However, alpha-fetoprotein, beta-human chorionic gonadotropin, human placental lactogen, CK20, tumor suppressor protein 53, and Kirsten rat sarcoma were negative. This article describes the clinical and histological features of rare sellar masses as well as survival after therapy.Mature cystic teratoma (MCT) is a benign germ cell tumor, histologically comprising components derived from mesoderm, ectoderm, and endoderm layer tissue. MCT usually has foci of intestinal components and colonic epithelia. Pituitary teratomas containing complete colon features are very rare. Here, we present three cases of sellar teratoma in two men aged 50 and 65 years and a woman aged 30 years. All patients presented with asthenia, adynamia, and loss of strength. A pituitary mass was incidentally observed on magnetic resonance imaging. Histological features showed a mature teratoma formed by gut and colonic epithelium, extended lymphoid tissue with the formation of Peyer’s patches, and muscular layer vestiges with a fibrous capsule. The immunohistochemical panel showed reactivity to cytokeratin (CK)7, CKAE6/AE7, carcinoembryonic antigen, octamer-binding transcription factor 4, cluster of differentiation (CD)20, CD3, vimentin, muscle actin, and  Pituitary adenomas are among the most common central nervous system (CNS) tumors, representing an estimated 10-15% of all CNS tumors, and are the cause of approximately 25% of all surgical resections for these tumors . The mosHere, we report three rare cases of mature cyst teratoma (MCT) that clinically mimicked a pituitary non-functional adenoma and histologically revealed colonic and gut features.Case oneA 65-year-old man presented to the institute with a two-month history of fatigue, nausea, vomiting, headache, drowsiness, limb weakness that caused an inability to walk, general loss of strength, physical deterioration, visual disturbances, and progressively developing amaurosis. He had suffered from alcoholism since the age of 34. Serum levels of AFP and carcinoembryonic antigen (CEA) were normal. Magnetic resonance imaging (MRI) showed meningeal hyperintensity as well as a homogeneous hyperintense mass in the sellar region with three well-rounded lobes that followed the medial line confined to the subventricular space causing ventriculomegaly. It was diagnosed as a non-functional pituitary macroadenoma. A transsphenoidal procedure was performed with adjuvant radiotherapy. He was discharged with no complications, and no follow-up was done. Imaging findings are illustrated in Figure Case twoA 50-year-old man presented to the institute with a four-month history of the same clinical presentation as case one. Serum levels of thyroid hormone were low with normal levels of AFP and CEA. The background was not relevant. MRI in T1 with gadolinium showed a diffuse heterogeneous sellar mass with a cyst-like appearance and a hyperintense capsule-like structure surrounding it. He was also diagnosed with a non-functional pituitary macroadenoma. A transsphenoidal procedure was performed, with no other therapy. He expired from neurological complications six months later. Imaging findings are illustrated in Figure Case threeA 34-year-old woman presented to the institute who six months after delivery complained of an inability to breastfeed, visual disturbances, headache, amenorrhea, drowsiness, depression, progressive loss of strength, and limb weakness that caused difficulty walking and standing. Serum prolactin levels were elevated, with normal AFP and CEA levels. MRI and computed tomography (CT) showed a sellar mass with multiple hypointense and hypodense areas, respectively, with different densities in its content and walls that limited a hyperintense content within a solid component. She was surgically intervened. Unfortunately, brain surgery was complicated by pneumonia and she succumbed 10 days later. Imaging findings are illustrated in Figure We present three cases of sellar teratomas in two men and one woman. All presented with headache, muscle weakness, and loss of tone and strength within a short duration (two to six months). The three patients had large tumors and showed heterogeneous imaging patterns, with one being cystic. Only case one received radiotherapy. No autopsy was performed. Results