Branchial cleft cysts are congenital diseases of the neck caused by abnormal embryonic development of the first to fourth branchial clefts. Most branchial cleft cysts are found in the head and neck, but branchial cleft cysts arising in posterior mediastinum are rarely reported. We report a 44-year-old Chinese man who was found to have a right-posterior mediastinal mass on chest computed tomography (CT) during a physical examination. The size of the mass was about 30.6 mm * 25.1 mm and enhanced CT of the chest showed an occupying lesion in the right parietal esophagus of the upper-posterior mediastinum with no significant enhancement. The patient was considered to have a neurogenic tumor with cystic change and underwent posterior mediastinal tumor resection. Postoperatively, pathological examination confirmed the final diagnosis of bronchial cleft cyst. The patient was discharged on the 7th day after surgery. One year postsurgery, no obvious recurrence was found in reexamination. Clinically, the second branchial cleft cyst is more prevalent, accounting for approximately 90% of all branchial cleft cysts , followeA 44-year-old Chinese man was hospitalized due to a posterior mediastinal mass found on physical examination, without dyspnea, dysphagia, repeated fever and other discomforts. Chest CT showed that the right side of the upper and posterior mediastinum could be seen as a round and slightly low-density shadow with a clear boundary, the size of the lesion was about 30.6 mm * 25.1 mm, the CT value was about 27.0 HU, and the adjacent trachea and esophagus were pushed . ContrasThe patient underwent posterior mediastinal cyst totally excise through video-assisted thoracoscope surgury (VATS) under general anesthesia. It was found that the lesion was located above the azygos arch in the superior and posterior mediastinum, on the right side of the esophagus, and protruded from the surface of the mediastinal pleura, with a size of about 30 mm * 30 mm. Intraoperatively, the cyst had a smooth surface, fixed position, clear boundaries and no invasion of surrounding tissues. The cyst was completely excised. The patient recovery and was discharged on the 7th day after surgery. Postoperative pathology examination showed the fibrous cyst wall lined with squamous epithelium and cuboidal epithelium, with hyperplasia of lymphoid tissue and formation of lymphoid follicles, consistent with branchial cysts . One yeaIn 1832, Ascherson firstly suggested that branchial cysts were the result of imperfect obliteration of a pharyngeal cleft . There aThe most common clinical presentation in patients with bronchial cleft cysts is a non-painful neck lump on the left side along the anterior border of the SCM . SimilarMediastinal cysts comprise 12 to 20 percent of the total mediastinal masses and foregut cysts are the most general occurring mediastinal cysts, including bronchogenic cysts and esophageal cysts . It's tyThe preoperative diagnosis of our patients was neurogenic tumor with cystic change. Neurogenic tumors are the most representative of posterior mediastinal tumors. Cystic changes are more common in schwannomas than in other neurogenic tumors. Schwannoma presents on CT as a clearly defined round or oval mass. Areas of heterogeneity correlate with cystic lesions, hemorrhagic lesions, or cellular reduction. Punctate, mottled, curvilinear calcifications can be observed along the walls of schwannomas . Other cBranchial cleft cyst is a congenital disease of the neck caused by abnormal degeneration of branchial organs, which occurs very rarely in the posterior mediastinum. When cystic lesions of the posterior mediastinum are found, bronchial cleft cysts are one of the noteworthy differential diagnoses. Radiological examinations (CT and MRI), ultrasound-guided FNA may assist in preoperative diagnosis. Surgery is the most effective method for the treatment of this disease.


We herein present a case of periventricular leukomalacia (PVL) with secondary optic pathway denervation atrophy, which was initially labeled as normal tension glaucoma. However, given the discordant clinical and ophthalmologic findings, brain magnetic resonance imaging was requested which proved PVL to be the underlying process to the patient's decreased visual acuity. In addition to presenting the ophthalmologic findings, we are emphasizing the pivotal role of neuroimaging in ruling out central causes of optic atrophy/hypoplasia and making this clinical distinction by demonstrating optic pathway atrophy associated with PVL. In patients with decreased visual acuity, ophthalmologic evaluation is the initial assessment step. The appearance of the optic disk, the exit point for ganglion cell axons leaving the eye, is essential in such evaluation. In patients with decreased visual acuity and associated abnormal cup to disk ratio, glaucoma is the initial diagnostic consideration. However, imaging can play a central role excluding central brain disorders mimicking glaucoma. Radiologists shall be aware of the potential brain pathologies that clinically present in a glaucoma-like fashion. On the other hand, it is imperative for the ophthalmologists to recognize the clues that suggest an alternative diagnosis to avoid the burden of inappropriate management.A 21-year-old gentleman presented to the ophthalmology clinic with decreased visual acuity, more severe on the right side. Five years back, he was diagnosed with normal tension glaucoma and was prescribed topical prostaglandin analogue and combination topical carbonic anhydrase inhibitors-beta blockers. Despite compliant treatment, his conditioned deteriorated. This prompted a new referral to our clinical for further ophthalmologic evaluation.Our patient's past ophthalmologic history was remarkable for childhood squint surgery as well as bilateral visual impairment. Co-existing medical conditions included diabetes mellitus, hypertension, and dyslipidemia. Initial ophthalmologic examination revealed that the best corrected visual acuity was 6/36 for the right eye and 6/12 for the left eye. The intraocular pressure was 14 mmHg and gonioscopic exam was normal. The eye fundus was evaluated following appropriate pupillary dilatation and showed rounded normal size optic disk. However, the cup was deep and large bilaterally yielding a cup to disk (C/D) ratio of 0.5 on the right side and 0.7 on the left side. In addition, there was significant circumferential thinning in the neuroretinal rim . FurtherThe aggregate findings of this comprehensive ophthalmologic evaluation again suggested a diagnosis of normal tension glaucoma. However, the patient age as well as the disease progression despite adequate treatment raised the possibility of an alternative central pathology. Hence, nonenhanced magnetic resonance imaging (MRI) of the head and orbits was pursued. Orbital evaluation on the MRI showed diffuse thinning of optic nerve sheath bilaterally extending to the optic chiasm . Also, tPVL is a known phenomenon in the context of perinatal insult in premature babies PVL preferentially involves the deep periventricular white matter tracts in the actively myelinating tracts in the peritrigonal area. This results in the characteristic ex-vacuo dilatation of the ventricles with wavy ventricular outline Although PVL is not a rare finding in neuroimaging, the added value of linking the patient clinical data to their imaging findings is substantial. Findings on MRI that might suggest an alternative etiology to patient's clinical diagnosis of glaucoma include PVL, predominantly involving the optic radiation, and optic pathway atrophy On the other hand, it is essential to the treating ophthalmologist to carefully evaluate patients’ medical history, including perinatal history. If such history is overlooked, the relationship of optic disk cupping, field defects, with possible prematurity can lead to a potential misdiagnosis with normal tension glaucoma. This is especially important as advances in perinatal care improved survival rates among premature infants, who may present later in life with visual field defects, nystagmus and strabismus.Additional clues to a potential misdiagnosis of normal tension glaucoma include younger patient age as well as bilateral inferior defects respecting the horizontal midline on perimetry. This contrasts with the older age and the visual field defects corresponding to the appearance of the optic disk typically seen in patients with normal tension glaucoma It is essential to differentiate glaucoma from other pathologic entities that present with glaucoma-like (pseudo-glaucomatous) ophthalmologic picture. Our case illustrates how PVL can rarely induce optic disk changes that might mimic the more common entity of normal tension glaucoma. Although both conditions might have similar clinical presentation, they dictate a disparate management plan. Thus, awareness of this clinical scenario is crucial to both radiologists and ophthalmologists who are involved in the care of these patients.


