile on repeated measurements should raise the suspicion of a neural crest tumor, especially if other signs and symptoms are present. This sign is not always present. For example, Swift et al.  presenteIida et al. , in theiWerner–Morrison Syndrome is a well-defined clinical and pathological condition that is uncommon but most frequently associated with malignancies that produce VIP . Most peIn most pediatric diseases, the microscopic appearance is represented by a neoplasm of neuroectodermal origin composed of a mixture of neuroblasts and ganglion cells in different proportions. The stage of neuroblast development differs between ganglioneuromas, ganglioneuroblastomas, and neuroblastomas.Ganglioneuromas, which include both Schwann and ganglion cells, are regarded as benign tumors.In 1915, Robertson defined ganglioneuroblastomas as a transitional tumor of sympathetic cell origin that contained malignant neuroblastomatous and benign ganglioneuromatous element .It is a tumor on the way to differentiation/maturation, but this process is not complete. Microscopic Ganglioneuroblastoma-intermixed (Schwannian stroma-rich) is scattered “residual” microscopic neuroblastomatous foci that come in the form of neuropil pockets containing a variable amount of neuroblastic cells at different stages of maturation and/or ganglion cells. The ganglioneuromatous constituent’s proportion within the microscopically assessed total tumor volume must surpass 50% .Over time, ganglioneuroblastomas have been a challenge for both pathologists and oncologists because there have been no clear and objective boundaries between neuroblastomas and ganglioneuroblastomas and between ganglioneuroblastomas and ganglioneuromas but also because clear guidelines for predicting the tumor biology and clinical prognosis of individuals with ganglioneuroblastomas have not been established. After the update of the WHO 2017 classification system for neuroendocrine tumors (NETs), the histological diagnosis followed this reporting format, which emphasizes a mitotic rate and a Ki-67 index . The IntThe majority of ganglioneuroblastomas are thought to be more aggressive tumors that typically develop in young children, with a mean age of onset of around two years. They are less mature forms made up of neuroblasts and ganglion cells.In our case, molecular biology tests showed N-MYC negative; this is also found in studies of neuroblastic tumors where most Ganglioneuroblastomas do not show MYCN amplification . The neuThis patient presented several specific clinical features from the diagnostic algorithm described above, such as young age at the onset of the disease, chronic watery diarrhea refractory to fasting food and total parenteral nutrition, facial flushing, and sweating. In our case, the blood pressure was normal, although high blood pressure is well known to be associated with neurogenic tumors in children.Individuals diagnosed with ganglioneuroblastomas generally experience a favorable outlook, given that these tumors have the potential to regress naturally or develop into ganglioneuromas. Regressive changes are observed in approximately 1–2% of all cases, and the underlying reasons for this phenomenon remain unidentified .In the 1960s, an initial hypothesis emerged suggesting that watery diarrhea resulted from the excessive secretion of catecholamines, a well-established phenomenon associated with neural crest tumors . SubsequThe presented case report indicated an elevation in VIP concentration. Furthermore, hypokalemia was observed. Roughly 90% of children diagnosed with neuroblastoma will exhibit elevated levels of vanillylmandelic and homovanillic acid production. If vanillylmandelic acid and other catecholamine levels fall within the normal range, the likelihood of a neuroblastoma diagnosis in the child is reduced. However, it is important to note that this diagnosis cannot be completely ruled out . CatechoIn this instance, the 24 h urinary levels of vanillyl mandelic acid and homovanillic acid were within the normal range. However, the serum level of neuron-specific enolase was elevated. This, coupled with the elevated serum levels of VIP, pointed towards the diagnosis of a VIP-producing ganglioneuroblastoma. This diagnosis was subsequently confirmed through histopathological examination and immunohistochemical staining.Following the International Neuroblastoma Risk Group (INRG) classification , this paThe infant presented in this case report was presumed to have a gastrointestinal disorder, consequently delaying the diagnosis. The patient underwent four weeks of outpatient different investigations, and the interval starting from hospital admission to diagnosis was two weeks. She received empirical trials of treatment for various unproven gastrointestinal disorders. The prompt identification of the diarrhea’s secretory nature might have expedited an early diagnosis, potentially preventing needless investigations and reducing associated morbidity.While bacterial and viral gastroenteritis can lead to secretion, sustained secretory diarrhea is infrequent. Secretory diarrhea may stem from uncommon hereditary electrolyte transport abnormalities, like congenital chloride diarrhea or congenital sodium diarrhea, yet these disorders typically manifest in early infancy . In thisIn a publication authored by Bourdeaut (2009)  involvinA literature review with a case series presentation published by Pai-Jui Yeh in 2020  describeConcerning histological alterations, in our instance, the neuroblasts did not exhibit the distinctive pseudo-rosette arrangement, and there was an absence of cystic degeneration. The presence of neuromelanin pigment inclusions, which are rarely documented in the literature, was not observed. Ganglioneuroblastomas present a challenging clinical and pathological scenario. Distinguishing morphological and immunohistochemical differences between ganglioneuromas, neuroblastomas, and Schwannomas displaying neuroblastoma-like features is considered a complex diagnostic task. Reaching a definitive diagnosis for these uncommon pathological cases involves a multifaceted process that heavily relies on integrating the patient’s clinical presentation, imaging findings, routine histopathological analysis, and supplementary immunohistochemical assessment of the samples.VIPomas frequently manifest with diarrhea as the sole clinical manifestation, necessitating meticulous gastrointestinal evaluations and therapeutic approaches.This instance depicts a distinctive occurrence of a VIP-secreting ganglioneuroblastoma in an infant, wherein diarrhea emerged as the primary clinical indicator. When faced with a pediatric patient experiencing persistent watery diarrhea of uncertain origin, it is advisable to conduct comprehensive gastrointestinal assessments alongside abdominal ultrasonography and/or CT scans to ascertain the presence of an underlying neoplasm. Morphologically, the examined tumor exhibited distinct characteristics that rendered the differential diagnosis a complex endeavor.