Intestinal nonrotation and cecal volvulus are rare clinical entities, and their coexistence is exceptionally uncommon. We present a case of a 41-year-old male patient with symptomatic intestinal nonrotation and associated cecal volvulus. Diagnostic imaging played a crucial role in recognizing the conditions and guiding surgical intervention. The patient underwent laparotomy and right hemicolectomy with a favorable postoperative course. This case highlights the challenges in diagnosing and managing these rare conditions. Further studies are needed to optimize the management strategies for this unique combination of pathologies. Cecal volvulus is a rare cause of large bowel obstruction that results from an axial twisting of the redundant cecum around its mesentery or its upward folding resulting in a closed loop. Over time, it may progress to strangulation, ischemia, and sepsis, with potentially deleterious consequences. It accounts for 30% of all colonic volvulus, occupying second place after sigmoid one, and has a peak incidence in the elderly .Normal development of the embryonic gut includes a 270° counterclockwise rotation that occurs in three phases, around the superior mesenteric pedicle . It can The coexistence of nonrotation and cecal volvulus is exceedingly rare. Diagnostic imaging plays a critical role in identifying intestinal nonrotation and its complications. Plain abdominal radiographs and computed tomography (CT) are commonly used for evaluation. Findings that may suggest intestinal nonrotation include a left-sided cecum, reversed relationship between the superior mesenteric artery (SMA) and superior mesenteric vein (SMV), and malposition of the DDJ ,3,4. WhiWe report the case of a 41-year-old male patient who presented with symptoms suggestive of intestinal obstruction, and was found to have an intestinal nonrotation with associated cecal volvulus on imaging. He was managed by laparotomy and right hemicolectomy, after which had an uneventful postoperative recovery.3), lactate dehydrogenase of 159 , normal lactic acid of 1.2 mmol/L, serum electrolytes within range, and no other relevant findings. Based on the clinical presentation, a preliminary diagnosis of intestinal obstruction was made. A plain abdominal radiograph, shown in Figures A 41-year-old male patient with no significant medical history presented to the emergency room with a complaint of colicky abdominal pain lasting for 24 hours. The pain was diffuse and mild, accompanied by nausea, and he experienced one episode of vomiting. The patient noted the absence of flatus and bowel movements since the onset of pain. He attempted self-medication with oral hyoscine butylbromide but found no relief. Vital signs were within normal limits. On abdominal examination, mild distension was observed Figure , bowel sTo further evaluate the condition, an abdominal contrast-enhanced CT scan was performed, which indicated signs suggestive of cecal volvulus and intestinal nonrotation Figures , 3A, 3B.The patient was taken to the operating room for an emergency laparotomy. A midline incision was made, and surgical findings included a markedly dilated cecum upwardly folded to the left hypochondrium with a 360° clockwise twisting upon its mesentery; no signs of necrosis or free liquid were noted; and no Ladd bands were found Figures , 4B. A rThe postoperative period was uneventful, with no complications observed. The patient was discharged home on the third postoperative day, resuming his normal diet and experiencing regular bowel movements.Intestinal nonrotation is a rare congenital condition that results from abnormal embryological development of the gut and is mostly found in children. When found in adults incidentally, without associated symptoms, treatment is unnecessary. However, there have been a few reported cases of symptomatic nonrotation that require specific surgical management .Cecal volvulus is an uncommon condition that can lead to substantial morbidity if not promptly diagnosed and treated. It is associated with cecal hypermobility, which occurs due to the failure of fusion between the parietal and visceral peritoneum of the right colon. This results in a loose mesentery that allows the cecum to fold upward and twist axially upon itself, leading to cecal bascule and axial cecal volvulus, respectively ,2. Regarding the coexistence of these two pathologies, it appears to be a highly unique combination. We were able to find only one case report by Pramod et al., describing a 15-year-old male with acute abdominal pain diagnosed of cecal volvulus and associated malrotation. The patient underwent a Ladd's procedure for management [In the case we present, cecal volvulus was associated with nonrotation, the latter occurs when the physiological 270° counterclockwise rotation of the gut fails. As a result, the small intestine and the DJJ remain on the right side of the midline, while the large bowel remains on the left .Diagnostic imaging, particularly abdominal radiographs and CT scans, plays a crucial role in evaluating symptomatic nonrotation. Plain abdominal radiographs may reveal nonspecific findings unless complications such as volvulus are present. In our case, the marked dilation of the cecum was clearly visible in the left hemiabdomen, along with signs of large bowel obstruction, as shown in Figure CT scans provide more detailed information, allowing for identification of the characteristic signs: abnormal position of the DJJ and inversion of the SMA and SMV, the latter being at the left ,3,4,5,7.Surgical management is the primary treatment approach for cecal volvulus, typically involving resection of the twisted bowel segment . ConversIn the reported case, the patient underwent a laparotomy and right hemicolectomy for resection of the volvulated cecum with the creation of a primary anastomosis. The successful surgical intervention resulted in the resolution of symptoms and a favorable postoperative course with no complications.Given the rarity of intestinal nonrotation and cecal volvulus, it is crucial to raise awareness among healthcare providers to facilitate early diagnosis and management. Timely intervention can significantly reduce the risk of complications such as bowel ischemia, perforation, and sepsis, which carry substantial morbidity and mortality.In conclusion, we report a rare case of a 41-year-old male patient with symptomatic intestinal nonrotation and associated cecal volvulus. This combination of pathologies is exceedingly uncommon, posing diagnostic and management challenges. Abdominal contrast-enhanced CT scan played a crucial role in recognizing the conditions and guiding the conduct. Surgical management, through laparotomy and right hemicolectomy, successfully resolved the symptoms and led to an uneventful postoperative course. Further studies are needed to enhance our understanding of optimal management strategies for these unique clinical entities.


Proteus syndrome (PS) is a rare genetic disorder usually caused by mutations in AKT1 or PTEN genes, characterized by multiple, asymmetric tissue overgrowth with high clinical variability. Sinonasal neuroendocrine carcinomas (SNEC) are exceptionally rare tumors encountered in the ethmoid sinus, nasal cavity, or maxillary sinus. We report a 35-year-old patient with PS, who underwent successful surgical removal of a well-differentiated SNEC obstructing his nasal cavity and highlight the role of the otolaryngologist for safe airway management, minimal surgical intervention and coordination of the multidisciplinary care. Histologically, focally hyperplastic mucosal epithelium of respiratory type of the nasal chamber was noticed along with seromucinous glands and capillary congestion of the subepithelial fibrovascular tissue. The limited presence of neoplastic tissue with histomorphological and immunophenotypic features of a neuroendocrine neoplasm was focally observed. Tumor cells grow in the form of islets within a vascular stroma; these neoplastic cells are immunohistochemically positive for synaptophysin, CD56, EMA, Ki67 , CD31, chromogranin and pancytokeratin AE1 / AE3 as well as for S-100 protein (weak intensity)This first description of a SNEC in a PS patient, might hint towards a common basis between the two conditions, due to the mosaic AKT1 variant and an activated AKT/PIK3CA/PTEN pathway.  PTEN hamartoma tumor syndrome (PHTS) encompasses several clinical syndromes with germline mutations in the PTEN tumor suppressor gene, including Cowden Syndrome (CS), while Proteus Syndrome (PS) is considered a subgroup of CS characterized by multiple, asymmetric tissue overgrowth with high clinical variability . SinonasA 35-year-old man with PS presented with a right nasal cavity obstruction associated with sleep apnea. A clinical examination revealed multiple abnormalities. The patient had an extensive past surgical history, including right leg amputation, inguinal hernia surgery, tonsillectomy, removal of testicular mesothelioma, mastoidectomy (brain abscess), dental extractions, and endoscopic removal of nasopharyngeal angiofibroma. A CT scan showed a protruding mass stemming from the region of the sphenopalatine foramen and localizing to the nasal cavity, maxillary sinus, and nasopharynx . The endoscopic biopsy and subsequent histologic examination revealed a malignancy with neuroendocrine features. A multidisciplinary medical team decided to treat the patient surgically. Preoperative fiberoptic evaluation revealed anatomic malformations of the epiglottis. Intubation was successfully performed with a fiberoptic bronchoscope. The tumor was resected endoscopically using the “one bleed at a time” method. Middle meatal antrostomy and ethmoidectomy were avoided due to the relatively small size of the maxillary sinus. Histologically, focally hyperplastic mucosal epithelium of the respiratory type of the nasal chamber was noticed along with seromucinous glands and capillary congestion of the subepithelial fibrovascular tissue. The limited presence of neoplastic tissue with histomorphological and immunophenotypic features of a neuroendocrine neoplasm was focally observed. Tumor cells grow in the form of islets within a vascular stroma; these neoplastic cells are immunohistochemically positive for synaptophysin, CD56, EMA, Ki67 , CD31, chromogranin and pancytokeratin AE1 / AE3 as well as for S-100 protein (weak intensity) . First comment: Neoplasms are rare and heterogeneous in PS patients. Except for lipomas, reported neoplasms include monomorphic adenoma of the parotid gland, cystadenomas of the ovary, testicular tumors, meningiomas, and mesotheliomas . PatientOn the other hand, Greidinger et al. report that there is a significant enrichment of Neuroendocrine Tumors (NETs) among PHTS patients, pointing to PI3K/mTOR pathway activation as important in the etiology of NET of the pancreas and other organs . While there is no good evidence that Proteus syndrome is caused by PTEN variants, there is a distinct and interesting phenotype designated as Type II Segmental Cowden syndrome that is caused by a mosaic of second-hit mutations in PTEN . Τhis paper demonstrates the opinion that the presence of two rare clinical entities, PS and SNEC, in the same patient may not be a coincidence, moreover, that a germline PTEN mutation may confer increased risk for NETs in addition to other tumors. While we cannot exclude a coincidental occurrence, we hypothesize that individuals with Proteus syndrome are susceptible to SNEC, due to the mosaic AKT1 variant and an activated AKT/PIK3CA/PTEN pathway. Further studies are needed to confirm this observation. Second comment: The most common symptoms of SNEC reported are nasal obstruction, facial pain and epistaxis. The proposed treatment should include surgery alone for resectable well-differentiated tumors . A meta-The patient described in this report is an adult PS patient and the removal of his SNEC will improve the quality of his life, while his life expectancy is difficult to predict. He was staged as stage III according to the AJCC (T3N0M0), and was decided to be treated only surgically, via a minimal ENT intervention, since this is the main option for typical carcinoids.Third comment: To the best of our knowledge, only ten cases of ENT surgical procedures in PS patients have been published to date including parotidectomy (two cases), thyroidectomy (one case), tonsillectomy (four cases), functional endoscopic sinus surgery (one case) and canaloplasty with tympanoplasty and mastoidectomy (two cases) -15. ThisThis paper demonstrates the opinion that the presence of two rare clinical entities, PS and SNEC, in the same patient may not be a coincidence, moreover, that a germline PTEN mutation may confer increased risk for NETs in addition to other tumors. Further studies are needed to confirm the hypothesis that individuals with Proteus syndrome are susceptible to SNEC due to the mosaic AKT1 variant and an activated AKT/PIK3CA/PTEN pathway.