are presented in Table Histological features of all cases showed a tumor formed by ectodermal, mesodermal, and endodermal components, with predominant colonic epithelial forming cyst structures Figure . MoreovePituitary tumor transforming gene-1 (PTTG-1) was also positive in small cells mixed in the inflammatory layer 20 and negative for cytokeratin (CK)7 Figure . CEA wasr Figure . MCT wasTeratomas occur more frequently in children and young adults than in the older population and are more predominant in males compared to females. Teratoma is histologically formed by three germ cell layer tissues, namely, endoderm, ectoderm, and mesoderm (most teratomas are overweight). They are classified as mature or immature subtypes . TeratomIn this article, we reported three cases of sellar teratoma in a 65-year-old man with a history of chronic alcoholism, a 50-year-old man, and a 34-year-old young woman with menarche at nine years, irregular menstrual cycles, and four deliveries. During her last pregnancy, she developed visual disturbances that subsided after delivery. It is noteworthy that she could not breastfeed. All patients presented with a very similar clinical picture of progressive fatigue until drowsiness, visual disturbances, chiasmatic syndrome, and a large sellar lesion classified as Harvey IIIC and D, respectively. This type of tumor is more frequent in young women than in men. In all cases, the anterior pituitary hormone levels were normal. The female patient presented with a post-surgical middle cerebral artery infarction and passed away. In our cases, this tumor was more aggressive in women than in men, as the woman passed away quickly after surgery.Teratoma diagnosis can be suggested through neuroimaging findings. MRI remains the preferred modality for assessment. As mentioned, neuroradiological findings are those of mixed cystic and solid components or inclusions of teeth, fat, and calcification. In our cases, cystic and solid structures with heterogeneous components not specific to fat, bone, or teeth were observed . NeverthThe ideal treatment for mature teratoma is neurosurgical excision ,5. HowevPituitary apoplexy (PAp) is a severe and hypothetically life-threatening medical emergency , categorTumor markers such as β-hCG and AFP can be useful for diagnosing teratoma as well as for distinguishing immature teratomas, mixed GCTs, and mature teratomas from immature or malignant components -4. In ouThe mature subtype has all mature elements, containing adipose tissue, hair, sebaceous glands, and stratified squamous epithelium. The immature subtype contains immature epithelial, neuroepithelium, mesenchymal elements, or blastemal. Depending on the degree of differentiation of its components, immature tumors are more likely to exhibit malignant transformation ,5. We reSpecific types of GCTs produce biological tumor markers such as AFP and/or hCG, and choriocarcinoma produces hCG. In teratomas, there are no specific markers, while they can express CK7 . SimilarKRAS and wild-type BRAF genes [PTTG has been detected in both functional and non-functional pituitary adenomas as well as in teratomas [Malignant changes or transformation and recurrence of mature intracranial teratoma are extremely rare. It is estimated that approximately 2% of all MCT cases show a malignant transformation. Squamous cell carcinoma is the most common type of tumor accounting for 83%, the rest are adenocarcinoma, sarcoma, and carcinoid tumors . AdenocaAF genes . ConditiAF genes . Similareratomas . PTTG-1 has been used to identify pituitary changes in teratomas [It is interesting to mention that cases of ovarian teratomas that present pituitary tissue and/or the combination of both teratomas with pituitary adenoma or normal pituitary tissue have been reported . PTTG-1 eratomas , as wellWe presented three rare cases of pituitary teratoma in patients of different ages and gender, but with the same symptoms. Only one case presented with endocrine disorders, associated with non-lactation and elevated prolactin. All patients had a rapidly evolving clinical picture and similar histological findings. Despite being benign, the prognosis for this tumor appears to be poor, as two patients deceased within a period of six months after therapy.