Pylephlebitis is a rare and life threatening thrombophlebitis of the portal vein. It commonly occurs following intra abdominal infections like appendicitis.It is even rarer in the pediatric age group. The nonspecific presentation impedes the diagnosis. Timely use of appropriate antibiotics and control of infection is paramount in its treatment and this case report highlights the same.11 year old female child from a rural area was referred from a local hospital for persistent fever and abdominal pain despite medical treatment. Workup revealed perforated appendicitis, pylephlebitis, and multiple liver abscess. She was successfully treated with appendicectomy followed by antibiotics and anticoagulants.Pylephlebitis secondary to appendicitis was frequently lethal in the pre-antibiotic era. Doppler ultrasonography and CT scan are the investigations of choice to establish the diagnosis by showing a thrombus in the portal vein. With use of antibiotics, early diagnosis by imaging and surgical control of the primary infection, appendicitis-associated-pylephlebitis now has improved outcomes. Larger scale studies are required to establish the role of anticoagulants.Early diagnosis and intervention of this fatal condition is life saving but numerous gaps exist in the literature regarding the treatment recommendation. •Pylephlebitis, the septic thrombophlebitis of portal vein is rare and fatal.•It usually occurs as a complication of intra abdominal infections.•Non specific presentation impedes it diagnosis.•Its progression can lead to bowel ischemia, sepsis and death.•Prompt treatment with antibiotics and control of infection is crucial for better outcome. It is an uncommon but life threatening complication of intra abdominal infection. It usually occurs following diverticulitis or appendicitis, and may also occur following necrotizing pancreatitis, inflammatory bowel disease, hemorrhoidal disease, perforation by foreign body, acute cholecystitis, Behcet's syndrome and amoebic colitis .Nonspecific clinical presentation makes the diagnosis difficult and it may progress to mesenteric and splenic vein thrombosis causing bowel ischemia/infarction, hepatic abscess and even death . The mosBacteroides fragilis owing to its coagulation promoting surface and capsular components [The incidence in children is unknown as it is rare but adult studies show an incidence of 2.7 per 100000 person-year . Though We describe a case of a female child presenting with pylephlebitis secondary to perforated appendicitis who was successfully managed with antibiotics and anticoagulation following appendectomy. With this case report, we aim to highlight that delay in diagnosis of common conditions like acute appendicitis may be encountered especially in developing countries, leading to rarer complications like pylephlebitis in our case. Timely diagnosis and appropriate antibiotics use with control of infection is of paramount importance for better outcome in such cases.2This case is reported in line with the SCARE 2020 checklist .3An 11-year-old female child, presented at our hospital with pain in the right lower abdominal pain for the past ten days. She was from a rural part of Nepal and was in her usual state of health until four weeks before when she presented with a history of abdominal pain, low grade fever with chills accompanied by vomiting and loose stool. The abdominal pain was located in the right lower quadrant, progressive in nature and moderate in severity. She was initially treated in a local hospital near her hometown. As her pain worsened and her symptoms did not improve after repeated treatment, she visited our hospital for further medical management. Her past medical and surgical history, family history, personal history and allergic history were insignificant.On arrival at the emergency via ambulance, she presented with persistent fever and right lower abdominal pain. She was ill-appearing, cachectic girl with a temperature of 101 °F, blood pressure of 90/60 mmHg and heart rate of 110 beats/minute. She was in tachypnea with a respiratory rate of 28 per minute. Her initial abdominal examination demonstrated tenderness in the right iliac fossa with positive rebound tenderness with normal bowel sounds but the remainder of the physical examination was unremarkable. Her laboratory values on admission showed a leukocyte count of 28,000/mm3, hematocrit of 30.5%, prothrombin time of 14.2 seconds, fibrinogen of 497 mg/dL, ALT (U/L)of 97, AST of 91 IU/L, and albumin of 2.98 g/dL ..Table 1Since the patient was hemodynamically unstable, she was admitted to the Paediatric Intensive Care Unit (PICU) for further workup. Our differential diagnosis included appendicular abscess and mesenteric lymphadenitis which were subsequently ruled out by imaging. She underwent Ultrasonography (USG) abdomen and pelvis which showed an ill-defined heteroechoic lesion with anechoic component measuring 2.7 X 3.1 × 3.3 cm in left lobe of liver with feature suggestive of hepatomegaly with liver abscess. There was a cavernous transformation of portal vein at intrahepatic part and collaterals at periportal region with intraluminal echogenic contrast suggestive of portal vein thrombosis at mid part suggestive of portal hypertension with minimal ascites. She further underwent Contrast Enhanced Computed Tomography (CECT) abdominal pelvis which confirmed hepatomegaly with multiple coalescing hypodense lesion diffusely scattered in liver parenchyma largest measuring (6.5X7.5 × 7.2 cm) in segment VII and enhancing internal septations within suggestive of lesion of infective etiology with multiple liver abscess. Portal vein thrombosis with periportal collaterals, mild ascites was also confirmed. The scan also showed wall enhancing collection in Right Iliac fossa (RIF) in close proximity to the appendix likely to be appendicular perforation with an abscess formation. . A, B.FiBased on the history, physical examination, and radiographic findings, a presumptive diagnosis of pylephlebitis secondary to a perforated appendix was made. She was started on broad spectrum antibiotics. An emergency open appendectomy was performed. Intraoperatively, significant abdominal ascitic fluid was noted. An appendiceal remnant in a retrocecal position with perforation on the tip of appendix was found. A periappendiceal abscess of around 10ml was found with extensive serosal fibrous adhesions consistent with perforated appendicitis. The base of the appendix was healthy and was tied and the abdomen was closed by the placement of subphrenic drain.3 day with resolution of abdominal symptoms. During her stay in the hospital she was prescribed an anticoagulant for portal vein thrombosis for the duration of three months. Histopathology of the appendectomy specimen confirmed perforated appendicitis. A computed tomography (CT) scan on postoperative day five showed significant reduction in the right hepatic abscesses, persistent left hepatic abscesses, and persistence without progression of the portal venous thrombosis. She then underwent ultrasound-guided percutaneous drainage of about 40 mL of pus from the left lobe of the liver. Antibiotics were continued for a total duration of 6 weeks. At 6 weeks follow up, she was asymptomatic and doing well.Postoperatively, the patient continued to have fever for the first day. On the second postoperative day, her leukocyte count significantly decreased to 11,000/mm4Pylephlebitis can be defined as the thrombophlebitis of the portal vein and its tributaries. Appendicitis leading to pylephlebitis in children is uncommon but a life threatening complication . Other aThere are only a few case reports published about pylephlebitis in children secondary to perforated appendicitis.Pylephlebitis secondary to appendicitis used to be the most common cause of hepatic abscess in the pre antibiotic era (50%) and was frequently lethal .In the current era of early treatment with antibiotics and timely diagnosis with imaging, incidence of pylephlebitis is rare. However, in developing countries like ours where lack of tertiary care facilities in rural areas and patient misinformation is prevalent, its incidence may be more. Diagnostic delay of pylephlebitis is attributable to its non-specific clinical presentation ranging from fever, abdominal pain, jaundice and weakness to septic shock and death . Delay iAn uncontrolled infection in the regions drained by the portal system initially leads to thrombophlebitis of the small mesenteric veins and eventually spread to the portal vein and liver parenchyma, leading to pylephlebitis and hepatic abscess . Apart f5Pylephlebitis secondary to appendicitis albeit rare in the era of modern medicine, may still be prevalent in developing countries. The early intervention of this fatal condition is life saving but numerous gaps exist in the literature regarding its treatment recommendation. Empirical regimen and duration of antibiotics, role of anticoagulants and its clinical endpoint if used must be clearly delineated by large scale studies. On the clinician's part, pylephlebitis must be kept in mind when the patient presents a long standing abdominal sepsis.Not required in our case.None.Sunil Basukala (SB) = Conceptualization, Supervision.Sunil Basukala (SB), Soumya Pahari (SP), Manju Shrestha (MS) = Writing - original draft.Soumya Pahari (SP), Manju Shrestha (MS), Sunil Basukala (SB), Pooja Kafle (PK), Yugant Khand (YK), Ojas Thapa (OT), Kalpana Rai (KR), Anup Thapa (AT) = Writing - review & editing.All the authors read and approved the final manuscript.1.Name of the registry: Not applicable2.Unique Identifying number or registration ID: Not applicable3.Hyperlink to your specific registration (must be publicly accessible and will be checked): Not applicableSunil Basukala (SB).Written informed consent was obtained from the patient's parent for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.No conflict of interest.


Hydatidosis is a cosmopolitan parasitic disease which presents a real public health problem especially in endemic countries of which Morocco is part. The objective of the present work is to analyze the clinical, paraclinical, therapeutic, evolutionary and prognostic aspects of disseminated peritoneal hydatidosis with multiple localization.Peritoneal hydatidosis represents the whole of the phenomena due to :The seeding, essentially secondary, of the peritoneal serosa by Echinococcus Granulosus larvae, Peritoneal hydatidosis is characterized by its polymorphic symptomatology, and the diagnosis is based on a combination of epidemiological, clinical, biological and imaging findings.We report a case of a particular form of peritoneal hydatidosis in the department of visceral surgery I of the ibn rochd hospital in casablanca.Our patient was admitted for management of disseminated peritoneal hydatidosis. The clinical examination, apart from an epigastric crust, was unremarkable. The biological work-up showed a slightly disturbed liver balance and the hydatid serology was strongly positive. The preoperative diagnosis of HP was established by CT scan showing a supra- and sub-mesocolic peritoneal hydatidosis with a multi-cystic spleen and a liver with a type V segment V hydatid cyst measuring 4 cm by 6 cm.The treatment consisted of a total cystectomy of the hydatid cysts, almost 100 cysts with multiple peritoneal and parietal locations, one of which was fistulized in the skin, associated with a total splenectomy, retrograde appendectomy and a disconnection of the cholecysto-duodenal fistula with duodenal closure and a retrograde cholecystectomy associated with a choledecotomy with extraction of 3 stones at the level of the choledochus and drainage of the VBP by Kehr drain. The postoperative course was simple and the patient was discharged on the sixth day with an adjuvant treatment with albendazole for three months.Through this observation and in the light of the data in the literature, we were able to insist in our present work on the diagnostic difficulties generated by this unusual location of the hydatid cyst as well as the considerable contribution of imaging (CT++) allowing both a positive and very precise topographic diagnosis. We were also able to focus on surgical treatment as an indispensable pillar of the management of this disease as well as the increasingly fundamental role of medical treatment, particularly in the prevention of recurrences.Peritoneal hydatidosis is a rare but serious complication of hydatid disease.The positive diagnosis is based on epidemiological, clinical, and paraclinical arguments represented essentially by CT scan.Early diagnosis and treatment of primary sites as well as optimal surgical management of peritoneal hydatidosis determine the prognosis. •Peritoneal hydatidosis is a parasitic condition secondary to seeding of the peritoneal serosa by Echinococcus granulosus larvae.