Sandwich technique. This technique is safe, feasible, and associated with no short-term complications or relapses.Grynfeltt hernia (GH) is extremely rare among all abdominal wall hernias, so both diagnosis and treatment can be challenging. Surgery, open or laparoscopic, is the only definitive treatment. We present a case of a 71-year-old woman with GH , its approach, and treatment. We performed a hernioplasty with two meshes  by an open approach:  The anatomical area delimited superiorly by the 12th rib, inferiorly by the iliac crest, medially by the erector spinae muscles, and laterally by the external oblique muscle is called the lumbar region [Although it was initially mentioned by Barbette in 1672  and, up The clinical manifestation of this herniation is extremely rare . LH may Diagnosis of an LH is clinical, with evidence at palpation of swelling of the lumbar area characterized by volume increase during a Valsalva maneuver . DifferePlease see below Figure We present the case of a 71-year-old retired woman, with 69 kg and 1.56 m . Her past medical history is gastroesophageal reflux disease and her surgical history includes excision of squamous cell carcinoma on her right leg, right shoulder arthroplasty, total hysterectomy, appendicectomy, and orthopedic surgery because of proximal humerus fracture. She was medicated with omeprazole, naproxen, calcium carbonate, glucosamine, simethicone, and ginkgo biloba. In a follow-up appointment after the orthopedic surgery, she spontaneously reported swelling on the left flank, with about 1 year of evolution and progressive growth. She reported pain in that location and difficulty lying down, causing occasional discomfort. She denied a history of heavy lifting or trauma. On examination, she presented a soft swelling on the left lumbar region, bordered superiorly by the 12th rib, medially by the erector spinae muscle, and laterally by the internal oblique muscle  and subaponeurotic polypropylene meshes, separated by a muscular layer -- “Sandwich technique” . The postoperative course was uneventful. She was discharged on the second postoperative day. At follow-up fourth month, she denied any complaints and no relapse was seen on examination Figure .Technical ReportThe procedure was carried out with the patient in the ventral decubitus. A left lombar region incision (around 7 cm) was performed Figure , followeAfter dissection, the hernia sac (15 cm of longest axis) was liberated of its adhesion and isolated Figures -7. Its cThe hernia content was reduced with no difficulty. The hernia neck measured approximately 5 cm with minor muscular atrophy. A preperitoneal Ventralex® mesh was placed and its fixation was done with separated stitches of Prolene 2/0 Figures -10.Reinforcement of the hernioplasty was made with a subaponeurotic polypropylene mesh above the external oblique muscle, fixed with separated stiches of Prolene 2/0 Figure . Hemostasis was adequate. A submuscular aspiration drain was placed. Closing was made by plans and staples were used on the skin.Lumbar hernia is a rare entity, so both diagnosis and treatment can be challenging -3. CliniConfirmation of this condition can be made by US examination, but it requires a skilled radiologist in order to exclude differential diagnosis and characterize the defect . So, freThe preoperative classification and the experience of the surgeon are important factors in the management of GH and in the surgical approach . The onlThe most commonly performed technique is the open approach. It is safe, effective, and less expensive than laparoscopy. Since the first reports, different surgical techniques have been described: primary closure, fascial or gluteal flaps, and the use of meshes . HoweverAs an alternative and safe method to the open approach, the laparoscopy approach can be performed. It avoids lumbar incision allowing better esthetic results, improves the visualization of the abdominal wall defect and the relation with anatomical landmarks, allows the placement of a large mesh, and allows faster recovery, reducing postoperative pain and surgical site infection rates . HoweverThe use of meshes and the advent of laparoscopy have by far overcome the previous techniques described, although so far, no method can be recommended as preferable. There are no comparative studies between open techniques and laparoscopy for primitive LH . TreatmeGrynfeltt hernia is rare and requires a careful evaluation for the correct operative management. CT scan or MRI of the abdominal wall can be used for diagnosis and characterization. As a rare hernia, there is no gold-standard surgical treatment. As published in the literature, we showed that “sandwich repair” is a safe procedure with good outcomes.


We present a case of a 10-month-old male infant who initially presented with polyuria, polydipsia, drowsiness, and fever. Neuroimaging using non-contrast computed tomography (NCCT) demonstrated obstructive hydrocephalus associated with a suprasellar mass, for which emergency neurosurgical intervention was performed with right parietal medium pressure ventriculoperitoneal (MPVP) shunting. For fever, no cause was found with sterile cerebrospinal fluid (CSF) analysis, and empirical antibiotics were administered. The patient exhibited polyuria with hypernatremia and was diagnosed with arginine vasopressin (AVP) deficiency, further complicated by visual impairment due to left optic atrophy. Hormonal workup revealed secondary hypothyroidism and hypocortisolism. Imaging by contrast-enhanced magnetic resonance imaging (CEMR) revealed a lobulated solid-cystic suprasellar mass with flow void, suggestive of adamantinomatous craniopharyngioma initially.However, despite multiple neurosurgical interventions, the patient's condition deteriorated with recurrent fever and seizures, leading to a revision of ventriculoperitoneal shunts. Repeat CEMR showed an increase in the size of the lesion with spinal leptomeningeal metastasis, suggesting a different pathology. Transventricular biopsy confirmed an atypical teratoid and rhabdoid tumor (AT/RT), World Health Organization Classification of Tumors of the Central Nervous System (CNS WHO) grade 4, characterized by diffuse growth pattern, moderate nuclear pleomorphism, clear cytoplasm, and prominent nucleoli. Immunohistochemistry revealed positive vimentin staining and loss of integrase interactor 1 (INI1) expression, consistent with AT/RT.The patient's parents were counseled on the need for multimodal management, including surgery and chemotherapy. However, due to socioeconomic constraints and a guarded prognosis, they chose to leave against medical advice. This case illustrates the diagnostic challenges in distinguishing AT/RT from other suprasellar masses and emphasizes the importance of a multidisciplinary approach in managing complex pediatric cases. Sellar and suprasellar tumors constitute about 10% of all pediatric central nervous system (CNS) tumors. They encompass a wide range of distinct entities, having characteristic radiographic findings and histological origin .SMARCB1 tumor suppressor gene, which encodes for SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1. It is present on chromosome 22q. It is responsible for coding of integrase interactor 1 (INI1) subunit of the SWI/SNF chromatin remodeling complex, which is involved in DNA double-strand break repair and nucleotide excision repair (NER) [AT/RT is characterized by reduced expression of the ir (NER) . A revieir (NER) . A systeir (NER) . In the Here, we report a sellar suprasellar mass in a 10-month-old male, which turned out to be an AT/RT.A 10-month-old male, born out of a non-consanguineous marriage with a normal birth history, a birth weight of 3.5 kg, and normal milestones until six months of age, presented with complaints of polyuria, polydipsia, drowsiness, and fever for 15 days. Parents also noticed decreased crawling and feeding efforts. On examination, he had generalized hypotonia. Non-contrast computed tomography (NCCT) scan of the head was done, which was suggestive of obstructive hydrocephalus with a suprasellar mass. Emergency neurosurgery consultation was sought, and right parietal medium pressure ventriculoperitoneal (MPVP) shunting was done Figure .2 with a subsequent decrease in urine output and increase in urine osmolality suggestive of arginine vasopressin (AVP) deficiency. The patient had poor eye contact, and his response to confrontational stimuli was poor. The ophthalmological evaluation was suggestive of left optic atrophy with almost complete loss of vision on the right side. Repeat CSF analysis was done given a history of fever, which was normal. CSF alpha-fetoprotein (0.67 ng/mL) and human chorionic gonadotropin (HCG) (0.6 mIU/mL) were not found to be elevated. Family history was not significant. Hormonal workup was suggestive of secondary hypothyroidism and hypocortisolism, and the patient was supplemented for the same  analysis, so the patient was treated with empirical antibiotics. The patient was also analyzed for polyuria and found to have an output of 6 mL/kg/hour with serum sodium of 160 mEq/L with high serum osmolality(334 mOsm/kg) and low urine osmolality(94 mOsm/kg). He was given vasopressin 1 unit/mContrast-enhanced magnetic resonance imaging (CEMR) of the hypothalamus and pituitary area was done, which revealed a 4.3 × 4.6 × 3.8 cm lobulated, solid-cystic suprasellar mass with flow void, which was corroborated with CT and found to be internal foci of calcification. It was hyperintense on T2-weighted imaging and iso-hypointense on T1-weighted imaging with heterogeneous post-contrast enhancement Figure , 1E. GivPost-MPVP shunting, the patient again had a recurrence of fever and episodes of seizures with no improvement in symptoms. Hence, antiepileptics were stepped up, and repeat NCCT was done, which was suggestive of gross hydrocephalus with impaired function of the shunt. The patient underwent revision of the right shunt, followed by placement of another shunt on the left side as well. Post-procedure, there was a decrease in seizure frequency, but polyuria persisted, and the patient was treated with vasopressin infusion. Repeat CEMR sella was suggestive of an increase in the size of the lesion to 4.7 × 3.5 × 5.9 cm (30% increase in tumor volume as compared to baseline within one month) with spinal leptomeningeal metastasis Figure , and givThe histopathology showed a tumor arranged predominantly in a diffuse pattern with few nests separated by thin fibrovascular septa with individual tumor cells showing moderate nuclear pleomorphism, with moderate to abundant clear cytoplasm, and a prominent nucleolus Figure , 2B. BriThe patient's parents were explained regarding the need for multimodality management including surgery and chemotherapy. However, they refused further treatment because of socioeconomic constraints and guarded prognosis and left against medical advice. Till the report of the case, the patient was in stable condition.The differential diagnosis for sellar suprasellar tumors in infancy is quite vast as shown in Table Clinically and radiologically, it is challenging to differentiate AT/RT of the sellar suprasellar area from other tumors. Clinically, they present with headache, diplopia, and features of arginine vasopressin deficiency and secondary pituitary hormonal deficiencies. On imaging, they are characterized by tumors with solid, cystic, and necrotic areas. On CT, calcification is present in 50%, which was seen in our patient. On MRI, signal intensity is variable in T1- and T2-weighted imaging; however, diffusion restriction and post-contrast enhancement are typical . The indThere is no consensus on the treatment of AT/RT; however, most patients are given multimodality treatment including surgery, radiotherapy, chemotherapy, and autologous hematopoietic stem cell transplantation. However, radiotherapy is generally not offered to infants, and only surgery and chemotherapy are offered. They are associated with poor prognosis despite the above, especially when they originate at age <3 years . The cheIn conclusion, we report a rare and challenging case of a 10-month-old infant presenting with a suprasellar mass, hydrocephalus, and a constellation of endocrine abnormalities. Initially, the clinical presentation mimicked adamantinomatous craniopharyngioma, a common pediatric suprasellar tumor. However, the subsequent clinical course, including recurrent fever, seizures, and rapid tumor progression with leptomeningeal metastasis, led to the diagnosis of an atypical teratoid and rhabdoid tumor (AT/RT), a highly aggressive malignancy. This case underscores the importance of considering rare neoplastic entities, such as AT/RT, in the differential diagnosis of suprasellar masses, particularly when clinical features and radiological findings do not align with the expected course of more common tumors. Furthermore, the coexistence of endocrine dysfunction, including AVP deficiency, secondary hypothyroidism, and hypocortisolism, highlights the intricate interplay between tumor location and hormonal regulation in the hypothalamic-pituitary axis.Early recognition and prompt multidisciplinary management are crucial in cases of pediatric suprasellar tumors, especially when associated with hydrocephalus and endocrine abnormalities. While AT/RT remains a formidable diagnostic and therapeutic challenge, accurate and timely diagnosis is essential for optimizing treatment strategies and improving patient outcomes. This case serves as a reminder of the need for a comprehensive and individualized approach to managing complex pediatric endocrine and neurosurgical conditions.