Bezoars are a rare complication causing small bowel obstruction. A phytobezoar causing terminal ileum obstruction following a Roux-en-Y gastric bypass (RYGB) is extremely rare. A middle-aged woman with post-sleeve gastrectomy weight regain, converted to RYGB, presented 17 months after surgery with obstructive symptoms due to an impacted phytobezoar in the terminal ileum. Diagnostic laparoscopy, enterotomy, and extraction of the large impacted phytobezoar from the terminal ileum relieved the obstruction. Swallowing improperly masticated food in altered gastrointestinal anatomy due to RYGB can cause a phytobezoar in any part of the gastrointestinal tract. These patients need proper nutritional counseling and psychological evaluation to prevent this rare complication. Phytobezoars, though rare, require prompt intervention when they cause small intestine obstruction. Bezoar formation after gastric bypass is extremely rare. Accelerated gastric pouch emptying insufficiently masticated food in altered gastrointestinal anatomy due to Roux-en-Y gastric bypass (RYGB) can cause a phytobezoar in any part of the gastrointestinal tract . Bezoars2 with comorbidities of hypertension, dyslipidemia, and back pain. She underwent laparoscopic sleeve gastrectomy in December 2014. She reduced her weight to 75 kg following a sleeve gastrectomy, then increased her weight to 98 kg and her BMI to 36.17 kg/m2, and developed reflux symptoms. Hence, she underwent RYGB in August 2017. During the RYGB, 30 ccs of gastric pouch were created, and 80 cm of biliopancreatic limb and 130 cm of roux limb were constructed. A gastrojejunostomy was created with a circular stapler, and a jejunojejunostomy (j-j anastomosis) was created with a linear stapler. Both the j-j anastomosis mesenteric defect and Peterson’s defect were closed. After the RYGB, she reduced her weight to 80 kg and BMI to 28.36 kg/m2, and her reflux symptoms resolved immediately after revision surgery. Postoperatively, she attended the bariatric clinic without any complaints.The patient was a 50-year-old Emirati woman with an initial weight of 132 kg and body mass index (BMI) of 46.80 kg/m3/µL) with absolute neutrophilia (8.3 × 103/µL). An X-ray of the abdomen showed small dilated bowel loops with multiple air-fluid levels  scan with contrast was performed, which showed dilated jejunal and proximal ileal loops with multiple air-fluid levels without passage of oral contrast to the distal ileum. A string of beads sign and a small bowel feces sign in the distal ileum suggested obstruction Figure .The CT scan findings were discussed with the patient, and an emergency diagnostic laparoscopy was planned. During the diagnostic laparoscopy, the gastric pouch was found to be of normal size, but there was distention of jejunal loops to the distal ileum. The j-j anastomosis was dilated but patent. In the distal ileum, there was a transitional zone around 40 cm from the ileocecal junction with an intraluminal globular mass obstructing the distal ileum Figure . With a The patient’s postoperative recovery was smooth. She started tolerating liquids from the first postoperative day, progressed to a soft diet on postoperative day two, and was discharged from the hospital on the third postoperative day. When we spoke to her during the postoperative period, she admitted to consuming orange segments directly without proper chewing three days before while in the office during her busy working hours. She was advised to chew her food properly before swallowing to prevent such a complication in the future. She was also advised to regularly follow up with the bariatric and dietician clinics.Post-gastric bypass patients need specific diet plans in the preoperative and early and late postoperative phases. They should strictly abide by the diet plan and visit a dietician for any change or modification according to their weight loss pattern in the postoperative phase. The post-RYGB weight loss pattern and any development of complications depend on the patient’s discipline and consistency in her diet quality and quantity.Immediately after surgery, post-gastric bypass patients should follow their dietician’s plans and should be limited to liquid diets only, then progressing to a puree diet. After one month, they should start a soft diet, with proper mastication. After surgery, the newly formed gastric pouch restricts intake, and the patient should be careful while eating and drinking. Once they understand and get familiar with the eating pattern, it becomes easier for them to swallow food of different textures. Usually, after six months to one year, they can eat every type of food. Hence, this complication is prone to occur after six months of surgery if the patient swallows food without proper mastication.Bezoars are retained undigested bolus materials in the gastrointestinal tract. A bezoar predominantly consists of food (phytobezoar) or hair (trichobezoar). Factors contributing to the development of phytobezoars include high-fiber food rich in cellulose, less gastric acid from the small stomach pouch after RYGB, improper mastication, and delayed gastric emptying post-RYGB . In our Internal hernia is the most common cause of small intestinal obstruction following RYGB -5, folloTreatment options for phytobezoars include chemical dissolution, upper gastrointestinal endoscopic fragmentation and extraction, or surgical intervention. Patients with acute obstruction with an impacted bezoar in the distal small bowel usually need laparoscopic intervention or, if failed, conversion to open exploration. For a phytobezoar in the distal ileum, an initial attempt of intraluminal fragmentation by external compression, milking through the ileocecal valve, to relieve the obstruction should be made to prevent enterotomy . If thisPost-gastric bypass patients need regular follow-ups with a nutritionist regarding their diet plan and their dietary habits. On enquiring in the postoperative period, this patient accepted consuming orange segments without proper mastication, three days before developing obstructive symptoms, which emphasizes the need for regular follow-ups with a dietician to discuss their eating habits and any modification of diet plans according to their job demand. Patients also need regular follow-ups with a psychologist for assessment and counseling to adjust to their changed lifestyle and environment.Although phytobezoars are a rare complication, they should be considered a differential diagnosis in post-gastric bypass patients presenting with obstructive symptoms. Going through the literature, this is the first reported case of a phytobezoar causing small bowel obstruction following revision bariatric surgery. For any suspicion of obstruction, diagnostic laparoscopy should be maintained as a prompt intervention option to rule out any other extrinsic cause of obstruction. Radiologically imaging and diagnosing phytobezoars is key to early intervention ,11.Phytobezoars are a rare cause of small bowel obstruction following revision gastric bypass. They should always be suspected in patients presenting with a small bowel obstruction following RYGB due to their anatomical alteration and change in dietary habits. Laparoscopic management of bezoar-induced intestinal obstruction by experienced surgeons is feasible with early recovery. These patients need regular nutritional counseling and psychiatric evaluation to prevent further bezoar formation.