•It is often secondary to rupture or fissuring of hepatic hydatid cysts. It's a rare parasitic condition.•Its diagnosis is based essentially on biological and especially radiological exploration techniques, it's treatment remains primarily surgical, Antiparasitic medical treatment is prescribed to prevent recurrence.•The prognosis is generally good and the evolution is essentially marked by the risk of recurrence, especially in disseminated and late forms. It is often secondary to rupture or fissuring of hepatic hydatid cysts.It is a rare parasitic condition. It represents about 5–16% of all hydatid cyst locations [Its diagnosis is based essentially on biological and especially radiological exploration techniques . Its treThe prognosis is generally good and the evolution is essentially marked by the risk of recurrence, especially in disseminated and late forms .We report a case of peritoneal hydatidosis of a particular form with more than 100 abdomino-pelvic hydatid cysts with multiple hepatic, peritoneal, parietal, splenic, and pelvic localizations in the department of visceral surgery 35 of the Ibn Rohd Hospital in Casa.1.1She is Mrs xxx 29 years old, married and mother of 2 children. Admitted to the Visceral Surgical Emergency Department 35 of the IBN ROCHD Hospital for the management of a disseminated peritoneal hydatidosis. No particular pathological history.The clinical examination revealed a soft abdomen with an epigastric crust.The physical examination, and the rest of the somatic examination was unremarkable.The biological workup showed a slightly disturbed liver function test and a strongly positive hydatid serology.An abdominal ultrasound revealed: a reshaped hydatid cyst of segment III of the liver fistulized in the subcutaneous soft tissues measuring 4.19*1.98 cm and contunited with another formation measuring 3*2 cm.Abdominal and pelvic CT scan in axial and sagittal section showed multiple hepatic, splenic and peritoneal cystic formations of which one of the hepatic lesions is fistulized to the wall of which the most voluminous is pelvic measuring 40*43mm and a cystic umbilical formation measuring 13*10mm with main biliary tract seat of a dense material with upstream dilatation of 10mm hydatid origin with the presence of an aerobilic that can evoke a bilio-digestive fistula, the pre-anaesthetic assessment was correct.✓For the peritoneal cysts, they were small with a weight varying between 10 gramsand 45 g✓for the parietal cysts, 17 cysts were estimated to weigh 50 g each with the largestbeing fistulised to the skin with an estimated weight of 100 g✓for 12 pelvic cysts, they were small with a weight ranging from 15 g to 70 g✓❖with a total weight of all cysts estimated at 1kg 360g Fig. 2Aftfor one multi-cystic spleen, its weight was estimated at 350 gA surgical operation was programmed with the presence of several abdomino-pelvic hydatid cysts which were distributed as follows 70 peritoneal cysts, 18 cysts of parietal localization of which one was fistulized to the skin, 12 pelvic cysts with a multi-cystic spleen , we alsoThe surgical procedure consisted of a total cystectomy of the hydatid cysts associated with a total splenectomy, retrograde appendectomy and a disconnection of the cholecysto-duodenal fistula with duodenal closure and a retrograde cholecystectomy associated with a choledecotomy with extraction of 3 calculi at the level of the choledochus and draining of the VBP by Kehr drainthe surgical procedure was performed on a scheduled date with a correct pre-anesthetic assessment; the procedure was performed by an assistant professor in general surgery and two residents in the same specialtyThe surgical procedure was performed on a scheduled date with a correct pre-anesthetic assessment; the procedure was performed by an assistant professor in general surgery and two residents in the same specialty.The operation was performed in the operating room of the P35 visceral emergency department at the CHU ibn rochd hospital in Casablanca, Morocco the patient was satisfied with the intervention and the improvement of his health in the short and long term.The postoperative evolution was without particularity, with the patient leaving the hospital at 6 days post-op. patient follow-up was every 15 days with a complete clinical examination with primary care of the surgical wound with instructions to change the dressing every 2 days and take antibiotics, anticoagulants and antlagics for 15 days and to avoid carrying heavy loads until complete healing then the patient was hospitalized after 6 months for residual calculus at the level of the VBP where she benefited from an ERCP (endoscopic retrograde cholangiopancreatography)This case report is compliant with the SCARE Guidelines 2020 [2Peritoneal hydatidosis is a rare but serious complication of hydatid disease . It reprIn the literature, the age distribution of hydatidosis generally shows a predominance of the disease in young adults ,8.Peritoneal echinococcosis is most often secondary to rupture or fissuring of a KH of hepatic origin in 66–85% of cases or of splenic origin in 10–20% of cases [Primary peritoneal hydatidosis is exceptional. The rupture of the primary cyst is spontaneous in 78% of cases . It is fThe stages that follow the rupture reflect the reaction of the peritoneal serosa to the hydatid aggression. They present as simple hydatid ascites when the cyst is monolocular, free hydatids in the large peritoneal cavity when the cyst is multivesicular, or hydatid peritonitis if the cystic content is infected.In the absence of treatment, these early forms can evolve in two ways: either subserosal grafting with development of peritoneal cysts is by far the most frequent, it can be either a multiple generalized form with sometimes more than 100 cysts as in the case of our patient or a form localized to a region of the abdomen. It can be a pseudo-tubercular form with a cluster of multiple punctiform cysts reminiscent of the miliary granulations of tuberculous peritonitis. The nature of the infecting material remains controversial. FERRAND and BARSOTTI  attributEncysted collections are characterized by the existence of a neoformed encystment membrane isolating the ruptured cystic content from the rest of the peritoneal cavity.Acute intraperitoneal rupture is often discovered per operatively during emergency laparotomy .However, the association of an acute abdominal picture with signs of allergy  should attract attention, especially in the presence of the notion of trauma.The classic forms, late and insidious, have a polymorphic symptomatology. This can easily be explained by the diversity of cyst locations .The functional signs are dominated by pain and signs of compression  which represented in our patients the clinical translation of the very advanced forms. The general state remains preserved for a long time.Medical imaging is an essential step in the diagnosis of peritoneal hydatidosis. Imaging techniques are nowadays numerous and efficient and the images obtained are often highly suggestive. They allow to establish the positive diagnosis of hydatid cysts, to confirm their location, to contribute to therapeutic strategies and to ensure the monitoring of treated subjects.Ultrasound is considered to be the first-line examination for the diagnosis and detection of hydatid disease in its abdominal locations, with a reliability of more than 90% in most series reported in the literature. As far as peritoneal hydatidosis is concerned, it has been proven that ultrasound demonstrates the primary Hydatid Cyst in case of secondary peritoneal hydatidosis, and facilitates the diagnosis of single or multiple primary peritoneal hydatid localizations in case of isolated peritoneal hydatidosis [Ultrasound has other advantages. Indeed, it allows a study of the relationship of the hydatid cyst with the portal bifurcation, the suprahepatic veins, the inferior vena cava and the upper urinary tract in search of possible compression.In abdominal hydatidosis, CT has completely revolutionized lesion and topographic diagnostic management, as well as therapy .Since its advent, it has become an indispensable complementary examination as soon as a surgical decision is indicated. CT not only confirms the disease but also provides a very useful lesion map for the surgeon.Abdominal CT (in spontaneous contrast and/or with intravenous iodine injection) allows an easy and much more precise diagnosis than ultrasound of the hydatid cyst, particularly in its peritoneal location ,10,12. C(MRI) easily allows the diagnosis of rupture, but also to monitor the evolution of the hydatid cyst under medical treatment .The differential diagnosis can be made with other cystic or pseudocystic masses of the peritoneum: tuberculosis, gelatinous disease, serous cyst and cystic lymphangioma. Hydatid serology is therefore of great interest; however, it is a good means of monitoring after treatment.The treatment of peritoneal hydatidosis remains above all surgical ,16. The In acute forms, the treatment consists of evacuating the peritoneal effusion  and cure the infesting viscera. A meticulous peritoneal cleansing completes the operation.In late forms, the peritoneal time may require the association of several procedures depending on the topography of the lesions and the importance of the dissemination. However, certain rules must be borne in mind: always free the pelvis first, otherwise pelvic compression may occur, the prognosis of which may be vital; adapt the duration of each operative stage to the resistance of the patient, starting with the destruction of the largest cysts or those that compress the intestine, and evacuate the primary hydatid cysts from the first operative stage. A complete cure in a single operation is desirable ,18. It iThe prognosis of the disease depends on the extent of peritoneal dissemination, the existence of visceral localizations and their severity, the general condition of the patient and the number of operations he/she has already undergone, the completeness of the cure and the experience of the surgeon.Long term monitoring is essentially aimed at detecting recurrences requiring reoperations. Since one can never be sure of having performed a complete and exhaustive removal of the lesions, any patient operated on for peritoneal hydatidosis must therefore be monitored as regularly and closely as possible, from the immediate postoperative period until several years later . This mo3Peritoneal hydatidosis is a rare but serious complication of hydatid disease.The positive diagnosis is based on epidemiological, clinical, and paraclinical arguments represented essentially by CT scan.Surgery is the mainstay of treatment of peritoneal hydatidosis, based on radical or conservative methods which must act on both the peritoneal hydatidosis and the primary visceral hydatid cyst. Medical treatment also has its place in this condition. It represents an interesting therapeutic alternative for inoperable patients and for disseminated and multivisceral hydatidosis. It is also of interest in the prevention of relapses and recurrences by providing a framework for the surgical intervention.Long-term postoperative surveillance is necessary to detect any recurrence. It is essentially based on ultrasound/CT and serology.Early diagnosis and treatment of primary sites as well as optimal surgical management of peritoneal hydatidosis determine the prognosis.I declare on my honor that the ethical approval has been exempted by my establishment.None.El Bakouri Abdelilah: writing the paper and operating surgeon.Fatine Amine: writing the paper and operating surgeon.Eddaoudi Yassine: Corresponding author writing the paper and operating surgeon.Bouali Mounir: study concept.El Hattabi Khalid: study concept.Bensardi Fatimazahra: study concept.Fadil Abdelaziz: correction of the paper.Researchregistry2464.Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient.Dr Fatin Amin.Not commissioned, externally peer-reviewed.The authors declare having no conflicts of interest for this article.