Segmental absence of intestinal musculature (SAIM) is a partial defect of the intrinsic muscular layer of the intestinal tract. In this report, we describe a case of perforation of the sigmoid colon due to SAIM accompanied by vascular Ehlers–Danlos syndrome (vEDS), which was successfully treated by surgical therapy.A male in his 30 s was being followed up for vEDS diagnosed by genetic testing. He had undergone two major vascular surgeries, abdominal aortic artery revascularization and thoracic endovascular aortic repair for a residual dissection and enlarging abdominal aortic aneurysm. On postoperative day 11, the patient developed perforation of the sigmoid colon for which intraperitoneal lavage and drainage, Hartmann surgery, and transverse colostomy were performed. Histological findings showed no disturbance of blood flow or diverticulum but did show a defect in the intrinsic muscular layer around the perforation site, leading to the pathological diagnosis of SAIM and associated perforation of the sigmoid colon. Postoperatively, the patient had no complications and was discharged on postoperative day 18. The patient is being followed as an outpatient and has experienced no relapse.Both SAIM and vEDS, which may be related diseases, are associated with the presence of tissue fragility and have a high potential to cause intestinal perforation Caution should be exercised during surveillance in patients with constipation and examinations that cause increased intestinal pressure. Segmental absence of intestinal musculature (SAIM) represents a partial loss of the intestinal intrinsic muscular layer and is a common cause of intestinal stacking and perforation in newborns and low-birth-weight infants, but it is very rare in adults. Vascular Ehlers–Danlos syndrome (vEDS) is a congenital disorder caused by a genetic mutation of type III procollagen that results in abnormal connective tissue. Thus, vEDS could also be a cause of gastrointestinal perforation. There has been no report of these diseases occurring together in an adult to date. We report the first case, to our knowledge, of an adult patient with vEDS who developed perforation of the sigmoid colon due to SAIM and underwent life-saving emergency surgery.A male in his 30 s was being followed up for vEDS diagnosed by genetic testing. He suffered residual dissection of his thoracic aorta and enlargement of an abdominal aortic aneurysm and had recently undergone two major vascular surgeries, abdominal aortic revascularization and thoracic endovascular aortic repair, in the cardiovascular surgery department of our hospital. On postoperative day 7, he complained of abdominal pain, and a computed tomography (CT) scan showed free air and ascites in the upper abdomen, which led to suspicion of perforation of the upper gastrointestinal tract, and emergency surgery was performed. A hematoma was found in the retroperitoneum, but no evidence of intestinal fluid leakage was observed. Thus, we performed only hematoma removal and drainage. On postoperative day 11 after the first surgery, abdominal pain recurred, and follow-up CT showed free air in the retroperitoneum around the vascular graft and in the left colon mesentery . Considering past reports, gastrointestinal perforation due to SAIM is common in the sigmoid colon, but it can occur anywhere in the lower digestive tract. Thus, in the treatment strategy for SAIM, it is important to consider the possibility of residual SAIM lesions in the remaining colon. In our case, there were some localized defects of the muscularis propria besides that at the perforation site  results in connective tissue fragility. It is a serious type of EDS that causes rupture of arteries, the intestinal tract, and internal organs, and its frequency is estimated to be 1/50,000–1/250,000. Half of the cases are inherited in an autosomal dominant manner, and half are reported to be solitary cases due to mutations and are rarely diagnosed without symptoms and a family history [vEDS is one of the six types of EDS classified according to the causative gene. Mutation of the type III procollagen gene ( history , 11.The present patient had a sigmoid colon perforation due to SAIM and coexisting vEDS, for which there is a high risk of intestinal perforation. Only one case of intestinal perforation in a patient with vEDS and SAIM has been reported to date, that in a child . Both diThere is no established treatment for the development of intestinal perforation in either SAIM or EDS . In the Because the risk of perforation of the residual bowel is high in both diseases, these patients must be followed with caution for conditions or procedures that increase intestinal pressure. In particular, because constipation is frequently observed in patients with vEDS, it is important to follow them with good bowel control and to consider the possibility of residual SAIM lesions in the remaining colon. Besides, it is important to care post operative paralysis of intestine, because vEDS patients often are performed operation of abdominal aorta. In our case, there was retroperitoneum hematoma, which we suspect that vEDS patients’ hemorrhagic diathesis caused, and it caused paralysis of intestine and increasing of intestinal pressure. We inserted gastric tube, but it was not enough to decrease gastrointestinal pressure.We experienced an adult case of perforation of the sigmoid colon due to SAIM accompanied by vEDS, which was treated with the Hartmann procedure. It is important to keep in mind both that SAIM is one of the less common causes of gastrointestinal perforation and the tissue fragility resulting from EDS.


Intramuscular cysts are rare at the proximal calf. However, their etiology is varied, making accurate diagnosis and treatment really difficult. Ganglion cyst (GC) of the proximal tibiofibular (PTF) joint is a very rare entity with an estimated prevalence of 0.76%. Intramuscular extension of the GC arising from the PTF joint is an even rarer lesion, and only a few cases have been published in the literature. Hereby, we report an infrequent case of a GC arising from the PTF joint with a sizable pedicle and intramuscular  extension to the posterolateral aspect of the right calf. Cysts around the knee include the well-known Baker’s cysts, meniscal cysts, and cruciate ligament ganglia. Intramuscular cysts of the proximal calf are very rare, and the causes of these cysts are varied. The five types of these cysts are the following: cysts from the knee joint, intramuscular cysts arising from the muscular tissue, neurilemma, cysts from the proximal tibiofibular (PTF) joint, and pigmented villonodular synovitis (PVNS) . GanglioA 57-year-old previously healthy male presented with complaints of swelling in the right calf of five months duration. It was not initially associated with any symptoms. However, for two months, he noticed an increase in the size of the swelling and developed an aching pain over the calf, especially during walking and prolonged standing. He denied any history of trauma. Clinical examination revealed an oval cystic swelling of size 7 × 5 cm on the posterolateral aspect of the right leg over the proximal expanse of the gastrocnemius muscle. The swelling was compressible with no obvious involvement of the knee joint on physical examination. There was no evidence of associated neural or vascular compression. Routine laboratory investigations were within normal limits. The anteroposterior plain radiograph demonstrated the bulging of the lateral side of the proximal tibia Figure .An ultrasound scan revealed a cystic lesion in the posterolateral aspect of the proximal part of the right calf with extension to the PTF joint. Magnetic resonance imaging (MRI) scan was performed for accurate diagnosis of the lesion, which subsequently revealed a well-defined intramuscular T2-weighted and T2-weighted fat suppression hyperintense cystic lesion, with extension to the posterior aspect of the PTF joint with a pedicle Figures .The size of the cyst was 7.3 × 4.3 × 3 cm. Based on these findings, the diagnosis of an intramuscular cyst arising from the PTF joint was settled. Surgical excision of the lesion was decided based on our patient’s complaints of pain in the calf, especially in prolonged standing or walking. Complete excision of the cyst was done under general anesthesia and tourniquet usage in the prone position. The surgical incision was done over the lateral gastrocnemius muscle with proximal extension to the posterior aspect of the knee joint in a lazy s fashion. The lesion was found closely adherent within the fibers of the lateral head of the gastrocnemius muscle Figure , while tThe entire cyst was excised without any rupture of the wall Figure , while tHistopathological evaluation revealed flattened cells and fibrous tissue, findings consistent with ganglion. The patient had an uneventful recovery and was discharged the next day. The aesthetic result was satisfactory with a subsequent reduction of calf swelling Figure .The patient was admitted to our outpatient department for the scheduled follow-up at three, six, and 12 months after surgery, and no relapse was recognized.Intramuscular ganglion cysts arising from the PTF joint at the proximal calf are rare; nevertheless, their etiology is diverse, making accurate diagnosis and treatment really difficult . The mosIntramuscular ganglion cysts arising from the PTF joint are very rare entities. However, clinicians should include this entity in the differential diagnosis of cystic lesions around the knee joint. MRI is the gold standard for a definite diagnosis, while thorough surgical excision accompanied by the removal of the pedicle connected to the cyst to the PTF joint can reduce the chances of relapse. Based on the location of the cyst, symptoms may vary, and not rarely, it can be complicated with peroneal nerve palsy.