Endodermal sinus tumors are rare, especially extragonadal endodermal sinus tumors, which often occur in the midline of the brain, neck, chest, and abdomen.We present the case of a 37-year-old woman with a mass on the right edge of the liver. Color Doppler ultrasound, computed tomography, and magnetic resonance imaging examinations were performed before the operation. Given these results and the elevation of alpha-fetoprotein, the diagnosis of hepatocellular carcinoma was made. Postoperative pathological examination indicated an endodermal sinus tumor on the abdominal wall near the right liver. The causes of misdiagnosis were analyzed, and the related literature was reviewed.Primary endodermal sinus tumors on the abdominal wall near the right liver are easily misdiagnosed as hepatocellular carcinoma due to liver compression and elevated alpha-fetoprotein. The key point of differentiation is the wide basal connection between the tumor and the abdominal wall near the liver. In addition, the enhancement mode of endodermal sinus tumors is different from the enhancement pattern of hepatocellular carcinoma.Extragonadal endodermal sinus tumors often occur in the midline of the body. Here, we present a case of a primary endodermal sinus tumor on the abdominal wall near the right liver for the first time. An endodermal sinus tumor  is a primitive malignant germ cell tumor that is histologically similar to the mesenchyme of the primitive yolk sac , 2. It rPhysical examination showed that alpha-fetoprotein increased over 3 months, and a liver mass was found 0.5 months earlier.Half a month ago, the patient developed persistent low back pain for 1 day without inducement.The patient had no personal or family history.There was no specific finding on physical examination.Alpha-fetoprotein (AFP) was 206.2 ng/ml.Color Doppler ultrasound showed a strong echo with a range of 3.5 cm × 2.4 cm on the right edge of the liver. No obvious blood flow signal was found (The computed tomography (CT) scan showed a round, low-density mass on the right edge of the liver, with a size of approximately 3.1 cm × 1.9 cm. On contrast-enhanced images, non-enhanced liquefaction and necrotic zones were found in a cystic-solid mass. The solid part of the enhancement was located on the periphery of the mass, with irregular strips showing progressive and obvious enhancement. As time went on, the enhancement range of the mass expanded to the center. The CT value of the solid part was approximately 42 HU in the plain CT scan, approximately 90 HU in the arterial phase, approximately 111 HU in the portal vein phase, and approximately 107 HU in the solid phase (2). The average apparent diffusion coefficient (ADC) value of the tumor was 2.24 × 103 mm2/s. The size of the mass was approximately 3.0 cm × 2.1 cm. On contrast-enhanced images, non-enhanced liquefaction and necrotic zones were found in a cystic-solid mass. The solid part of the enhancement was located in the periphery of the mass, with irregular strips, showing progressive and obvious enhancement. The enhancement of the liver parenchyma around the mass in the arterial phase showed a halo sign. An enhanced capsule was seen on the surface of the mass (A magnetic resonance imaging (MRI) scan showed a round long T1 and long T2 signal mass on the right edge of the liver, which showed high signal intensity on diffusion-weighted imaging (DWI)  combined with chemotherapy.Pathological examination indicated an EST on the abdominal wall near the right liver. Pathologically, cuboidal tumor cells were arranged in a microcystic structure. Their cell nuclei were large, deeply stained, or vacuolated, and nuclear division was active. Schiller–Duval bodies could be seen in the region. The tumor stroma was rich in blood vessels  for the publication of any potentially identifiable images or data included in this article.SC conceived and wrote the manuscript; FC acquired, analyzed, or interpreted the data for the work. XX revised it critically for important intellectual content and agreed to be accountable for all aspects of the work by ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors contributed to the article and approved the submitted version.