Primary aldosteronism (PA) is a common cause of secondary hypertension, whereas pheochromocytoma is a rare cause of it. Thus, concomitant PA and pheochromocytoma is a very rare condition.123I- metaiodobenzylguanidine (MIBG) scintigraphy showed a marked tracer uptake in the left adrenal mass with no metastatic lesion. After preoperative management with α-blockade, laparoscopic left partial adrenalectomy was performed. Immunohistochemical examination of the tumor showed chromogranin A positivity leading to the diagnosis of left pheochromocytoma.A 52-year-old woman was admitted to our hospital with suspected PA based on the presence of hypertension, spontaneous hypokalemia, and a high aldosterone-to-renin ratio. She had no catecholamine excess symptoms other than hypertension. Abdominal computed tomography (CT) showed a right lipid-rich adrenal mass and a left lipid-poor adrenal mass. PA was diagnosed by the captopril challenge test. The 24-h urinary fractionated metanephrines were slightly elevated. Adrenal vein sampling (AVS) confirmed that the right adrenal gland was responsible for aldosterone hypersecretion. Medical therapy with eplerenone was started because the patient refused surgery. Five years later, she requested surgery for PA. The second AVS confirmed right unilateral hyperaldosteronism, as expected. Repeated abdominal CT showed the enlargement of the left adrenal mass. The 24-h urinary fractionated metanephrines had risen to the diagnostic level. We report an extremely rare case of concomitant unilateral PA and contralateral pheochromocytoma. When diagnosing unilateral PA by AVS, especially in cases with a lipid-poor adrenal mass, clinicians should rule out the possibility of the presence of pheochromocytoma before proceeding to undergo unilateral adrenalectomy. Although there is no standard treatment for this rare condition, it is essential to select personalized treatment from the perspective of conserving the adrenal gland. Hypertension is classified as essential hypertension, which has no known cause, and secondary hypertension, which results from an underlying identifiable cause . Case-de123I-MIBG scintigraphy showed a tracer-avid left adrenal mass but no metastatic lesions , spontaneous hypokalemia , and a high aldosterone-to-renin ratio (ARR 71 ng/dL per ng/mL/h [plasma renin activity (PRA) 0.2 ng/mL/h and plasma aldosterone concentration (PAC) 14.2 ng/dL]). On admission, the patient showed no clinical features of Cushing syndrome, and no catecholamine excess symptoms other than hypertension, such as headache or excessive sweating. Before the initiation of antihypertensive drugs, PA was confirmed by the positive captopril challenge test  have been reported due to increased use of CT for abdominal imaging or family screening . Unlike When we diagnose unilateral PA by AVS, it is essential to exclude the coexistence of pheochromocytoma before adrenalectomy for two reasons. First, in cases of coexisting pheochromocytoma, the preoperative α-adrenergic blockade is necessary to prevent a hypertensive crisis caused by the rapid release of catecholamines from the tumor due to intraoperative stimulation . In our Second, when unilateral PA and pheochromocytoma are present on the contralateral side, as in our case, personalized treatment should be considered to prevent permanent hypocortisolism after complete bilateral adrenalectomy. Medical treatment with an MR antagonist is the standard treatment for bilateral PA because it improves hypertension and other deleterious cardiovascular effects , whereasLast, the mild autonomous secretion of cortisol after 1mg DST in both 2015 and 2020 should be discussed. First, the patient showed no clinical feature of Cushing’s syndrome, which ruled out Cushing’s syndrome. In addition, the diurnal variation of serum cortisol was not disappeared, which ruled out subclinical Cushing's syndrome according to the guidelines of the Japan Endocrine Society . We alsoIn conclusion, when clinicians encounter unilateral PA with a lipid-poor, vascular adrenal mass, the coexistence of pheochromocytoma should be excluded. Even if the tumor is small and is in the pre-biochemical phase, it should be closely monitored for tumor growth and an associated increase in catecholamine metabolite levels, which should help diagnose pheochromocytoma. Although the standard treatment for unilateral PA is unilateral adrenalectomy, in cases of unilateral PA with contralateral pheochromocytoma, we suggest trying the medical treatment with an MR antagonist first to preserve adrenal function.


The current high detection rate of adrenal tumors  is attributable to a widespread use of variety of imaging studies, especially a computed tomography. Most of them represent clinically silent and biologically indolent incidentalomas, but some adrenal tumors may pose a significant clinical challenge. Thus, in every patient with an adrenal tumor, a decision on further management is made after careful hormonal and radiological evaluation. All hormonally active tumors and those with radiological features suggesting malignancy are qualified for surgery. Approximately 80% of adrenal tumors are adrenocortical adenomas, hypertrophy, or nodular adrenocortical hyperplasia. Other histopathological diagnoses include pheochromocytoma, adrenocortical carcinoma, metastases, mesenchymal tumors, lymphomas, cysts, and ganglioneuromas. Adrenal tumors are more commonly diagnosed and better studied in elderly patients. In younger patients, under 40 years old, focal adrenal lesions are relatively rare, and histological distribution of diagnoses differs from that in elderly individuals. Younger patients are more likely to display endocrine symptoms, which raise the suspicion of an adrenal mass. In the current study, we present a case series of seven adrenal tumors occurring in young patients. The cases presented below, along with the literature review, demonstrate that the diagnosis and treatment of adrenal tumors are crucial due to endocrinopathy-derived complications and a potential risk of malignancy. Adrenal tumors occur in approximately 4–10% of the general population, and their detection rate has increased with age ,3,4 as aThe vast majority of adrenal tumors (approximately 80%) are adrenocortical adenomas, hypertrophy, or nodular adrenocortical hyperplasia. Other lesions include pheochromocytomas (PHEO) 3–6%), adrenocortical carcinomas (ACC) 2–5%), adrenal metastases 1–2%), mesenchymal tumors , lymphomas, cysts, ganglioneuromas [–2%, mese–5%, adre–6%, adreThis article presents seven cases of adrenal tumors detected in patients younger than 40 years old. In this age group, adrenal masses are relatively rare, and histological distribution differs from that in elderly individuals. They were divided into two groups—the first three cases concern hormonal active tumors, whereas the next four adrenal lesions did not show hormonal activity.A 31-year-old man was admitted to hospital to investigate the cause of arterial hypertension that had occurred 1 year before, with peak values >200/100 mmHg and concomitant headache, reduced exercise tolerance, exertion dyspnea, and unintentional weight loss (6 kg) over past 3 months. On admission, he was in good general condition, with a BP of 170/100 mmHg. Physical examination revealed a systolic heart murmur and lower extremities edema. Biochemical tests showed hypokalemia (2.8 mmol/L) with normal sodium concentration (143 mmol/L) and proteinuria. Abdominal ultrasound (US), head computed tomography (CT), doppler ultrasound of carotid, vertebral, and renal arteries showed no clinically significant abnormalities. Transthoracic echocardiography (TTE) revealed left ventricular hypertrophy and left atrium enlargement. On fundoscopic examination, a third-grade hypertensive retinopathy in Keith–Wagner–Barker classification was diagnosed. An abdominal CT scan demonstrated low-density  right adrenal gland mass, measuring 15 × 11 × 19 mm with radiological features of adenoma, and thickening of the medial limb of the left adrenal gland up to 9 mm . In hormTo confirm lateralization of aldosterone secretion, adrenal venous sampling (AVS) was performed, clearly indicating the right-sided aldosterone hypersecretion. A therapy with lercanidipine, ramipril, eplerenone, doxazosin, bisoprolol, and potassium was introduced, with effective control of BP and normokalemia. The patient underwent right-sided laparoscopic adrenalectomy. The surgery resulted in the normalization of potassium levels and a significant improvement in BP control. Histopathologic examination of specimens showed an adrenocortical adenoma with moderate cellular pleomorphism, without mitotic activity, features of necrosis, or vascular invasion .2), and liver enlargement were noted. A subsequent non-contrast  CT scan revealed the presence of a mass originating from the right adrenal gland, measuring 56 × 58 × 56 mm . His past medical history included hypertension  and nocturia. On admission, increased BP (160/100 mmHg), obesity (Body Mass Index (BMI)—31 kg/m × 56 mm . Based o × 56 mm . Due to poor BP control, as well as for clinical and radiological suspicion of PHEO, an alpha-blocker doxazosin was added to antihypertensive therapy in up-titrated doses to 16 mg per day. Two months after the diagnosis, the patient underwent a right-sided laparoscopic adrenalectomy, which required a switch to open laparotomy due to intraoperative bleeding. Histopathological examination revealed PHEO. A single tumor embolus in a blood vessel was found, suggesting a malignant character of the lesion .A CT scan performed two months after surgery showed no recurrence of the disease. Levels of urinary catecholamines were normal. Renal function did not improve significantly after surgery. The patient remained under nephrological care, and has recently received a pre-emptive kidney transplantation.A 37-year-old male patient was referred to the hospital for the diagnostics of poorly controlled arterial hypertension, diagnosed two years before. He was treated with two antihypertensive drugs , with office BP values of 140/100 mmHg. Family history revealed that the patient’s mother was diagnosed with Cushing’s syndrome. 2), but otherwise, no signs of endocrinopathy were noted. CT scan detected a right adrenal mass measuring 36 × 29 mm with radiologic features of adenoma. Hormonal work-up revealed hypercortisolism  revealed no suppression of cortisol secretion. The patient was subjected to an adrenalectomy. He did not require preoperative treatment with steroidogenesis inhibitors. In histopathological examination, an adrenal cortical adenoma was diagnosed.2), with hemiparesis resulting from cerebral palsy, and tenderness on palpation of the umbilical and left hypochondriac region. Biochemical tests showed no significant abnormalities in hormonal work-up  over the past year. A physical examination revealed the patient to be underweight  . After aA 33-year-old woman was referred to the endocrinology department due to a right adrenal mass. It was detected in ultrasonography performed due to dyspeptic symptoms. A tumor was described as a solid, heterogeneous, highly vascularized mass, merging with the liver capsule, and measuring 42 × 33 × 49 mm. Moreover, enlarged lymph nodes of the hepatic hilum were identified. The CT scan confirmed the presence of a right adrenal gland tumor with radiological features suggestive of malignancy or PHEO  .On admission, the physical examination, as well as hormonal evaluation, revealed no clinically significant abnormalities . Due to The increasing detectability of adrenal tumors resulting from the widespread use of imaging studies allows us to make decisions regarding further management. The decision about follow-up or surgical intervention is often preceded by multidisciplinary examinations, carried out in accordance with the current guidelines ,2. The hImaging studies of adrenal tumors are assessed to distinguish between benign and malignant lesions ,19,20,21The diagnosis of PHEO, especially in mildly symptomatic or non-secreting forms, remains a diagnostic challenge . If a maEventually, adrenalectomy is recommended for hormonally active lesions, tumors with radiologic features described in imaging studies as “non-adenoma”, tumors with a diameter >3–6 cm ,25,26, oAdrenal cortical carcinoma is the primary tumor of adrenal glands with the worst prognosis. It affects females more frequently and displays two peaks of incidence in the first and fifth decades of life. It remains asymptomatic for a long time, so it is often diagnosed at the locally advanced or generalized disease stage. Despite a better understanding of the molecular pathology of ACC, and improved diagnostics and treatment, it still has high mortality rate ,28,29.The most common lesion originating from the adrenal medulla is PHEO, which accounts for approximately 3–6% of diagnoses ,2,6. CliAdrenal metastases are common. Adrenal glands constitute the fourth most common site after lungs, liver, and bones of metastatic disease . In histCysts are rare lesions of the adrenal glands. The incidence of adrenal cysts is below 0.2%, and shows a predilection for women . They caGanglioneuroma (GN) is another rare adrenal gland tumor. The prevalence of adrenal GN is estimated at 0.3–2%. GN is a benign, well-differentiated neurogenic tumor that arises from neural crest cells, and it is composed of intermixed Schwann cells, ganglion cells, and nerve fibers . GanglioThe prevalence of adrenal tumors increases with age, and, therefore, the elderly are most often hospitalized in endocrine departments. The majority of these patients are those with adrenal tumors which are found incidentally in image studies. Image studies, especially with the use of ionizing radiation techniques, are performed in younger patients only in the case of reported symptoms. Presented cases of adrenal tumors in the group of patients under 40 years of age describe both hormonally active and nonfunctional lesions. They can cause clinical symptoms and lead to serious complications. An active search for adrenal gland pathologies in that patient group has an important diagnostic and therapeutic significance. We should not underestimate adrenal tumors found in image studies, since, in this age group, diagnoses of ACC occur more frequently ,6,27. Mo


Gastric duplication cyst associated with ectopic pancreas is rare and we aimed to alert clinician to this congenital anomaly.A 15-year-old girl presented with intermittent vomiting. Gastroscopy showed a submucosal tumor with an approximate diameter of 40 mm in the anterior wall of the gastric antrum. The lesion had a central umbilication and was diagnosed preliminarily as gastric ectopic pancreas with pseudocyst formation on the basis of its appearance. However, computed tomographic scan showed a thick-walled cystic lesion with an enhanced outline of the cystic wall in the antrum of stomach, suggestive of duplication cyst. Serum amylase was normal. Endoscopic ultrasonography revealed a solid-cystic lesion; the solid portion were inhomogeneously mixed with echoes, and had indistinct border to muscularis propria; the cystic portion had echogenic internal mucosal layer and distinct border to muscularis propria. Endoscopic submucosal dissection (ESD) was suggested for the patient to relieve symptoms and diagnose the lesion definitely. The operation procedure was uneventful and the solid-cystic lesion was resected completely. Histopathologic examination revealed that the solid portion was ectopic pancreas, and the cystic portion was gastric duplication cyst. After resection, the patient discharged successfully and neither symptoms nor tumors recurred during the 9 months follow-up period.This is the first case of a solid-cystic lesion with central umbilication in the stomach diagnosed as gastric duplication cyst associated with ectopic pancreas. ESD could be an optional treatment to provide a definitive diagnosis.The online version contains supplementary material available at 10.1186/s12893-022-01837-z. Gastric duplication cyst associated with ectopic pancreas is a rare congenital anomaly . When prA 15-year-old girl presented with intermittent vomiting for the previous 5 years and worsened for 1 month. Medical history, physical examination, routine laboratory examination, electrocardiogram and chest X-ray were unremarkable. Gastroscopy showed a submucosal tumor with an approximate diameter of 40 mm in the anterior wall of the gastric antrum Fig. . Serum aGastric duplications represent about 7% of gastrointestinal tract duplications and are even more uncommon associated with ectopic pancreas . The etiPreoperative diagnosis of gastric duplication cyst with ectopic pancreas is difficult, largely due to their rarity and the absence of characteristic findings. CT and EUS may provide some informative findings. Contrast-enhanced CT scan typically demonstrates gastric duplication cyst as a thick-walled cystic lesion with enhancement of the inner lining . HoweverIn conclution, gastric duplication cyst with ectopic pancreas should be included in the differential diagnosis of a solid-cystic lesion with central umbilication in the stomach. ESD could be a minimal invasive treatment to provide a definitive diagnosis.Additional file 1: Fig S1. The cystic portion’s cyst wall was lined by epithelium of gastric and surrounded by smooth muscle .Additional file 2: Fig S2. The solid portion was pancreatic tissues composed of acini, ducts, and islets of Langerhans .


Dear Editor,This case report describes a four-year-old child, followed for three years, with normochromic plaques covered by a cluster of follicular openings filled with keratin, comedo-like, along a large extension of the right side: cervical and retroauricular regions, trunk, buttocks, lower limb and foot.The lesions were located along the Blaschko's lines . DermoscNevus comedonicus is a rare type of epidermal nevus, characterized by a developmental change affecting the pilosebaceous unit. The most frequently affected regions are the face, cervical region, and trunk. It affects both sexes equally, can be congenital, and, in most cases, appears in children before the age of ten.The first report of nevus comedonicus was presented in 1875 by Kofmann, and a little over 200 cases have been reported in the literature since then.The diagnosis of nevus comedonicus is usually a clinical one and may be aided by dermoscopy, especially to differentiate it from other epidermal nevi, such as nevus sebaceous.This rare hamartomatous condition may be associated with genetic syndromes affecting other systems, and it is important to investigate skeletal, ocular, and central nervous system alterations, which, if present, constitute the nevus comedonicus syndrome.The treatment can be topical with the use of emollients, corticosteroids in inflammatory lesions, and keratolytic agents. The use of topical tretinoin has been reported, but there is limited data regarding its efficacy.None declared.Gessica Ramos Barroso Diniz: Collection of data; drafting and editing of the manuscript or critical review of relevant intellectual content; approval of the final version to be submitted.Flávia Vasques Bittencourt: drafting and editing of the manuscript and critical review of relevant intellectual content; approval of the final version to be submitted.None declared.


Our patient is a 58-year-old female patient that presented with right shoulder pain. An MRI showed a shoulder mass, and percutaneous bone biopsy demonstrated morphology suggestive of an MPNST; besides, on immunohistochemistry, SOX10 was positive, and H3K27me3 expression was entirely lost. The patient underwent total resection of the right proximal humerus and endoprosthetic shoulder reconstruction, followed by radiation therapy and chemotherapy. Only a few cases in the mandible, spine, maxilla, ulna, metacarpal, tarsal, and one in the humerus have been published. In this paper, we contribute with an additional case of primary intraosseous MPNST in the humerus and a brief literature review.Malignant peripheral nerve sheath tumors (MPNSTs) usually arise in the soft tissues. Intraosseous MPNSTs are rare. They may arise  The most common sites for MPNSTs are the peripheral nerves of the trunk, extremities, head and neck regions, and spine. Histologically, these tumors are highly cellular with frequent mitoses and exhibit anaplasia, necrosis, infiltrative growth pattern, pleomorphism, and high proliferative activity. Most MPNSTs are high-grade and stain for the S100 protein; weak staining is associated with a higher risk of distant metastasis. NF1-associated MPNSTs have a worse outcome compared to patients with sporadic MPNST. Treatment for peripheral nerve tumors is surgical resection [Primary intraosseous malignant peripheral nerve sheath tumors (MPNSTs) are rare, highly aggressive tumors arising from peripheral nerves, usually present as soft tissue masses with pain or loss of function. Patients complain of constant pain and are more likely to have motor and sensory deficits in the distribution of the parent nerve. They usually affect adults in the third to fifth decades of life and are frequently associated with neurofibromatosis (NF) type 1 and 2 or previous radiotherapy treatment. About 50% of MPNSTs occur esection .Our patient is a 58-year-old woman with a history of hypertension, hyperlipidemia, osteopenia, gastric bypass, and hysterectomy. The patient had been complaining of right shoulder pain for several months; she rated the pain as 3/10 in intensity, intermittent, aching, sharp, and throbbing in nature, aggravated by position. She had a magnetic resonance imaging (MRI) of the right shoulder performed one year prior, which showed a shoulder mass. The patient mentioned that she had only been using heating pads or acetaminophen for the pain.A right proximal humeral mass was found on physical examination, and radiographs showed a malignant lesion with a somewhat nonspecific radiographic appearance. An MRI and computerized tomography (CT) scan were ordered. The MRI of the upper extremity without contrast showed a 3.9 x 2.6 x 7.7 cm neoplasm in the proximal right humeral meta diaphysis, disrupting the posterior cortex. A CT of the chest, abdomen, and pelvis with contrast showed a lytic lesion involving the proximal right humerus measuring up to 4.4 x 3.6 x 3.5 cm. The next day, a percutaneous bone biopsy was performed to study the lesion further Figure .Per pathology, intersecting fascicles of monomorphic spindled cells with wavy or buckled nuclei associated with cells showing granular cell change were found. On immunohistochemistry (IHC), complete loss of H3K2me3 was seen; S100 protein, SOX10, Desmin, Myogenin, MyoD1, and CD34 were negative. A diagnosis of high-grade spindle cell sarcoma showing morphologic and IHC features of an MPNST was made. The patient was scheduled for total resection of the right proximal humerus and endoprosthetic shoulder reconstruction. The patient underwent a radical resection of a right proximal humerus sarcoma with brachial plexus neurolysis, reverse total shoulder arthroplasty, lattisimus dorsi, and teres major transfer, myofasciocutaneous advancement to assist with primary wound closure and incisional wound vacuum-assisted closure placement. She tolerated the procedure well and was transferred to the intensive care unit in stable condition. The humerus head and a segment of humerus bone with attached muscular tissue were sent for pathology examination. The humerus head measured 5.5 x 5 x 4.5 cm, with a piece of humerus bone measuring 6 cm in length. The attached tissue measured 8 x 7 x 5.5 cm. The soft tissue margin was sectioned to reveal a tumor 0.2 cm from the posterior margin and a grossly narrow lateral margin. The humerus head was sectioned perpendicular to reveal a well-defined white tumor measuring 7 x 4.5 x 4 cm, occupying most of the head, and extending distally into a portion of the humerus bone  a month later. Three weeks after initiating XRT, she had an MRI of the head, which showed no evidence of malignancy. The patient completed XRT, and three months later, she returned to medical oncology; laboratory results included white cell count 3.2 (3.8 - 9.8 K/mm3), absolute neutrophil count 1.9 (2.0 - 7.6 K/mm3), hemoglobin 11.6 (11.2 - 14.9 g/dL), hematocrit 36.5 (33.2 - 43.5 %), platelets 215 (129 - 368 K/mm3), creatinine 0.72 (0.52 - 1.04 mg/dL). An echocardiogram reported left ventricular ejection fraction (LVEF) 55/60% with no abnormal wall motion. The patient started doxorubicin 60 