A spinal epidural angiolipoma is a rare, benign tumor of adipocytes and blood vessels that accounts only for a small percentage of all spinal axis tumors. We report a case of a 44-year-old male who presented with three months of progressive decreased sensation and strength from about six cm above the umbilicus down to his feet bilaterally. He presented to the emergency room when he could no longer walk. He also had neurogenic urinary retention and likely neurogenic constipation. Physical exam was notable for decreased sensation, decreased strength, and increased patellar reflexes bilaterally. MRI of the thoracic spine showed a posterior epidural mass that spanned from T2 to T3, measuring 1.2 x 1.7 x 4.3 cm, and severely compressed the spinal cord posteriorly. The patient underwent an urgent laminectomy for decompression and mass resection. Pathology was consistent with an angiolipoma. Postoperatively, he experienced a drastic improvement in strength and gross motor skills. The sensation had a partial return following surgery and continued to improve over the hospital stay. In general, the literature reports significant symptomatic improvement in patients with spinal epidural angiolipomas after surgical resection. A spinal epidural angiolipoma is a benign tumor made up of mature adipocytes and thin-walled blood vessels. It is a variant of lipomas with an extreme degree of vascular proliferation. Angiolipomas occur mostly in subcutaneous tissue and rarely in the spinal epidural region. They account for only 0.04-1.2% of all spinal axis tumors and about 2-3% of all extradural spinal tumors [Our patient is a 44-year-old male with a past medical history significant for morbid obesity (BMI of 55) and tobacco use of 10 pack-years. The patient initially presented to the emergency department with three months of recurrent falls, lower extremity weakness, and numbness that had worsened in the last two weeks. His symptoms began with numbness, which he first noticed upon waking up one morning. He described his numbness as starting at a horizontal line about six inches above his umbilicus and extending down to his feet bilaterally. This included perineal and penile numbness. Then, gradually over several weeks, he started to have lower extremity weakness and began using a cane. When he presented, he could no longer walk, and he could barely sit up in bed without assistance. He had difficulty relaxing his sphincter tone during defecation, which manifested as severe constipation. He also had worsening difficulty initiating urination. Of note, he denied any back pain and had no point tenderness on examination. He had not had any recent illness, trauma, or surgery on his back.After being admitted, his bladder scan showed a post-void residual urine volume of 1,000 ml, and a Foley catheter was inserted. On examination, he was pleasant and was not in distress over his condition. His neurological exam was significant for 2+ strength in bilateral lower extremities, most prominently in the hip girdle with abduction. There was a significant decrease in fine touch, pain, and vibration sensations bilaterally from the torso down, with worsened sensation distally. He had hyperreflexia with 3+ patellar reflex bilaterally. His cranial nerves were intact. Strength and sensation were normal in the bilateral upper extremities.An MRI of the thoracic and lumbar spine showed a homogeneously enhancing mass within the posterior epidural space, spanning from the superior endplate of T2 to the inferior plate of T3 Figures -2. This On hospital day two, the neurosurgery team took the patient for a decompressive laminectomy with mass resection and biopsy. Upon visualization during the surgery, the mass appeared largely hemorrhagic with aspects of fat. Intraoperative frozen section revealed a lipomatous/angiomatous mass without gross signs of malignancy. Post-operatively, there was immediate improvement in neurological symptoms seen on examination. Immediately after surgery, the patient endorsed a subjective 30% improved sensation from the torso down, most improved proximally. A follow-up MRI thoracic spine completed 48 hours after surgery showed findings consistent with T2-T3 laminectomy and complete removal of the epidural mass. It also showed volume loss of the dorsal left side of the spinal cord at T2, which indicated myelomalacia (Figure Following the surgery, the patient experienced drastic symptom improvement for the rest of his hospital stay. While working with physical therapy, he was able to transition from sitting on the edge of the bed to taking steps with a walker, walking across the room with a walker, and sitting in a chair. In his final physical therapy note before leaving for rehabilitation, his ODI score was calculated to be 10 points or 20%, meaning minimal disability. His severe neurogenic urinary retention resolved. His post-void residual urinary volume was within normal limits after the removal of the Foley catheter three days after the operation. His constipation, which possibly had a neurogenic component, resolved with stool softeners. He was discharged to an acute rehab facility to continue physical therapy on hospital day nine.Angiolipomas are very rarely found in the spinal canal. The cause of these tumors is not well understood. Proposed mechanisms suggest they may be derived from local pluripotent stem cells that have divergent differentiation caused by an unknown stimulus . Other pThis case report highlights the rarity and clinical significance of spinal epidural angiolipomas. These benign tumors, though uncommon, can lead to significant neurological deficits if not promptly diagnosed and managed. Through a multidisciplinary approach involving neurosurgery and radiology, our patient underwent successful surgical resection, resulting in a remarkable improvement in neurological function and quality of life. This case brings forward the importance of considering epidural angiolipomas in the differential diagnosis of spinal cord compression and the favorable outcomes achievable with timely intervention. Further research and awareness are warranted to better understand this condition and optimize its management.


Vestibular schwannomas (VSs), also known as acoustic neuromas, are benign, slow-growing tumors. If not detected early or treated appropriately, these tumors can lead to complications such as pressure on adjacent intracranial structures that can affect vital functions. The present report discusses a rare case of a residual VS in a 46-year-old female patient. The patient was a known case of left-sided VS who underwent partial excision of the tumor four years ago and had complete hearing loss on the left side since then. She reported to the clinic with progressive headaches and imbalance while walking. Magnetic resonance imaging of the brain revealed a large left residual VS compressing the brainstem and cerebellum, which was completely excised, and the patient did well postoperatively. Incomplete resection of VS carries a significant risk of tumor regrowth, necessitating the importance of complete resection with periodic follow-ups. This ne protein .The most common clinical presentation of VS is unilateral hearing loss, followed by unilateral tinnitus, ataxia, vertigo, and headache. Larger tumors are associated with abnormal tandem gait, subjective facial weakness, abnormal facial sensation on examination, and headache . ManagemThis article focuses on the regrowth of a residual VS to a large size within a short duration after initial resection in a middle-aged female patient with associated brainstem and cerebellar compression which was treated by total surgical excision.A 46-year-old female patient presented with a chief complaint of progressive headaches for the past eight months. She also complained of frequent vomiting, nasal regurgitation to fluids, change in voice, dizziness, and imbalance while walking in narrow passages and crowded areas for the past three months. She reported a history of left-sided VS for which she was operated on four years ago by left retrosigmoid suboccipital craniectomy and partial excision of the tumor was done with duraplasty using fascia. A small portion of the tumor remained on the seventh and eighth cranial nerves. The patient had complete left-sided hearing loss postoperatively. On physical examination, mild seventh, ninth, and tenth nerve paresis was present on the left side. Tandem walking was impaired. Left-sided horizontal gaze-evoked Bruns nystagmus was present. MRI of the brain (plain and contrast) of the initial tumor showed a 2.2 × 1.3 cm intensely enhancing lesion in the left cerebellopontine angle with intracanalicular extension  of the recurrent case revealed a 3.5 × 3.7 × 4 cm heterogeneously contrast-enhancing left cerebellopontine angle mass. There was no extension into the internal auditory meatus Figure .The mass compressed the brainstem and the cerebellum on the left side Figure . There wThe patient was admitted, and the nature of the disease, the need for surgical re-exploration, and the pros and cons of the surgery were explained to the patient and her relatives. After obtaining informed consent, she underwent re-exploration of the left retrosigmoid suboccipital craniectomy and total excision of the large left residual VS under general anesthesia.On gross examination, the tumor appeared well-circumscribed and encapsulated. The cut surface was yellowish-white in color. Areas of hemorrhage and cystic changes were seen, which were suggestive of degenerative changes. Histopathological sections were stained with hematoxylin and eosin and viewed at magnifications of 4×, 10×, and 40×.Microscopic features revealed a biphasic pattern. Cellular areas of streaming fascicles of spindle-shaped cells with elongated cytoplasmic processes and regular oval nuclei arranged in a palisaded or regimen pattern were observed exhibiting the characteristic Antoni type A. The spindle-shaped cells surrounded acellular, eosinophilic areas known as Verocay bodies. The adjacent microscopic tissue section demonstrated less organized, hypocellular areas with randomly distributed spindle cells interspersed within a loose, myxomatous connective tissue stroma with fibrillar collagen exhibiting the characteristic Antoni type B Figure . In addiPostoperatively, the patient experienced left seventh, ninth, and tenth cranial nerve paresis along with incomplete closure of the left eye. On discharge, the patient did well. Left ninth and tenth nerve paresis and left cerebellar signs completely improved, but the left lower motor neuron seventh nerve paresis was still present with incomplete closure of the left eye. No other deficits were observed, and the operative wound was seen to be healing well. The patient was asked to continue her medications regularly. She was advised to avoid any trauma to the operated site and avoid heavy lifting and straining. However, ongoing mobilization was encouraged to help recover the sense of balance and decrease dizziness. She was also advised facial physiotherapy and an occasional review with the ophthalmologist for the incomplete eye closure and was prescribed tear drops to avoid dryness. She was reviewed every month. Gradual improvement of left facial nerve paresis and left eye closure with every visit was observed, and there was no recurrence in the follow-up period of one year.According to Dandy, “if any neurologic surgeon were asked to name the most difficult tumor to extirpate, his answer would doubtless be the acoustic tumor” . HoweverThe Koos grading scale Table  is a freRadiosurgery is not advised for large tumors measuring >2.5 cm in diameter, brainstem dysfunction, significant mass effect, raised intracranial pressure, rapidly progressing symptomatology, and young patients (<50 years) with any tumor size. These lesions usually require surgical resection . In thisThe incidence of regrowth is high in cases of incomplete resection of VS requiring subsequent intervention, as in the present case. The risk factors for progression in VSs after incomplete resection include larger preoperative size, larger residual tumor volume, or irregular internal auditory canal expansion. Strict follow-up at shorter intervals is necessary in these patients to detect early progression .In theirOur patient had unilateral hearing loss following the initial resection. Tumor size correlates with postoperative hearing preservation. Matthies et al. reported hearing preservation of 56% for T1  tumors, 57% for T2  tumors, 44% for T3 (medium-sized tumors), and 20% for T4 tumors with brainstem compression or dislocation . MultiplFacial nerve dysfunction was the main neurologic complication following the microsurgical removal of VS . TranslaAlthough morbidity and mortality rates related to the management of VSs have improved significantly over the last few decades, radical surgical removal of large VSs continues to be challenging regarding facial nerve preservation and hearing loss. To avoid regrowth, VSs should be removed entirely whenever possible. If any tumor fragment is left behind for fear of damaging the facial or cochlear nerves, long-term surveillance is necessary with sequential clinical and MRI follow-up protocol. A well-experienced multidisciplinary team involved in the preoperative study of the imaging and clinical data, intraoperative neuromonitoring, and providing high standards of postoperative care are key factors for good prognostic outcomes in patients with large VSs.