Echinococcus granulosus usually occurring in the liver and lungs. Hydatid cyst of the neck is a rarely described location and more so on the back of the neck. We present a case of a six-year-old girl with a slowly evolving mass on the back of her neck. Medical investigations revealed a secondary asymptomatic liver cyst. MRI of the neck mass was consistent with a cystic lesion. Surgical removal of the neck cyst was performed. Pathological examination results confirmed the diagnosis of hydatid cyst. The patient was put on medical treatment with a complete recovery and uneventful follow-up.Hydatidosis is a parasitic infection caused by the cestode  Echinococcus granulosus. Dog is the intended host, but the infection can occur in intermediate hosts such as sheep, cattle, and horses [Echinococcus infestation is endemic [Hydatidosis or echinococcosis is a zoonotic infection caused by the cestode d horses -3. As a d horses . Humans d horses ,3. The o endemic ,4.With only seven cases of hydatid cysts of the back of the neck reported in the current literature, our case would help highlight the importance of recalling hydatid cysts among plausible differential diagnoses of cervical masses.A six-year-old girl living in the countryside was admitted to the Department of Neurosurgery with a painless, slowly growing mass located in the neck, evolving for almost two years. The patient had no significant personal or family history and no contact history with dogs, but she was living in a rural area. Physical examination revealed a 4x5cm painless non-tender mass located on the midline of the suboccipital area. The mass was covered with healthy skin, non-reductible with a soft consistency, and non-adherent to the adjacent neck muscles. There were no signs of local inflammation. Abdominal and pulmonary examinations were normal and biological tests were within normal limits.Cervical magnetic resonance imaging (MRI) showed an encapsulated cystic mass with no septations, and no invasion of adjacent tissues and organs Figure . At thisFurther investigations were performed to search for other locations. The abdominal ultrasonogram found a solitary asymptomatic hepatic cyst type 1 (according to Gharbi's classification) of the right posterior segment measuring 19x17.5x21.2 mm. Chest X-ray and CT scan of the brain were normal, and indirect hemagglutination for hydatid disease was negative. A stool examination was not performed.The patient underwent total removal of the cyst under general anesthesia. Incision of the skin was followed by sharp careful dissection of soft tissues Figure . During Pathological examination confirmed the diagnosis of a hydatid cyst Figure . The patThe patient was seen on the six-months follow-up and showed no recurrence or secondary locations. The hepatic cyst was surgically removed and the post-operative follow-up was uneventful.Echinococcus granulosus tapeworm larvae in human tissue [Hydatid cyst is a zoonosis due to the development of n tissue ,3,6,7. In tissue ,8. Hydatn tissue ,3. Humann tissue ,3,6,9.The most common sites of occurrence are the liver 65-75%) and lungs 15-25%) in humans but it can occur in any location 5% in hum,10. Acco5-75% andThe diagnosis is rarely established in the presence of an isolated neck mass ,9. In ouHydatidosis of the neck affects all ages with a sex ratio of 1, but studies show a clear predominance amongst the pediatric population ,3,7. In Ultrasound of the mass remains the exam of choice, showing hydatid sands in a purely cystic lesion as well as floating membranes, daughter cysts, and vesicles ,11,14. MDefinitive treatment of hydatid cysts is surgery based on a complete en-bloc surgical resection of the cyst ,10,14. SFurther explorations are systematic in the presence of a hydatid cyst of the neck. Abdominopelvic ultrasound and chest x-ray may confirm the presence of other locations . MedicalPostoperative follow-up is usually uneventful if the surgical removal of the cyst is done properly; nevertheless, postoperative immediate and long-term follow-up is advised consisting of neck ultrasounds and hydatid serology ,6.Echinococcus granulosus. The cyst grows slowly and can compress blood vessels and nerves causing pain, difficulty swallowing or breathing, and other symptoms. The positive diagnosis is not always straightforward, especially in slow-growing neck masses in the pediatric population. Treatment involves surgical removal of the cyst followed by medication to prevent recurrence. Monitoring for potential complications during surgery, such as rupture or dissemination is a crucial step. Early detection and prompt treatment are essential to prevent further damage to surrounding tissues and organs as well as reduce the incidence of complications.Hydatid cyst of the neck is a rare condition caused by an infestation of the larvae of the