mg/m2, four cycles, every 21 days.Two months later, the patient was scheduled for a positron emission tomography (PET) scan; she had filgrastim injections at the same time as the PET scan, which revealed a right shoulder prosthesis with uptake in surrounding soft tissue between 3-5, no underlying mass noted. Right pubic bone and right acetabulum standard uptake value (SUV) 6, right upper lobe and right lower lobe nodules up to 0.8 cm with SUV up to 1.8. Laboratories results included white cell count (4.0 K/mm3), absolute neutrophil count (2.5 K/mm3), hemoglobin (11.6 g/dL), hematocrit (36.2 %), platelets (178 K/mm3), creatinine (0.8 mg/dL). Laboratory results can be further visualized in Table MPNST represents NF's most severe and often fatal complication. This term encompasses previously used designations such as malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma. These terms imply that the tumors recapitulate or originate from specific nerve sheath cells, such as Schwann cells, perineural fibroblasts, or fibroblasts .MPNSTs comprise approximately 5 percent of all malignant soft tissue tumors and have various origins. Most are derived from neurofibromas or arise de novo in normal peripheral nerves. Schwannoma, ganglioneuroma/ganglioneuroblastoma, or pheochromocytoma origin is infrequent. The majority of MPNSTs show some evidence of Schwann cell differentiation . SynoviaPrimary osseous MPNSTs of long bones are rare, with two cases in the femur, two in the ulna, and one in the humerus. They arise from the myelinated intraosseous nerve fibers . The diaMPNSTs typically occur in patients between the ages of 20 and 50 years and are almost equally distributed between the sexes. MPNSTs, both solitary and associated with NF, are rare in children. The mean age of patients diagnosed with these tumors related to NF is lower than that of patients with sporadic MPNSTs. The peak incidence of MPNSTs in NF is during the third decade of life. The peak incidence of sporadic MPNSTs is during the fifth decade of life .Helpful findings for diagnosis include an origin from the nerve, intraneural (intrafascicular) spread within or beyond the primary tumor mass, ganglion involvement, and identification of an associated solitary or plexiform neurofibroma. The majority of MPNSTs are composed primarily of spindle cells. Infrequently, bundles of such cells may show striking variations in cell density, imparting a "tapestry" appearance. The elongated tumor cells often feature hyperchromatic nuclei, are mitotically active, and possess moderate amounts of faintly eosinophilic cytoplasm. Nuclei generally have rounded or tapered ends but are not blunt. In less cellular areas, they sometimes have a wavy contour ,9.On IHC, MPNSTs are focally positive for so-called neural markers such as S100 protein, Leu-7, neurofilaments, and myelin basic protein. Although IHC evidence of neural differentiation is documented in more than 50% of patients, it is typically only focal. Neuron-specific enolase cannot be used alone as evidence of neural differentiation because it is frequently positive in many other lesions. SOX10, which is a neural crest transcription factor is expressed in the majority of MPNSTs, including those which are S100 protein negative. In solid uniform positivity for S100 protein cases, other spindle-cell neoplasms (primarily cellular schwannoma) should be excluded before the diagnosis of MPNST is rendered . This tuSurgical resection is the main treatment option for MPNST, aiming to obtain a 1-cm margin of clear tissue in all directions. Postoperative external beam radiation therapy is the most popular adjuvant radiotherapy approach; it is used to enhance local control, preserve tissue and function, and reduce local recurrence incidence. Based on 18 trials , chemothThis is a case of an intraosseous MPNST of a long bone with a rare site of origin. They usually occur in the mandible, spine, and maxilla; it has only been reported once in the humerus before. Our patient presented with shoulder pain. Even though radiologic findings are often unspecific, and the humerus is an unusual primary site, MPNST should be considered as a differential for prompt management. MPNSTs are highly aggressive, and the treatment for peripheral nerve tumors is surgical resection with or without adjuvant chemotherapy.


Gangliocytic paraganglioma (GP) is considered a rare neuroendocrine tumor (NET) most often located in the distal half of the duodenum. Insufficient reports describe tumors of this histological type located in the distal parts of the spinal canal, the conus medullaris and cauda equina. To date, nine cases of GP of the cauda equina and one case of GP of conus medullaris have been described. After analyzing all available treatment reports of GP, a study described it as a tumor with an extremely good prognosis in cases of total tumor removal. Here, we present a case of a female patient with a GP at the level of the L4 vertebra treated at Burdenko Neurosurgical Center using a minimally invasive approach through a tubular retractor. The tumor was removed en bloc through an intralaminar opening, and the patient was discharged two days after surgery with total regression of symptoms. Gangliocytic paraganglioma (GP) is considered a rare neuroendocrine tumor (NET) consisting of three main components: epithelioid, ganglion-like, and spindle-shaped (Schwann-like) cells .GP was first described as “ganglioneuroma” by Dahl et al. in 1957, and Kepes et al. coined the term “gangliocytic paraganglioma” in 1971 . DespiteIn this article, we describe the first case of resection of a gangliocytic paraganglioma in a 55-year-old patient using a minimally invasive spine surgery (MISS) through a tubular retractor.A 55-year-old female was referred to the Burdenko Neurosurgical Center by her primary care physician with complaints of severe lumbar pain radiating to both lower extremities along the lateral surface of the thigh and shin. About a week before admission, the patient noticed an increase in urination frequency and sensation of incomplete emptying of the bladder. On neurologic examination, no decrease in lower limb muscle strength or sensory disturbances were found. From the patient’s history, it became known that she experienced pain for about 1.5 years, for which she was treated conservatively with moderate to minimal improvement. Gradually, the symptoms progressed, and conservative pain management became ineffective.The patient’s magnetic resonance imaging (MRI) scan of the lumbar spine revealed an intradural tumor at the level of the L4 vertebra, hyperintense in the T1-weighted image, hypointense in the T2-weighted image, and actively accumulating contrast, 1.35 × 1 cm in size  using a tubular retractor system.Surgery was performed the next day after admission. The patient was positioned prone on a Wilson frame, and the L4 level was identified on lateral fluoroscopy. A 2-cm skin incision 3 cm off midline was made, after which a transmuscular approach was performed using sequentially placed dilators. We used an EasyGo tubular retractor, which was inserted after the dilators, its position verified using lateral fluoroscopy and fixed to the operative table  . In a stIn this regard, being an extremely rare tumor, GP remains poorly studied. In their study, Shankar et al. tried to explain how three types of cells , and ganglion-like) can occur in a single tumor and noted that the combination of cytokeratin-positive neuroendocrine, ganglion, and Schwann cells suggests that all these cell types are of neoplastic origin . On thisOkubo et al. collected all available reports of GP and analyze treatment results . It is wIt is worth emphasizing that for the patient, three months after surgery, no tumor recurrence was noted on the postoperative control MRI. We summarized the available data on surgical treatment of GP in Table Speaking about the choice of surgical approach, most recently, in their study, Lal et al. discussed the preference of laminotomy over laminectomy to prevent the formation of instability and other orthopedic problems at the level of the affected area of the spinal column . Helal eIn our experience, patients with cauda equina root tumors, with the proper experience and skill of the surgeon, can and should be operated on using a minimally invasive approach (MISS) due to their best orthopedic postoperative outcome, which is no less important for patients.


Mediastinal masses can present as a medical emergency when there is central airway obstruction, superior vena cava (SVC) syndrome, direct mediastinal extension of tumor, or obstruction of the central pulmonary vasculature. Diagnostic evaluation may include the need for invasive tissue biopsy under anesthesia, which can pose several distinct risks for patients. Among the many etiologies of mediastinal tumors, primary mediastinal germ cell tumors are a rare form with a favorable prognosis. Mediastinal masses historically were defined by their anatomic location in either anterior, middle, or posterior mediastinal compartments. The International Thymic Malignancy Interest Group (ITMIG) refined the classification of mediastinal tumor location into prevascular, visceral, and paravertebral compartments with boundaries that are more clearly defined on axial and sagittal CT or MRI imaging .Anatomic designation assists with determining the etiology of a mediastinal mass, although rare causes of mediastinal masses also exist. The location of a mass may also assist with the prediction of future symptoms or risk of airway or vascular compromise with the induction of anesthesia . Dependi2 of 85% on room air. He had labored breathing and remained in an upright tripod position as his breathing worsened while recumbent. Physical examination revealed bilateral lung wheezes, jugular venous distension, and prominent dilated vessels with arm swelling in bilateral upper extremities consistent with superior vena cava (SVC) syndrome. The patient was able to tolerate a rapid CT scan, which revealed a partially calcified large mediastinal mass measuring 16 cm × 15 cm × 8 cm with critical compression of the distal trachea and carina down to a 4 mm diameter airway along with extrinsic compression of right-sided pulmonary arteries  was elevated at 1,079 ng/mL , and quantitative beta human chorionic gonadotropin (hCG) level was 9 mIU/mL . Testicular examination was normal, and ultrasound of his testes did not reveal any nodules or other abnormalities. He was diagnosed with a primary mediastinal non-seminomatous germ cell tumor (NSGCT) yolk sac subtype, and treated with four cycles of cisplatin, etoposide, and ifosfamide chemotherapy with dramatic reduction in tumor burden. SVC syndrome resolved within initial cycles of chemotherapy, and tracheal stent was removed without difficulty three months later with complete resolution of airway stenosis Figure .Mediastinal masses can cause symptoms due to airway compression, SVC syndrome, direct mediastinal invasion, pulmonary vasculature obstruction, pericardial effusions, paraneoplastic syndromes, direct mediastinal invasion, and compression of the recurrent laryngeal nerve ,2. PatieThe elevated AFP serum markers and imaging were suggestive of a germ cell tumor, which was confirmed on pathology as a yolk sac non-seminomatous germ cell tumor. Primary mediastinal NSGCTs are analogous to non-seminomatous germ cell tumors of the testes but occur in the absence of any testicular pathology. Such tumors are believed to originate from precursor germ cells, which are retained within the primitive ectoderm instead of normal migration into the urogenital ridge during embryogenesis . AnotherMediastinal masses can present with symptoms of wheezing, cough, or dyspnea from central airway compression, hoarseness from recurrent laryngeal nerve impingement, SVC syndrome, central vascular obstruction, or direct mediastinal invasion. Successful perioperative management of patients with mediastinal masses requires planning to prevent airway and central vascular compromise with the induction of anesthesia. Primary mediastinal germ cell tumors are rare but carry a much more favorable prognosis and may be suggested by serum tumor marker elevation, as well as calcifications within mediastinal masses on CT scan.