Although laparoscopic cystectomy is a safe and effective management strategy for ovarian mature cystic teratoma (MCT) in pediatric and adolescent patients, it has been challenged because of its association with a higher risk of intraoperative spillage leading to chemical peritonitis, adhesion formation, and iatrogenic implantation of malignant cells. Here, we report a rare case of a 23-year-old female patient with MCT tissue during laparoscopic ovarian cystectomy that remained in the peritoneum, possibly becoming malignant thereafter. Intraoperatively, the cyst's contents leaked into the abdominal cavity. The abdominal cavity was thoroughly cleaned before the operation was completed. Pathological examination revealed an MCT without malignant findings. The patient's postoperative course was uneventful. Although the excised tissue was benign, the patient presented with a mass at the trocar wound (upper suprapubic area) 2 years after initial surgery. Biopsy results indicated squamous cell carcinoma. Moreover, peritoneal and bladder invasions were diagnosed. She subsequently experienced symptoms of cancerous peritonitis. Achieving a complete cure through surgery alone was deemed difficult; however, successful neoadjuvant chemotherapy and tumor reduction surgery kept her alive up until the publication of this case report, 3 years since diagnosis with squamous cell carcinoma. This case indicates that malignant transformation of MCTs can occur at any age. Laparoscopic cystectomy is a safe and effective method for managing ovarian mature cystic teratomas (MCTs) in children and adolescents , while mA 23-year-old female patient with no history of pregnancy suddenly experienced severe lower abdominal pain before going to bed, which intensified and became unbearable; she therefore visited our hospital late at night on a national holiday. A dermoid cyst was suspected following computed tomography (CT). However, the CT scan did not reveal any imaging findings indicative of malignancy Figures . BecauseTwo years after the initial surgery, the patient visited the hospital because of abdominal pain around a mass in the trocar wound (upper suprapubic area). The mass had irregular margins and was palpable. MRI revealed an indistinct mass in the 3 cm range, suspected of being either a primary tumor of the abdominal wall, carcinoma originating from a ureteric duct remnant, or malignant transformation of a teratoma attached to the peritoneum Figures . No uterAlthough carcinomas may arise from remnants of the ureteric duct, they are generally located at the bladder apex, and their histologic findings are similar to adenocarcinoma . MoreoveAdditional tests were performed to determine if radical resection of the abdominal wall around the trocar hole was feasible. Although MRI showed no utero-ovarian abnormalities, cystoscopy revealed cancerous invasion of the bladder . Positro2) plus carboplatin (CBDCA), with an area under the curve of 6, plus pembrolizumab chemotherapy (200 mg) and pembrolizumab maintenance chemotherapy. After four cycles of chemotherapy, the patient showed a partial response and was treated with pembrolizumab maintenance therapy; however, the abdominal pain worsened, and CT scan revealed that the tumor had increased in size. The treatment strategy was revised, and radiation therapy was initiated with a second-line palliative chemotherapy regimen comprising cisplatin (cis-diamminedichloroplatinum (II), CDDP) of 80 mg/m2+S-1  of 80 mg/m2 (SP3)+50 Gy radiation . Unfortunately, due to intense bone marrow suppression, only one dose of CDDP was completed, and S-1 was continued as maintenance therapy.Subsequently, the patient experienced symptoms of cancerous peritonitis and decided not to undergo radical surgery. After discussion with a multidisciplinary team of doctors, we decided to treat the patient with a first-line chemotherapy regimen comprising nab-paclitaxel (100 mg/m2) plus CBDCA, with an area under the curve of 6, was administered over four cycles. Finally, the patient was alive for 3 years with complete response after the initiation of chemotherapy. The patient provided informed consent for publication of the case data.Fortunately, chemoradiation therapy was successful, and a complete response was observed on imaging. Six months after chemotherapy was initiated, PET-CT revealed a suspected recurrent lesion on the right side of the uterus in the irradiated area, and the patient underwent reduction surgery. Total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, peritoneal dissection, and abdominal wall resection/plasty were performed. The abdominal wall could be closed as usual without requiring skin valvuloplasty. Pathology results revealed that residual invasive SCC was only detected on the right fallopian tube angle. Complete resection was achieved through surgery; however, the patient requested adjuvant chemotherapy. Therefore, CBDCA/pegylated liposomal doxorubicin (PLD) chemotherapy, comprising PLD (50 mg/mBenign ovarian cysts in women of reproductive age are common; indeed, by the age of 65 years, 1 in 25 females is admitted to the hospital for an ovarian cyst . MCTs arMalignant transformation of ovarian MCTs is a rare phenomenon that usually occurs in postmenopausal women . SCC oriGenerally, dermoid cysts > 8 cm in patients aged <30 years are prone to recurrence . MoreoveThe patient's SCC-related antigen level was within the normal threshold during the first procedure, and the excised tissue showed no evidence of malignancy. However, the SCC-related antigen level was elevated when the patient was re-examined after noting an abdominal wall mass. Although it is a matter of speculation, the tissue left behind may have undergone malignant transformation.In conclusion, although port-site metastasis could not be ruled out, we encountered what we believe to be an extremely rare case of malignant transformation of an ovarian MCT where tissue adherent to the abdominal wall trocar foramen underwent malignant transformation in a young patient. However, consensus on the ideal treatment is lacking because of its rarity. This case indicated that malignant transformation of MCTs can occur at any age. Because the preoperative diagnosis of malignant transformation of dermoid cysts in young women is difficult without abnormal imaging findings or tumor markers, other MCT cases may show distinct characteristics; therefore, management should be individualized.


Retroperitoneal ectopic pregnancy (REP) refers to abnormal implantation of the fertilized egg in the retroperitoneal cavity. REP can be divided into pelvic and abdominal positions. Extremely rare, the incidence of REP is less than 1% of ectopic pregnancy (EP). Herein, we report the first case of paraaortic-located REP in association with successful expectant management, thus raising awareness among healthcare providers, particularly in low-resource settings.A reproductive-age woman presented at our tertiary referral hospital because of amenorrhea and a positive pregnancy test. Based on serial serum β-hCG levels and imaging modalities including transabdominal ultrasound, transvaginal sonography, and magnetic resonance imaging (MRI), a REP of 7–9 weeks of gestational age adherent to abdominal paraaortic region was detected. Since the pregnancy was spontaneously arrested without clinical symptoms, expectant management was first indicated following careful evaluation. After a 1-month follow-up, the ectopic mass naturally degenerated without complications and her β-hCG concentration returned to a negative value. Therefore, the patient recovered completely and avoided unnecessary surgery as well as toxicity of medical treatment when using systemic methotrexate.In addition to transvaginal and transabdominal ultrasound, MRI is necessary for the diagnosis of nonviable REP. Alongside the great vessels in the abdominal cavity should be taken into consideration in all suspected cases relating to this rare entity. Expectant management may be carefully indicated in conditions of nonviable REP and unruptured REP, where applicable. Accompanying transvaginal ultrasound and transabdominal ultrasonic scan and MRI are necessary for a high index of suspicion of REP.Alongside the great vessels in abdominal cavity should not be neglected in suspected case related to this rare entity.Expectant management may be carefully indicated in conditions of absence of fetal cardiac activity and nonruptured REP, where applicable. Retroperitoneal ectopic pregnancy (REP) refers to the implantation of a fertilized egg in the retroperitoneal cavity. REP can be divided into pelvic and abdominal positions . The locRegarding clinical characteristics, symptoms can range from asymptomatic to severe manifestation associated with a ruptured EP which leads to immediate hypovolemic shock and death , 10. SimTreatment includes surgical intervention, medical treatment with methotrexate, and expectant management . In someHerein, we hereby describe an uncommon case of REP at our tertiary referral hospital and review the literature Table . To our A 38-year-old female patient (G5P2) was transferred to our hospital owing to suspicion of abdominal ectopic pregnancy. Her obstetric anamnesis included one vaginal birth, one cesarean delivery, one ectopic pregnancy with salpingectomy, and one preterm birth at 22 weeks GA. She had also undergone an appendectomy in the past year.On admission, the patient was stable. A general physical examination result was unremarkable. On gynecological examination, the uterus and bilateral ovaries were normal in size. The patient was asymptomatic and denied the use of urgent contraceptive pills. She complained of a retarded menstrual cycle of two weeks and her urine human chorionic gonadotropin (HCG) test was positive. Laboratory tests revealed the positive pregnancy tests. The quantitative serum beta-human chorionic gonadotropin (β-hCG) titer test was 51,586 m-international units per milliliter (mIU/mL).Abdominal ultrasound showed no intrauterine gestational sac, with an endometrial thickness of 6 mm, and both adnexal structures were normal. Several fibroid nodules approximate 8–11 mm in diameter were detected within the intramural layer. After observing the lateral sides of the uterus and pelvis, the abdominal cavity was carefully scanned. An abnormal heterogeneous mass was found in the retroperitoneal cavity adjacent to the abdominal aorta, corresponding to a gestational sac at 7–9 weeks with a visible embryo, but absence of fetal cardiac activity Fig. .Upon monitoring, the β-hCG concentration gradually decreased every 48 h Fig. . After cDespite the lack of criteria for laparoscopic imaging and pathologic examination, the retroperitoneal mass reduced in size and the β-hCG level returned to the normal range after 1 month, and she continued to be stable after 3 months. This regression supported the confirmative diagnosis. The patient was thankful to the team for successful management without surgical or medical intervention.In the present case, the patient had a retroperitoneal ectopic pregnancy with natural conception. This may be a rare occurrence since almost all cases have been reported relative to IVF treatment –18. RetrAccordingly, since the EP was hidden in the retroperitoneum, thus initial detection was relatively difficult. Therefore, it is necessary to rule out the possibility of EP and determine its location, especially alongside the large vessels . PreviouGenerally, REP is a life-threatening condition because the gestational sac is located next to the fragile structures. Therefore, the trophoblastic tissue directly invades the neighboring organs. Consequently, it is very difficult to completely assess and remove REP mass because it is covered by the peritoneum. Hence, the placental invasion commonly results in adverse outcomes such as massive hemorrhage . Owing tIn the present case, the early pregnancy failure occurred spontaneously before hospitalization. The etiology of early fetal death may be caused by implantation in area unfavorable for fetal development. Therefore, the gestational sac size was naturally limited. In our patient, the β-hCG concentration gradually decreased. Furthermore, the clinical manifestations were absent with stable hemodynamic parameters. These advantages have contributed to the success of expectant management. The limitation of the non-surgical intervention was noted that our team could not assess the REP tissue for histopathological examination and exclude other malignant pathologies. However, since no sign of proliferative vascularity was detected on ultrasound, the mass size was gradually decreased, and the serum β-hCG levels returned to negative value. All these progressions supported for the benign pathology of ectopic pregnancy.Similar to almost all ectopic pregnancies, treatment should be individualized and based on gestational age, gestational sac size, relative organs, clinical characteristics, β-hCG level, presence of fetal cardiac activity, and desire of the patient Table . AlthougIn summary, an importance of considering the possibility of REP should be emphasized in suspected cases of abdominal ectopic pregnancy with unknown location. In addition, expectant management can be considered in the case of nonviable pregnancy without complications, and multidisciplinary team could be immediately assessed.