Chordoid glioma (CG) is considered a slow growing glial neoplasm. We report two new cases with endocrinological presentation, management and outcome. Case reports: 1) An 18 year-old female patient was admitted due to headaches, nausea and vomiting and visual abnormalities. She was in amenorrhea. A brain magnetic resonance imaging (MRI) demonstrated a 35 mm-diameter sellar and suprasellar mass. An emergency ventricular peritoneal valve was placed due to obstructive hydrocephalus. Transcraneal surgery was performed. The patient developed central hypothyroidism, adrenal insufficiency and transient diabetes insipidus; she never recovered spontaneous menstrual cycles. Histopathologic study showed cells in cords, inside a mucinous stroma, positive for glial fibrillary acidic protein (GFAP). Due to residual tumor gamma knife radiosurgery was performed. Three years after surgery, the patient is lucid, with hypopituitarism under replacement. 2) A 46 year-old woman complained about a three year-history of amenorrhea, galactorrhea and headache. An MRI showed a solid-cystic sellar mass 40 mm-diameter that extended to the suprasellar cistern. She had hypogonatropic hypogonadism and mild hyperprolactinemia. The tumor mass was removed via nasal endoscopic approach. Histopathological study reported cellular proliferation of glial lineage positive for GFAP. The patient evolved with central hypothyroidism and diabetes insipidus. She was re-operated for fistula and again under the diagnosis of extradural abscess. She evolved with cardiorespiratory descompensation and death, suspected to be due to a thromboembolism. In conclusion, the first case confirms that best treatment for CG is surgery considering radiotherapy as an adjuvant therapy. The other case, on the contrary, illustrates the potentially fatal evolution due to surgical complications. Chordoid glioma (CG) is a primary tumor of the central nervous system . It was The name CG is due to the histological similarity to chordoma and the immunostaining with glial fibrillary acidic protein . It is aWe report two new cases with endocrinological presentation, management and outcome, with a review of the literature.An 18 year-old female patient was admitted due to a two-month history of intense headaches, nausea and vomiting. She referred visual abnormalities and progressive sleepiness. She was in amenorrhea for a period of 8 months. She did not refer polyuria.A brain magnetic resonance imaging (MRI) evidenced a sellar and suprasellar expansive mass of 35 mm diameter , with inOnce arrived in our hospital her consciousness worsened and she presented seizures. A ventriculoperitoneal shunt plus endoscopic septostomy with biopsy of the third ventricle mass was done at the same procedure, due to obstructive hydrocephalus. The biopsy initially suggested the diagnosis of chordoid glioma versus pilomixoid astrocytoma. Transcranial surgery was performed by a right pterional approach and trans-lamina terminalis partial resection of the tumor was done. Seven days post-surgery she presented left lung atelectasis requiring respiratory assistance. A pulmonary embolism was ruled out. Postoperatively the patient developed central hypothyroidism, adrenal insufficiency and transitory diabetes insipidus (DI), and she never recovered spontaneous menstrual cycles.Histopathology showed cells with oval nuclei, in cords, inside a mucinous stroma, positive for glial fibrillary acidic protein (GFAP), and negative for neurofilaments and epithelial membrane antigen (EMA). The diagnosis of chordoid glioma was settled .Three months post-surgery she developed poliphagia and memory disturbances. Eighteen months post-surgery, due to residual tumor of 10 per 19 mm , gamma kThree years after surgery, the patient is lucid, with a normal appetite, under replacement therapy with levothyroxine 125 µg/day, hydrocortisone 15 mg/day and etinilestradiol with levonorgestrel.A 46 year-old woman complained about a three year-history of amenorrhea and galactorrhea. Headache began one year before consultation, but worsened on the last month.An MRI showed a solid-cystic sellar mass of 22 × 25 × 40 mm, that extended to the suprasellar cistern, interpeduncular and prepontine region. It was homogeneous with gadolinium enhancement on T1 weighted images. The FLAIR and the T2 weighted sequences showed hyperintensity of the lesion. The normal pituitary gland was in situ below the tumor. Mild supratentorial ventricular dilatation was detected . Visual She had laboratory abnormalities that evidenced hypogonadotropic hypogonadism and hyperprolactinemia without alterations of thyroid and adrenal axis.With a presumptive diagnosis of papillary craniopharyngioma, the patient was referred for surgical treatment. The tumor mass was in a retrochiasmatic and third ventricle topography, and was removed by extended endonasal endoscopic approach: an extended transplanum transtubercullum sellae approach was done and a total tumor resection could be achieved. Histopathological study reported cellular proliferation of glial lineage with slight nuclear pleomorphism and moderate diffuse eosinophilic cytoplasm. There was sparse myxoid material and mild lymphoplasmocytic infiltrate. Immunostaining was positive for GFAP, with focal expression of EMA in plasma cells. Proliferation index Ki67 was below 1%. Chordoid glioma was diagnosed .The patient evolved postoperatively with temporal