Most hypopharyngeal disease presentations involve tumours or growths of various cellular origin. These lesions may include choristomas, hamartomas, hyperplasia, and harmless or dangerous neoplasms. A hamartoma is a benign focal malformation of tissue at the site of origin that lacks specific distinguishing features and is often designated as a neoplasm without consideration of pathology or biological behaviour. Here, we discuss the case of a 56-year-old man who presented with gradual dysphagia for 4 months along with sleep apnoea and voice changes. No palpable neck masses or abnormal lymphadenopathy was found. A left-sided homogeneous cyst-like mass was seen in the piriform fossa on laryngoscopy. The vocal cords were visualized and mobile. A contrast-enhanced computed tomography scan showed the obliteration of the piriform sinus on the left side by a faintly enhanced heterogeneous soft tissue lesion. The lesion originated from the left aryepiglottic fold, and was approached via micro-laryngoscopy and excised. Histopathological analysis led to a diagnosis of lipomatous hamartoma. Hamartomas are tumour-like malformations composed of tissue elements specific to the organs in which they occur. The word hamartoma is derived from “hamartia,” meaning error, and it indicates a defect and local developmental disorder rather than a true neoplasm.Head and neck hamartomas are rare but have been found in the sinuses, nasopharynx, oral cavity, oropharynx, larynx, hypopharynx, cervical oesophagus, ears, parotid glands, trachea, parathyroid glands, and eyes. Hypopharyngeal hamartomas are extremely rare lesions, with very few cases documented in the medical literature. Additionally, their pathology is not well explained, or histological documentation is lacking.Head and neck hamartomas can markedly differ in appearance depending on their location. However, laryngeal and pharyngeal hamartomas may be detected incidentally in asymptomatic patients, or may cause dysphagia, hoarseness of voice, and stridor when large.The management of head and neck hamartomas depends on their location. However, hypopharyngeal hamartomas are usually adequately treated through excision via micro-laryngoscopy. Open surgery is rarely required for large lesions.Hamartomas of the hypopharynx are very rare. To better understand this disease, we report a case of hypopharyngeal hamartoma in a 56-year-old man who presented with dysphagia, changes in voice, and apnoea. The case is noteworthy because a provisional preoperative diagnosis could be made only through imaging, and led us to proceed with surgery through a conservative transoral route rather than an external approach.A 56-year-old man who was an ex-smoker with a history of diabetes mellitus presented to our clinic with dysphagia to solid foods that had gradually increased in the prior 4 months. The dysphagia was accompanied by changes in voice, difficulty in breathing, and apnoea that was more prominent when the patient lay on his right side; he was more comfortable when lying on his left side or back. The patient underwent a thorough clinical examination, and a flexible fibre optic laryngeal examination indicated a smooth-surfaced polypoid mass originating from the left piriform fossa in the hypopharynx. His vocal cords were visualized and mobile.A computed tomography scan with contrast showed obliteration of the piriform sinus on the left side by a faintly enhanced heterogeneous soft tissue lesion measuring 2.5 × 2.4 × 1.2 cm in craniocaudal view, on the basis of transverse and anterior-posterior diameters . There wThe patient underwent micro-laryngeal surgery to excise the left piriform fossa mass under general anaesthesia, and specimens were sent for microscopic analysis. Histological examination showed a non-encapsulated lesion measuring 1 cm in its largest dimension, formed from a mixture of mature fatty tissue, fibrous tissue, and benign lymphoid tissue, with formation of lymphoid follicles within germinal centres. On the basis of the microscopic findings, a diagnosis of lipomatous hamartoma was made .Figure 3The patient improved after surgery. In the next follow-up visit after 6 months, the patient presented with a foreign body sensation in his throat and recurrent voice changes. On examination, there was no palpable mass in the neck. Repeat laryngoscopy showed mobile vocal cords and a left piriform cystic swelling prolapsing on the glottis . A second surgery was performed, and the mass was excised completely and was found to measure almost 5 cm in the largest dimension. Four months later, the patient appeared to be doing well and reported no changes in voice, dysphagia, or difficulty in breathing. Laryngoscopy examination indicated a normal-looking hypopharynx and larynx. Three years later, he presented again with the same prior complaint of a foreign body sensation in his throat and voice changes. On examination with laryngoscopy, a recurrent left-sided mass was seen in the piriform fossa; the rest of the examination was unremarkable. A third surgery was performed, and the recurrent left piriform fossa mass was excised. Its microscopic characteristics were similar to those of the previous specimens. The mass measured approximately 3 cm and was excised completely. The gross surgical specimen is shown in ,We present a rare case of lipomatous hamartoma in a 56-year-old man with complaints of dysphagia and dyspnoea due to a piriform fossa mass, as confirmed by histopathology findings. Hamartoma of the hypopharynx is an extremely rare, non-malignant non-neoplastic tumour: only ten cases with published histology have been reported in children and adults in the literature.,Several investigative modalities are used to examine tumours in the larynx. Although clinical examination is usually performed first, it can be limited by the position and size of the mass. Laryngoscopy can aid in examination of lesions. Simple radiography is less helpful in characterizing lesions but may show a lobulated mass in as many as 50% of patients.Lipomatous hamartoma in the hypopharynx has been found to have better prognosis than hamartomas in other parts of the body.Notably, our patient had diabetes and was an ex-smoker. Nevertheless, owing to a lack of data, we found no association between diabetes or former smoking and head and neck hamartomas recurrence. Our patient underwent three surgeries within 5 years of presentation and recovered completely after each surgery. Recurrence of lipomatous hamartoma was suspected when he presented again with changes in voice and a globus sensation in the throat.In our case, advances in imaging techniques allowed us to establish a preliminary diagnosis that led us to proceed transorally rather than following a more aggressive approach via lateral pharyngotomy, as described by Patterson et al.We report a unique case of a lipomatous hamartoma in the hypopharyngeal space that presented with dysphagia and dyspnoea. Advances in imaging techniques allowed us to establish a preliminary diagnosis and eliminate the need for a prior lesion biopsy. Histopathological studies confirmed the diagnosis. Subsequent follow-up and vigilance are necessary in such cases because of their rare nature and the importance of the location of occurrence. Inadequate resection and recurrence are common, but the likelihood of a complete recovery after recurrence is high.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.The authors have no conflicts of interest to declare.Ethics approval was exempted by the institutional review board of King Abdullah International Medical Research Center (KAIMRC), Riyadh, KSA. .The need for informed consent was waived, because no information identifying an individual person was included.The need for informed consent was waived, because no information identifying an individual person was included.KAA and BAA wrote initial draft of the article. AAA conceptualized the work, supervised, and wrote the final draft of the article. ALK contributed to the histopathology description. MAA contributed supervision, revised the article, and approved the final version. All authors have read and approved the final manuscript. All authors have critically reviewed and approved the final draft and are responsible for the content and similarity index of the manuscript.