Kissing nevus is a congenital melanocytic neoplasm arising in those parts of the body that split at some point during embryological development , resulting in two adjacent melanocytic nevi. To date, 23 cases of kissing nevus of the penis have been described, and dermatoscopic and histological findings are available in 4/23 cases. We report a dermatoscopic, histological and confocal microscopic analysis in a new case of the kissing nevus of the penis in a 57 years old man. Dermatoscopic analysis showed large globules in the central area and a peripheral pigment network; the histological examination confirmed the presence of an intradermal melanocytic nevus with minimal junctional component and congenital features. Moreover, we reported, for the first time, confocal microscopy findings in the kissing nevus of the penis, revealing the presence of dendritic cells in correspondence with the epidermis and suggesting a state of cellular activity. Considering the clinicopathological features of the lesion, a conservative approach was adopted, and a clinical follow-up was planned after six months. Kissing (or divided) nevus is a peculiar congenital melanocytic neoplasm arising in those parts of the body that split at some point during embryological development, resulting in two adjacent melanocytic nevi. The first description of this phenomenon was by Von Michael in 1908 in the eyelid, and Fuchs , dividedThe mechanism responsible for the formation of kissing nevus of the eyelids can be explained in relation to the embryologic formation of these structures. The eyelids start to form at weeks 5~6 of gestation and fuse at weeks 8 to 9 of gestation, then split during the 24th week of gestation . TherefoA similar mechanism has been proposed for the kissing nevus of the penis ,7. IndeeWe herewith report a new case of the kissing nevus of the penis, describing for the first time the dermatoscopic and histological features and confocal microscopic findings.A 57-year-old man presented for a dermatological examination of two adjacent pigmented lesions on the glands and the inner foreskin that had been present for a long time, growing slowly during the years A. The twConfocal microscopy demonstrated the presence of various dendritic cells in correspondence with the epidermis, which is indicative of the state of cellular activity .Two incisional biopsies were performed in both involved anatomical sites in order to exclude malignancy and to consider a conservative treatment. The biopsies were fixed in formalin and embedded in paraffin, following standard protocols. Paraffin sections were cut at 5 µm using a microtome LEICA SM 2000 R , dewaxed in xylene, rehydrated through a series of graded ethanol solutions and stained with Gill’s Hematoxylin and Eosin . Immunohistochemistry was executed on an automated immunostainer , as previously described ,24. The The histological examination of the samples revealed similar microscopic features in both samples, showing an intradermal melanocytic proliferation with congenital features and a minimal junctional component. Melanocytes were arranged in nests in the upper dermis while splaying between dermal collagen bundles in the reticular dermis. Stromal melanin deposits were more abundant in the upper part of the lesion, as were dermal melanophages. Melanocytic aggregates protruding within a vascular channel with an empty and dilated lumen lined by flattened endothelial cells were also observed . ImmunohThe kissing (or divided) nevus of the penis is a rare entity, with only 23 cases reported. The age at diagnosis varies from 3 to 30 years old ,19. ThesThe dermoscopic examination of this variant of melanocytic nevus generally revealed a globular or composite pattern (globular-reticular pattern or pigment network at the periphery and homogeneous pattern with some globules in the center) ,19,20,21Microscopically, kissing nevi are compound or intradermal melanocytic neoplasms that are generally associated with numerous intradermal melanophages ,17,19,21We described a new case of the kissing nevus of the penis in a 57-year-old man. Dermoscopic analysis, in our case, displayed the characteristic pattern of a compound melanocytic nevus with large globules in the central area and a peripheral pigment network. This pattern was consistent with the previously reported dermoscopic description of kissing nevus of the penis ,19,20,21The diagnosis of compound melanocytic nevus was confirmed by histological examination. The two incisional biopsies performed on both involved anatomical sites revealed an intradermal melanocytic proliferation with congenital features and a minimal junctional component. In the upper part of the lesion, we observed abundant stromal melanin deposits together with scattered dermal melanophages. Similar findings were observed in previously reported cases of the kissing nevus of the penis ,15. InteMoreover, we reported, for the first time, confocal microscopy findings in a kissing nevus of the penis; the analysis revealed the presence of several intra-epidermal dendritic cells, suggesting a state of cellular activity. An increased number of epidermal dendritic cells was proposed as an independent risk factor for melanoma and can represent a diagnostic pitfall in the kissing nevus of the penis, although further data are needed to confirm this finding .The great majority of the kissing nevi of the penis described are benign lesions. For cosmetic reasons, surgical excision and reconstruction by skin grafting using remnant foreskin have been performed with satisfactory results . To date


Colorectal cancer (CRC) can occasionally coexist with diverticulitis, thereby complicating diagnosis and treatment. In cases of refractory diverticulitis, it is important to consider the possibility of malignancy and determine appropriate treatment strategies. An 85-year-old male presented with lower left abdominal pain; he was admitted for further examination and the treatment of suspected sigmoid diverticulitis. On examination, a firm mass was palpated in the lower left quadrant. Imaging revealed sigmoid diverticulitis, partial abscess formation, and the involvement of the small bowel and abdominal wall. Although malignancy was suspected, a definitive diagnosis was not made. Because of the refractory nature of the disease, early surgical intervention, sigmoid colectomy, partial small bowel resection, abdominal wall resection, and lymph node dissection, was performed in accordance with the malignancy protocol. Pathologic diagnosis revealed adenocarcinoma within the diverticulitis with negative resection margins, indicating curative surgery. The low preoperative diagnostic rate of CRC associated with diverticulitis highlights the need for vigilance. Refractory diverticulitis may indicate the presence of concealed malignancy requiring surgical intervention. In the management of refractory diverticulitis, it is important to consider the potential coexistence of cancer. Even if extensive investigations are performed and a definitive diagnosis remains elusive, surgery must be considered. Colorectal cancer (CRC) is sporadically encountered along with diverticulitis. In Western countries, there is an increased risk of CRC in patients with diverticulitis . AdditioHowever, the diagnosis of coexisting CRC at the onset of diverticulitis is challenging. Imaging studies often yield analogous findings for malignancy and diverticulitis, hindering their differentiation . Thus, wWe report the case of a patient in whom diverticulitis management was problematic and who had suspected concomitant CRC. The condition was completely resolved after prompt surgical intervention.An 85-year-old male presented to a local clinic with lower left abdominal pain. Based on the suspicion of diverticulitis, oral antibiotic treatment was initiated. However, the patient’s symptoms did not improve, and he was admitted to our hospital for further evaluation and treatment. The patient had a history of diabetes mellitus. On admission, a physical examination revealed a body temperature of 38.3℃ and a palpable, firm mass in the lower left abdomen. Laboratory tests revealed an elevated white blood cell count  and C-reactive protein level (10.4 mg/dL). Tumor marker levels were within normal limits. Abdominal computed tomography showed multiple diverticula in the sigmoid colon with circumferential wall thickening, as well as contrast enhancement and increased density in the surrounding adipose tissue. The wall structure was intact, but an abscess with air was present adjacent to the small bowel and abdominal wall Figure , 1B. A cBased on the above findings, the patient was diagnosed with sigmoid diverticulitis with abscess. Despite fasting and conservative treatment involving antibiotics, the inflammation recurred. Abdominal computed tomography showed no improvement in the diverticulitis, and conservative treatment was considered ineffective. A surgical approach was used, based on the possibility of CRC indicated by the palpable mass. During surgery, the sigmoid colon displayed adherence to a portion of the small bowel and abdominal wall. Sigmoid colectomy, partial small bowel resection, combined abdominal wall resection, and standard local lymph node dissection were performed in accordance with recommendations for the surgical treatment of CRC. The gross examination of the resected specimen revealed 10 cm circumferential wall thickening and sclerosis. The most constricted portion of the bowel exhibited significant bowing, which hindered the visualization of the mucosa, but no palpable tumor was apparent.Histopathologic examination revealed that the most constricted portion of the sigmoid colon had overlapping and folded intestinal loops that had fused because of inflammation Figure , 2B. WitThe one-year incidence of CRC in patients with diverticulitis is 1.9% , and divReports of CRC arising from diverticular mucosa are scarce, presumably because of the difficulty in confirming colonic diverticular mucosa-related carcinogenesis based on pathologic findings. CRC arising within diverticula tends to be more advanced because the lack of a muscular layer facilitates serosal penetration . MechaniThe preoperative diagnostic rate of CRC associated with diverticulitis is strikingly low, at 37% . This loIn cases where diverticulitis does not improve with conservative treatment, concealed cancer is a possibility. Several predictive factors for diverticulitis have been reported ; howeverInterval surgery is considered beneficial in the management of diverticulitis . HoweverThe diagnosis and treatment of colorectal cancer associated with diverticulitis are complex and challenging. In refractory diverticulitis with abscess, it is important to consider the possibility of concealed malignancy. However, preoperative diagnosis is typically difficult; thus, it is important to suspect cancer in older patients with diverticulitis and select treatment strategies accordingly.