and spatial disorientation, central hypothyroidism and diabetes insipidus. Treatment with levothyroxine and desmopressin was initiated. She developed cerebrospinal fluid fistula and pioventriculitis. Klebsiella pneumoniae was isolated from cerebrospinal fluid. She was re-operated for fistula closure and a temporary external ventricular shunt was placed. Systemic and intrathecal antibiotic treatment was completed with good response.Twenty-eight days after the second intervention, the patient presented fever again and an isointense mass in T1 sequence was observed on MRI, near the place where the fistula was repaired. Under the presumptive diagnosis of extradural abscess, she was submitted for rhinosinusal exploration without microbiological isolation. Sixteen days later, she evolved with cardiorespiratory descompensation, suspected to be due to a thromboembolism, and died.The chordoid glioma is a rare low-grade neoplasm mostly located in the third ventricle. Obstructive hydrocephalus may develop due to its location . The CG The CG mostly presents insidiously (74%) , rarely CG should be included in the differential diagnosis of third ventricle's masses. The most characteristic image suggestive of CG on MRI is an ovoid well-defined mass on the anterior region of the third ventricle or sellar region extending towards the hypothalamus. CG is usually isointense in T1 weighted images, with strong uniform contrast enhancement and with bilateral vasogenic edema . They maThe most difficult differential diagnosis is with craniopharyngiomas which have solid parts in adults. This was our presumptive diagnosis in case 2, inducing a transphenoidal approach.Tumors that originate in the anterior portion of the third ventricle should be considered. Pilocytic astrocytomas grow along optic structures and affect younger individuals, as it occurs in germinomas. Suprasellar meningiomas have the typical dural prolongation. Ependymomas have heterogeneous enhancement, frequent calcifications and internal hemorrhage .Histopathological findings consist of clusters and cords of epithelioid cells with abundant eosinophilic cytoplasm in a mucinous stroma with infiltrates of mature lymphocytes and plasma cells . By immuThe treatment of choice is transcranial surgery, but incomplete resections are not uncommon. Mean time from onset of symptoms to surgical management has been described in 22.1 months (range 0-20 years) . In a reSurgical approach has not been specified in many cases. In our first case the transcranial approach was chosen based on histopathologic possibilities obtained through the biopsy, since the best option for both diagnosis settled is the surgical resection through the transcranial approach. Our second case was approached endoscopally since was initially interpreted as a craniopharyngioma. There are no reports about the indication of extended endoscopic endonasal approach in gliomas, being our case the first described using this surgical approach. Currently, the most direct route to the suprasellar region is through an extended approach over the sella: the transplanum-transtuberculum approach, which allows the dissection of midline tumors over the pituitary gland and below the optic chiasm, without the need for brain retraction. This approach allows to follow the tumor mass located within the third ventricle and a visual control from below of the entire third ventricle, which cannot be obtained by any transcranial approach. Thus, the endoscopic approach has been used with more frequency in recent years for the treatment of tumors, especially craniopharingyomas, extending from the suprasellar region into the third ventricle .Surgical complications of endoscopic endonasal resection of tumors that extend into the third ventricle include neurological deficits as a result of direct neural tissue trauma or vascular compromise, pituitary hormonal dysfunction as a result of injury to the pituitary stalk or hypothalamus, and cognitive and psychological abnormalities as a result of injury to the frontal/temporal lobes or hypothalamus. Cerebrospinal fluid leakage and sinonasal morbidity are the most common approach-specific complications . The mosRadiotherapy may be indicated when the resection is not complete. Progression or recurrence occurs in 30% of the patients; in these cases prognosis is usually bad. Nakajima and cols.  presenteIn these cases surgery was the main treatment by a transcranial or endonasal endoscopic approach, considering radiosurgery as an adjuvant therapy particularly in those cases of partial resections. Hypopituitarism is a plausible adverse event. However, CG might fatally evolve due to surgical complications. This postoperative mortality in the management of a low-grade tumor should be taken into consideration in therapeutic decisions. Since the goal of treatment is complete total resection pursuing a curative intention, but due to the high perioperative morbidity, options should be balanced to define an individualized therapeutic approach. More experience is needed to bring conclusions about the endoscopic endonasal approach for the management of CG.