Hyperinsulinemic hypoglycemia is most commonly caused by a single, sporadic insulinoma. Multicentric insulinoma disease (insulinomatosis) as well as metachronous neuroendocrine tumors of the pancreas, known also as neuroendocrine adenomatosis, represent a very rare condition, if not associated with multiple endocrine neoplasia type 1 syndrome (MEN1) or Von Hippel Lindau disease. We report a 9-year follow-up of a 41-year-old woman, initially presenting with hypoglycemic syndrome caused by two insulin-producing tumors, who underwent subtotal pancreasectomy in 2012, with histology compatible with multiple small neuroendocrine tumors. An approximately 1-cm insulin-producing tumor recurred at subsequent biochemical and radiological follow-up, and was cured with the somatostatin analog octreotide as a single treatment, until remission of symptoms and complete regression of the pancreatic lesion achieved after only 16 months of treatment. The possible mechanisms for these findings are discussed and the literature is briefly reviewed. Insulinomas are the most frequent cause of hyperinsulinemic hypoglycemia. They are usually benign sporadic tumors, but in about 10% of patients they may be associated with hereditary diseases, such as multiple endocrine neoplasia type 1 (MEN1) , 3. At pMedical therapy for patients with neuroendocrine neoplasms (NEN), including insulin-producing ones, is aimed at decreasing the circulating hormones which cause typical syndromes and control of tumor growth.Approximately 50% of insulinomas express somatostatin receptors on their cells’ surface, mainly receptor subtypes 2 and 5. The somatostatin analogs octreotide and lanreotide are able to inhibit the signal-transmission pathways by binding these receptors [We report a case of hyperinsulinemic hypoglycemia caused by multiple recurrent small insulin-producing tumors which recurred after surgery and responded to somatostatin analog treatment with complete remission of symptoms and regression of the pancreatic lesions.A 41-year woman presented as an outpatient at our Hospital in November 2012 for her annual thyroid condition follow-up (simple goiter associated with autoimmune chronic thyroiditis), complaining of neurological symptoms such as poor cognitive abilities and decreased alertness over the previous two years, sometimes associated with sweating and sudden hunger. During one of these episodes, the laboratory workout revealed low capillary blood glucose levels (36 mg/dl). On the suspicion of hypoglycemic syndrome, the patient was admitted to the Endocrine Unit of Forlì Hospital. At the physical examination her weight was 70 kg and BMI 24.5; blood pressure was 110/70 mmHg and no abnormalities were found at abdominal, cardiac, pulmonary and neurological examination. Other causes of hypoglycemia were also excluded upon admission, namely exogenous insulin and oral hypoglycemic agent administration, as well as insulin autoimmune syndrome (Hirata disease), as insulin autoantibodies were negative.A supervised 72-h fasting test was performed, showing low venous glucose values , parathormone, serum calcium, insulin-like growth factor 1, as well as all pituitary hormones, i.e. prolactin, thyroid-stimulating hormone, adrenocorticotrophic hormone, luteinizing hormone, follicle-stimulating hormone and growth hormone, were normal in two different determinations. Additional diagnostic evaluations for MEN1 syndrome, such as parathyroid scintigraphy and magnetic resonance imaging (MRI) of the sella turcica, were also negative.In December 2012, subtotal pancreasectomy was performed, and detailed histopathological examination revealed 2 tumors measuring 16 and 10 mm in the pancreatic body-tail along with several other small tumors , computed tomographic scanning demonstrated a 1 cm tumor in the body of the pancreas, showing enhancing features typical of neuroendocrine lesions. Hence, a 68 Ga-DOTATOC positron emission tomography was performed, showing moderate uptake of the radioligand at the level of the above-described lesion. An FDG positron emission tomography was also performed which showed no uptake in the pancreas or other abdominal and extra-abdominal sites. Pancreatic ultrasound endoscopy confirmed the presence of a 13 mm tumor located in the body of the pancreas.In January 2014, another supervised 72-h fasting test was performed at the Endocrine Unit of Forlì Hospital, showing low venous glucose values , medical treatment with the somatostatin analog octreotide (30 mg given intramuscularly every 28 days) was started and regularly administered during the subsequent follow-up.In the following 6 months, the patient did not show any symptoms or signs of hypoglycemia and the treatment with octreotide was tolerated without particular side effects. The plasma levels of CgA, glycemia and insulin were within the normal range. The abdominal MRI performed in July 2014 showed a 12 mm angioma of the liver and apparently no lesions of the pancreas.In November 2015, a CT scan of the abdomen was performed, showing no lesions within the pancreas and confirming the presence of a stable angioma of the liver.A second opinion was given by the Oncological Centre of Milan, confirming the treatment with somatostatin analogs, which was eventually discontinued in September 2016, because of the patient’s desire for pregnancy.A 68 Ga-DOTATOC positron emission tomography performed in September 2018 showed no uptake of the radioligand.In October 2018, pancreatic ultrasound endoscopy confirmed the absence of lesions in the head-body of the pancreas.In the following years until April 2021, the patient continued with regular 6-month clinical check-ups, including measurements of basal glycemia, insulin, C-peptide and CgA, which were always unremarkable, and annual imaging techniques, both with CT scans of the abdomen Fig.  and MRI In April 2021 the patient was admitted to Endocrine Unit of Forli Hospital in order to once again perform a supervised 72-h fasting test, about 9 years after the first one: the venous glucose values were never below 45 mg/dl and the levels of insulin (4.5 mU/l) and C-peptide (0.26) measured at the lowest sugar levels were not consistent with recurrency . Hypoglycemic episodes occur mostly during fasting periods and sometimes during the post-prandial phase . In ordeAnother interesting and very rare pathological condition in our case is the presence of pancreatic neuroendocrine microadenomatosis and, more specifically, insulinomatosis. This is a neoplastic pancreatic disease defined by the presence of multiple small and large insulin-producing tumors which develop simultaneously or metachronously . The recPancreatic neuroendocrine lesions equal to or less than 0.5 cm are defined neuroendocrine microadenomas. Multiple neuroendocrine microadenomas are called neuroendocrine adenomatosis. The prevalence of this condition is very low and may occur sporadically or associated with the above-mentioned genetic syndromes as for insulinomatosis . As for Somatostatin receptors are expressed on the cell surface of approximately 80% of NENs and 50% of insulinomas, allowing somatostatin analogs octreotide and lanreotide to bind to receptor subtypes 2 and 5 . Thanks Somatostatin analogs are the second-line medical treatment for controlling hypoglycemia in patients with insulinomas, mainly for malignant insulinomas with recurrent hypoglycemic events. Octreotide was shown to be effective in controlling hypoglycemia in 59% of patients with insulinomas . Other mAlthough somatostatin analogs were developed primarily for reducing the levels of circulating hormones causing typical endocrine syndromes, the results of PROMID  and CLARInterestingly, in our case not only was there a complete remission of the symptoms after starting the somatostatin analog octreotide, but the antiproliferative effect on the 10 mm lesion of the pancreas was also observed. As a matter of fact, no lesions were visible at abdominal CT scan or MRI, nor at endoscopic ultrasonography, which has a very high diagnostic accuracy of about 98%, after a total of 16 months of treatment.The possible mechanisms of this unexpected achievement with biotherapy in our patient have to be sought in the quite exceptional overexpression of somatostatin receptor type 2, with a density of about 95% on the neoplastic surface cells, as demonstrated by immunohistochemistry examination Fig.  and at 6We therefore believe that further studies should investigate the role of biotherapy with somatostatin analogs in newly-diagnosed primary well-differentiated neuroendocrine tumors for clinical practice.In summary, we describe a rare case of a patient with hypoglycemic syndrome caused by multiple neuroendocrine tumors of the pancreas (insulinomatosis). The response obtained by antineoplastic treatment resulted in tumor load decrease up to complete remission of the disease; the use of somatostatin analogs in treating single or multifocal insulinomas as well as functioning and non-functioning neuroendocrine multiple subcentimetric tumors should thus be considered as a pharmaceutical option, when surgery is not possible or surgical eradication is not achieved.


Congenital brain tumors are extremely rare; even with advances in prenatal imaging tests, it is still uncommon. Congenital glioblastoma (GBM) is a rare childhood tumor. With less than 50 cases described in the literature, it corresponds to less than 2% of tumors in children under two months of life. Moreover, it has a markedly poor prognosis due to the risk of intracranial hemorrhage, especially during surgical resection. This study reports the case of a 20-day-old asymptomatic child who presented with increased head circumference during a pediatric routine check-up. A transfontanellar ultrasound was performed, exhibiting hydrocephalus, large parenchymal hemorrhage, and expansive formation. Magnetic resonance imaging pointed to a massive infiltrative lesion, with heterogeneous enhancement, delimiting central areas of necrosis with hematic material inside, associated with a compressive effect on the adjacent parenchyma. Additional histopathological analysis, immunohistochemistry, and DNA methylation test confirmed the diagnosis of GBM. The patient was submitted to surgical intervention and chemotherapy, achieving a 26-month-old survival by the time this study was written. Congenital brain tumors are very rare; even with advances in prenatal imaging tests, it is still uncommon . CentralGlioblastoma (GBM) multiform  classification of brain tumor) is the most frequent malignant brain tumor within the adult population . On the Usually, it is diagnosed during the perinatal period via routine fetal ultrasound around 28-39 weeks .According to a systematic review performed by Hou et al., 40% of 35 patients were stillborn or expired within one week of life, and the mortality rate at two months of age increased to 60%. Patients who were submitted to surgical intervention with or without adjunctive therapy had a mean survival time equal to or above 96.5 weeks. The long-term prognosis is poor due to the risks of intracranial hemorrhage, especially during surgical resection [This case report was previously exhibited as an oral presentation at the 2021 American Society of Neuroradiology Annual Meeting Congenital on May 26, 2021.A 20-day-old child, born full-term without any complications, was asymptomatic when an abnormal increase in head circumference was verified during a pediatric routine check-up. A transfontanellar ultrasound was performed and hydrocephalus, extensive parenchymal hematoma, and expansive formation were observed. Magnetic resonance imaging (MRI) was performed to optimize the evaluation of the findings. MRI revealed intra-axial, temporoparietal expansive formation, with areas of low signal in the gradient sequence , as well as areas of high signal on T2 (compatible with cystic degeneration and/or necrosis), circumjacent vasogenic edema, with heterogeneous and predominantly peripheral enhancement after contrast administration, affecting the left occipital lobe, measuring approximately 6.8 cm craniocaudal (CC) × 6.0 cm anteroposterior (AP) × 5.8 cm laterolateral (LL). It exerted a marked mass effect, characterized by compression of the corresponding lateral ventricle posterior horn as well as the ipsilateral midbrain. In addition, there was moderate ectasia of the supratentorial ventricular system Figures -3. ImmunThe patient was submitted to surgery, followed by histopathological analysis, which suggested a dysembryoplastic neuroepithelial tumor, but requested additional immunohistochemistry to aid in the diagnosis. The panel result was positive for glial fibrillary acidic protein , integrase interactor 1 (MRQ-27), S-100 , CD34 , and Ki67 (clone 30-9) score was 70%. Synaptophysin was negative (clone SY38). Additionally, DNA methylation was executed, and, together, the results of these tests were compatible with GBM.After surgery, the patient was placed on chemotherapy. The expansive lesion enlarged since the first MRI, reaching 12.0 cm × 9.6 cm × 11.0 cm (CC × AP × LL) by 25 days post-operation, and regressed to 9.0 × 8.9 × 5.8 cm (CC × AP × LL) eight months posterior to surgical intervention and chemotherapy.Congenital GBM can be classified according to the time of diagnosis into three groups: definitely congenital, probably congenital, and possibly congenital, proposed by Solitare and Krigman ,11,12. OPatients diagnosed with GBM after birth frequently detain symptoms of increased intracerebral pressure. In our patient’s case, the clinical symptoms that predominantly raise suspicion of altered intracranial pressure include the abnormal head circumference, followed by lethargy and vomiting . RegardiBefore performing the MRI, the patient was submitted to a transfontanellar ultrasound. The anomalies observed were not discordant to what has been published in case reports. Identification of a heterogenous mass sometimes associated with hemorrhage and/or hydrocephalus are the primary sonographic findings of GBM ,5,10.MRI showed voluminous infiltrative lesion, with heterogeneous enhancement, delimiting central areas of necrosis, as well as the presence of an internal hematic substance, involving a large area of the left cerebral hemisphere, associated with substantial mass effect of the adjacent parenchyma Figures -3. TheseOur patient continues chemotherapy, with steady lesion progression and stable clinical condition, with a 26-month survival. Routinely, the overall prognosis is quite poor, with some studies stating an average survival of two months, even with chemotherapy and maximum surgical resection being performed ,14,16. WImprovements in perinatal care over the past few years have enabled premature diagnosis. Nevertheless, this advance was not reflected in clinical management, mainly because of this condition’s rarity, which limits studies and consequently the development of treatment protocols focused on improving patients’ survival rates and quality of life. A lot remains to be done to be sure of what can truly benefit patients. Therefore, bringing another case to light may help foster future research initiatives.