Dear Editor,Late Onset Focal Dermal Elastosis (LOFDE) is a rare, acquired cutaneous entity. Clinically, it usually presents as multiple, small, flat, white to pale yellow papules located on the sides of the neck and flexural areas.Our patient is a 57‐year‐old woman presenting with multiple, asymptomatic, unilateral yellowish papules located predominantly on the skin of the right lower extremity and groin. Single lesions were noticed on the right part of the abdomen Figure . The accOn dermatoscopy  white globules, corresponding to the accumulation of the connective tissue, presumably elastic tissue pushing collagen bundles were noticeable Figure . They weTo our knowledge, this is one of the very few described cases of late‐onset focal dermal elastosis, and the first one analyzed with non‐invasive skin imaging techniques such as dermatoscopy and high‐frequency ultrasound. A review of the available LOFDE cases in the literature is presented in Table Considering the fact that the pathogenesis of LOFDE has not been determined, it might be an underreported entity due to its peculiar clinical presentation. LOFDE papules clinically strongly mimic pseudoxanthoma elasticum (PXE) as well as the fibrotic phase of guttate morphea. In dermatoscopy, PXE reveals yellow‐to‐white clods on a reddish or whitish background, along with linear, irregular vessels,In conclusion, taking into account the clinical picture of LOFDE, it seems that both HFUS and dermoscopy may be helpful in the diagnosis of this rare entity, but in doubtful cases, histologic examination and histochemical staining will determine the cause of decreased hyperechogenicity of collagen fibers.No potential conflict of interest was reported by the authors.


Acute pancreatitis (AP) is increasingly rising globally, especially among elderly populations. In many cases, AP can progress to chronic pancreatitis (CP) and cause damage to the pancreas. Common causes of AP include gallstones and alcoholic injury, but periampullary diverticula (PAD) have emerged as a complex etiology. PADs are rare bowel-filled outpouchings located near the main papilla or common bile duct (CBD). In this study, we present a 66-year-old female with recurrent pancreatitis that is caused by a PAD. Due to the paucity of information regarding the management of PADs, we hope this case highlights the need to advance treatment options in this area. Acute pancreatitis (AP), or acute inflammation of the pancreas, has over 275,000 cases worldwide annually . Its incPADs are defined by luminal mucosal outpouchings of the duodenum that either originate near or contain the major/main duodenal papilla (ampulla of Vater) or the intramural section of the common bile duct (CBD) . PADs coA 66-year-old Hispanic female presented with a clinical history of hypertension, hypersensitivity lung disease, diabetes mellitus type 2, deep vein thrombosis (DVT), and two prior episodes of pancreatitis with severe upper abdominal pain and increased urinary frequency. She denied hematochezia, hematuria, chest pain, shortness of breath, dysuria, and any changes in bowel habits. She was afebrile with a blood pressure of 137/85 mm Hg, heart rate of 104 bpm, and respiratory rate of 18/minute. Surgical history was significant for right total knee arthroplasty, hysterectomy, and oophorectomy. Laboratory work was significant for elevated aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), lipase, bilirubin, and glucose with decreased hemoglobin and hematocrit  showed a mildly dilated proximal CBD measuring up to 1 cm in diameter. There was no evidence of choledocholithiasis in the proximal or mid-CBD. Mild prominence of intrahepatic biliary ducts was present, but no focal mass was identified in the neck, body, or tail of the pancreas. Abdominal ultrasound displayed an unremarkable right upper quadrant.Endoscopic retrograde cholangiopancreatography (ERCP) was then attempted, and it was discovered that the major papilla was located entirely within a diverticulum and inverted inside. Multiple attempts to cannulate and readjust the direction of the major papilla were unsuccessful using the ERCP scope and esophagogastroduodenoscopy scope or by clipping the edge of the diverticulum. After the ERCP procedure, the patient was returned to the hospital ward for ongoing care, which included intravenous fluid hydration, a clear liquid diet, laxatives , pantoprazole, and heparin for DVT prophylaxis. The patient left the hospital against medical advice before a repeat ERCP could be attempted. She later reported that her symptoms spontaneously resolved.Six weeks later, the patient was readmitted with similar symptoms. Ultrasound showed no evidence of gallstones but showed circumferential gallbladder thickening, for which she subsequently underwent a laparoscopic cholecystectomy. This provided a resolution to further AP episodes. Given that the patient's elevated liver function tests (LFTs) and bilirubin improved concordantly with AP symptom resolution during both admissions, an obstructive etiology of pancreatitis ) was presumed.PADs have taken on more significance in the field of clinical gastroenterology, with their increased incidence likely attributable to the rising life expectancy worldwide . While mCurrently, elective surgical treatment of asymptomatic or minimally symptomatic diverticula is not recommended, especially if the diverticula are found in the second part of the duodenum due to their retroperitoneal nature . SymptomERCPs for PADs are extremely difficult due to the risk of perforating the diverticulum and hemorrhage, and the success rates in previous studies are variable. The combination of ERCP paired with percutaneous transhepatic cholangiogram (TC) has shown greater efficacy in patients who are poor candidates for conventional surgical treatments. Developments of duodenoscopes with large operating channels and the "tuteur transcystique-transpappilaire" technique have demonstrated potential success for entering the duodenum without perforation; however, studies on these procedures are extremely limited . AdditioThere are a range of purported mechanisms by which PADs are implicated in the pathogenesis of acute, recurrent, and chronic pancreatitis. PADs at or near the ampulla of Vater can directly obstruct biliary passage. Additionally, PADs are associated with a higher occurrence and recurrence of bile duct stones , and PADPeriampullary diverticula are extremely rare luminal outpouchings that are associated with severe complications such as abdominal pain and acute/chronic pancreatitis, as seen in this patient. There is a need for well-informed, interdisciplinary guidelines for endoscopists, surgeons, and interventional radiologists for the diagnosis of PADs  and the management of symptomatic PADs.


To report the spontaneous regression of an isolated retinal astrocytic hamartoma in a newborn. During the seven-month follow-up duration, fundus photography and fluorescein angiography examinations were performed.An isolated retinal astrocytic hamartoma was detected in the nasal retina of the left eye of a 4-day-old male infant. At the time of initial presentation, we detected a solitary yellowish-white flat mass with an approximate size of 1.5 disc diameters in the nasal retina. Fluorescein angiography (FA) revealed a diffuse hyperfluorescence with slight fluorescence leakage. Seven months later, the fundus examination showed no lesion in the left eye, FA revealed mild tortuous vessels without leakage.In the present case, we established that the isolated retinal astrocytic hamartoma in this infant has underwent spontaneous regression. This case can point out that follow –up reexaminations are advisable for a solitary yellowish-white flat mass of the fundus in a newborn.The online version contains supplementary material available at 10.1186/s12886-023-03135-5. Retina astrocytic hamartoma (RAH) is a rare benign glial tumor arising from a retinal nerve fiber layer. It is the most common ophthalmoscopic finding in patients with tuberous sclerosis complex (TSC). RAH may also rarely occur in patients with neurofibromatosis type 1 (NF1) or as isolated cases [A 4-day-old male infant, who was born at 40 weeks’ gestational age and weighing 3660 g. He was delivered at term by episiotomy combined with forceps and admitted to the Department of Neonatology of The Second Hospital of Jilin University due to dyspnea in February 2022. No cutaneous lesions were observed. The patient underwent brain magnetic resonance imaging (MRI), the results of which showed a short T1 strip signal shadow (hemorrhage) under the cranial plate of the occipital region on both sides. He had no family history of diseases.The right eye appeared normal in the routine retinal screening performed with RetCam 3 . However, a color fundus photograph of the left eye revealed a solitary yellowish-white flat mass with an approximate size of 1.5-disc diameters located in the nasal retina (Fig. Retinal astrocytic hamartoma represents the most common ophthalmoscopic finding in patients with TSC. Based on two relatively large cohorts of patients with TSC, this tumor was detected in 34–36% of patients and, when present, it served as a marker for systemic tumors . ShieldsBased on its ophthalmoscopic appearance, RAH is categorized into three types . Type 1 The differential diagnosis of this particular lesion is retinoblastoma in an infant. In a larger series of 604 cases of pseudoretinoblastoma, 15 cases were with diagnoses of RAH. Of them, three were of children who were one year of age or younger in . AccordiBased on findings on fundus and fluorescein angiography images, and the process of regression, isolated RAH was a reasonable diagnosis in this patient. This case is a rare example of spontaneous regression of RAH in a newborn. This case can point out that follow –up reexaminations are advisable for a solitary yellowish-white flat mass of the fundus in a newborn.Below is the link to the electronic supplementary material.Supplementary Material 1


We report an 81-year-old patient who underwent microsurgical resection of a posterior fossa mass lesion. Intraoperative findings were suggestive of the presence of two distinctly different tumor types within the lesion, one of which was well-circumscribed and avascular, whereas the other one showed an adhesive growth pattern and extensive vascularisation. Histopathological analysis, including deoxyribonucleic acid (DNA)-methylation-based classification, substantiated the intraoperative impression and confirmed the presence of a subependymoma central nervous system (CNS) World Health Organization (WHO) grade 1 as well as the presence of a hemangioblastoma CNS WHO grade 1. To our knowledge, our patient represents only the second reported case of such a rare constellation. Even though DNA-methylation-based classification is not yet required for the classification of all CNS tumor types by the 2021 WHO classification of tumors of the CNS, it proved to be crucial to verify the final diagnosis in our patient. In the future, DNA-methylation analysis will most likely become an important asset in neuro-oncological diagnostics and further help to guide treatment strategies in complex or rare clinical cases. Posterior fossa tumors comprise approximately 15%-20% of all intracranial tumors in adults, with hemangioblastoma being the most common primary tumor type . HistopaEven though DNA-methylation-based classification of neurooncological neoplasms is not required for all tumor types by the 2021 World Health Organization (WHO) classification of tumors of the central nervous system (CNS), it has become an increasingly important tool in neuro-oncological diagnostics and consequent guidance on patient treatment .Here, we report a male adult patient who underwent neurosurgical resection of a posterior fossa mass lesion that intraoperatively displayed two surgically very distinct characteristics. Subsequent histopathological work-up, including DNA-methylation analyses, revealed a subependymoma as well as a hemangioblastoma, both corresponding to a WHO CNS grade 1, hereby confirming this very rare constellation of coexistence of two tumor entities within the same mass lesion. We provide detailed data on the performed surgery as well as the neuroradiological and neuropathological results. Written consent for publication of the case report was obtained.An 81-year-old male patient was admitted to the trauma unit of a local hospital after suffering a conservatively treated pelvic fracture due to a fall. Because of a self-reported involuntary weight loss of 15 kg and declining general health within the last two months, cranial computed tomography (cCT) was performed, revealing a mass lesion in the fourth ventricle. A subsequent magnetic resonance imaging (MRI) showed a contrast-enhancing, partly cystic tumor  was used to minimize brainstem manipulation. In hindsight, IOM should have been already used for the initial surgery to reduce the risk of surgery-associated neurologic deterioration.Histopathology and molecular genetic analysisNeuropathological analysis of the tissue obtained in the first surgery showed a glial tumor with clusters of small, monomorphic nuclei that were embedded in a loose fibrillary matrix in hematoxylin and eosin (H&E) stains Figure . No mitoNeuropathological analysis of the tissue obtained in the second surgery showed large areas with hemorrhage and well-vascularized tissue in H&E stains Figure . FurtherThus, histopathological examinations revealed two distinct tumor types within the resected mass lesion, a subependymoma CNS WHO grade 1 and a hemangioblastoma CNS WHO grade 1. Due to the histopathological results and postoperative MRI confirming gross total resection, no further adjuvant treatment was deemed necessary.We believe this case provides several interesting aspects of surgery and intraoperative findings, as well as neuroradiological and neuropathological diagnostics. A preoperative cCT and cranial MRI scan showed an intraventricular mass lesion in the posterior fossa. Based only on the preoperative imaging, a definitive suspected diagnosis could not be obtained. Due to the intraoperative ambiguous findings, with one tumor being avascular and well-circumscribed while the other one being extensively vascularized and adherent, the possibility of two completely different tumor types was already discussed between the operating surgeons. This presumption was eventually confirmed by neuropathological examination via DNA-methylation profiling. Our case represents the second reported patient with such a rare histological constellation with the coexistence of a subependymoma and a hemangioblastoma within one lesion. To the best of our knowledge, only one similar case has been reported thus far, dating back to 1985, and the incidence of such histopathological constellations remains unclear ,9. DNA-mAlthough DNA methylation-based analysis is not being implemented in the WHO grading of all CNS tumors ,9, we beDuring the initial tumor resection, we encountered severe difficulties due to the highly vascularized nature of the lesion and the patient's cardiovascular instability. The hemodynamic instability was most likely caused by manipulations of the area of the fourth ventricle during the attempted tumor resection leading to recurrent bradycardia, hypotension, and once a brief episode of asystole. After consulting with the anesthesiologist, the surgery was terminated, and a second surgery was planned after the stabilization of the patient the next days. In hindsight, IOM, as used in the second surgery, may have been helpful in the earlier detection of these complications.The coexistence of two distinct tumor types within one lesion is a very rare but possible phenomenon. This may pose special challenges to neurosurgeons if not identified in preoperative imaging. In this complex constellation, DNA-methylation profiling may be crucial to determine the eventual histopathological diagnosis and substantiate intraoperative findings allowing for optimal patient treatment.


Hydrometrocolpos is a rare congenital anomaly characterized by gross distension of the uterus and vagina with fluid, which may result in obstruction to the urine flow. The insertion of Foley catheter into the uterus can relieve the obstruction to the flow of urine and improve renal function. Herein we present a case of infant who was diagnosed with an abdominal mass and renal insufficiency that was managed by the placement of Foley catheter into the dilated uterus and the renal function recovered. Hydrometrocolpos is the accumulation of secretions in both the vaginal and uterine cavity. It is a rare condition with an incidence of around 0.006% per year in full-term babies , 2. It iA 4-month-old female infant, with a month history of gradual onset of abdominal distension that was associated with on/off vomiting, intermittent low-grade fevers, crying during both urination and defecation as she passed small amount of urine and hard stool. Other systems, prenatal, natal and postnatal history were unremarkable. She was febrile, temperature 39.9 °C, pale, pulse rate 102 b/min, saturating at 98% in room air and respiratory rate of 38 b/min. On examination of the abdomen, there was a smooth non-tender mass extending to the right upper and left lower quadrants. The mass was non-pulsatile with limited mobility, which could not go below its inferior margin. Bowel sounds were heard normally, and other systems were unremarkable.9 /L, with neutrophil predominance, platelets were 130 × 109/L−1, serum potassium was elevated at 5.62 mmol/L, sodium 137 mmol/L, urea −26 mmol/L, serum creatinine −406 micromoles/L. The urinalysis revealed protein +, leucocytes ++, Blood +++, WBC-10-15/HPF, RBC-1-5/HPF, bacteria +++. The urine culture grew Escheria Coli that was sensitive to ceftriaxone, cefepime, ceftazidime, ciprofloxacin, cefotaxime gentamicin, amikacin and meropenem, and resistant to amoxylin/clavulanic acid ampicillin and trimethoprim/sulfamethoxazole.Provisional diagnoses of infected large abdominal pelvic teratoma with a differential diagnosis of sascrococygeal teratoma, urachal mass and distended bladder were entertained. The full blood picture revealed haemoglobin of −8.5 g/dl, leukocytosis 16.79 × 10Abdominal X-RAY both supine and erect revealed a soft tissue mass involving the lower quadrant of the abdomen. However, there was no sign of intestinal obstruction. Abdominopelvic ultrasound showed a large, well-defined, anechoic intra-abdominal mass measuring 12 cm × 6 cm, bilateral moderate hydronephrosis and a thick-walled urinary bladder. The uterus could not be appreciated but ovaries were normal. Computed tomography (CT) revealed a centrally located hypodense cystic mass arising from the uterus connected to the upper vagina, located between the urinary bladder and rectum, measuring 7.6 cm × 3.5 cm × 4.6 cm. The mass was extending to the upper abdomen, abutting the rectum and pushing the urinary bladder to the right causing bilateral moderate hydroureteronephrosis .The infant was treated with ceftriaxone for 5 days and fever subsided. A urethral catheter was inserted to monitor urine output and making sure adequate bladder emptying, transfused with 2 units of blood and then planned for cystoscopy and examination under anaesthesia (EUA). During EUA, the anus was displaced more anteriorly, separated from the introitus by a very thin membrane , the extHydrometrocolpos is a rare congenital condition with an incidence of about 0.006% . It can Hydrometrocolpos can be isolated or associated with syndromes such as the Mckusick Kaufman syndrome, Mayer–Rokitansky–Küste–Hauser syndrome and Herlyn–Werner–Wundelich syndrome , 7. SomeHydrometrocolpos is characterised by distension of the vagina and uterus as a result of accumulation of fluid due to distal obstruction presenting as a cystic abdominal mass , 5, 7. TAs definitive reconstructive surgery may be complex and typically postponed until the infant is 2-year old, a temporary solution to relieve the obstructed urinary tract is paramount. The renal function of our patient recovered quickly following placement of the Foley catheter into the distended uterus. A catheter/tube placed into the uterus/vagina is effective in draining the accumulated fluid in the uterus and relieving obstructed urinary tract , 5. The Hydrometrocolpos is a rare congenital anomaly that should be considered in the differential diagnosis of an abdominal mass in female infant. It can be associated with obstructive uropathy that can be managed by insertion of Foley catheter into the uterus, a simple life-saving procedure.All listed authors declare that there is no conflict of interest related to publication of this work.None.


To the Editor,Mediastinal lymphomas can be challenging to manage for several reasons, including the acuity of presentation and the difficulties to secure a biopsy for appropriate classification of the disease and management thereafter. The mediastinum is a narrow area where vital structures are located or pass-through. Frequently, physicians are faced with tumors restricted to that area. Patients typically have nonspecific manifestations resulting from compression of surrounding structures. Often, patients are sick and quick tissue diagnosis is needed to start therapy. However, the area is not easily accessible, and sampling is frequently suboptimal which makes the establishment of an accurate diagnosis challenging. Lymphomas are the most common primary malignancy involving the mediastinum . Biopsy A healthy 17-year-old girl presented with 4-weeks of shoulder pain, cough, and shortness of breath. Chest-x-ray showed mediastinal mass, which was later confirmed by PET/CT-scan , primary mediastinal B-cell lymphoma (PMBL) and mediastinal gray-zone lymphoma (MGZL) arise from the thymic B-cells, and are the most commonly encountered mediastinal-lymphomas. Clinically, these are not distinguishable, as they all present with compressive symptoms and predominantly affect young female patients  . HistoloWe herein report a case that is not uncommon in clinical practice, where a patient presents with symptoms that require quick intervention, while the location of the tumor is difficult to reach and securing a representative biopsy is challenging. Our patient had CD20/CD30-positive tumor. CHL, PMBL and MGZL can have this phenotype. While CD20 can be positive in CHL, this is usually rare; additionally the acute presentation in this patient is atypical of CHL, where the disease progresses at a slower pace , 6. The Despite the tremendous advances in lymphoma management, some cases still fall into a grey-zone. Ours illustrates the importance of integrating the biological characteristics of the tumors with the response to prior therapy in order to plan the next line of treatment. It also highlights the importance and the need to divert from the standard conditioning and transplant platform for such cases. Multidisciplinary discussion and shared decision-making by patients and doctors in the context of the available therapies help to navigate the options and choose the appropriate strategy for a successful outcome.