Eosinophilic enteritis is a rare subset of eosinophilic gastrointestinal disorders. It typically presents with chronic symptoms of abdominal pain, nausea, vomiting, diarrhea, and ascites. However, the clinical presentation can vary due to acute flare-ups. Here, we present a case of eosinophilic enteritis in a young female patient with intractable vomiting and diarrhea, mimicking acute gastroenteritis in the absence of other gastrointestinal symptoms. This case illustrates the challenge of diagnosing acute and diverse presentations of eosinophilic enteritis. It also highlights the importance of promptly treating and confirming the diagnosis through urgent tissue histopathology in adolescents with unexplained vomiting and diarrhea. Eosinophilic gastrointestinal (GI) disorders constitute a heterogeneous group of rare diseases, including eosinophilic esophagitis, gastritis, enteritis, gastroenteritis, and colitis . In 1937The exact pathogenesis of EE is unknown; however, the literature suggests the roles of immunoglobulin E (IgE)-mediated allergy and delayed T-helper type-2 (Th2) responses . In mostHelicobacter pylori), inflammatory bowel disease, food allergies, neoplastic diseases (myeloproliferative disorders), celiac disease, immune-deficiencies (hyper-IgE syndrome), vasculitis , and collagen vascular diseases (scleroderma) were excluded by the serological tests and imaging modalities.We present a unique case of a young female patient who presented to us at our emergency department (ED) with intractable vomiting, diarrhea, and severe dehydration. In the absence of fever and abdominal pain, she was managed as a suspected acute gastroenteritis. Her blood workup revealed peripheral eosinophilia, elevated serum IgE levels, and high C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels. Considering a wide range of potential pathologies, we came to the diagnosis of EE after excluding the secondary causes of peripheral eosinophilia and elevated IgE levels, and histopathological findings supporting the diagnosis. The secondary causes of peripheral eosinophilia such as parasitic and bacterial infections  fluid, followed by the maintenance fluid at the rate of 100 milliliters per hour. She was also administered injectable ondansetron 8 mg every 8 hours and omeprazole 40 mg once daily. All her oral medications were discontinued, and she was shifted to the intensive care unit.On the day first of hospitalization, her symptoms didn’t improve significantly but she was vitally stabilized. Her initial blood workup was ordered on December 2, 2022, as shown in Table Suspected for an underlying gastric outlet obstruction and/or inflammatory bowel disease, upper GI endoscopy was performed on the third day of hospitalization. Although non-specific, endoscopic findings ruled out gastric outlet obstruction but displayed patchy areas of erythema in the duodenum and proximal jejunum, as shown in Figure On the fifth day of hospitalization, her symptoms started improving. Her vomiting and diarrhea resolved completely by the tenth day in hospital. She was shifted to oral prednisolone 20 mg twice daily, montelukast 10 mg once daily, and ketotifen 2 mg once daily. A repeated blood count performed on December 11, 2022, showed normal eosinophil count, normal CRP levels, decreasing IgE levels, and declining pattern in ESR. Subsequently, she was discharged to home on oral medications.Upon follow-up a week later, this patient had no GI complaint, and a tapering regimen of prednisolone was planned at 5 mg per week for four weeks until discontinuation. The allergy food panel was found to be normal. Furthermore, her histopathology report, which was also available at the follow-up visit, revealed dense infiltrates of eosinophils with degranulation in the lamina propria and muscularis mucosa of the duodenum and jejunum, as shown in Figure Eosinophilic GI diseases are a rare group of chronic and immune-mediated conditions that can affect any segments of the GI tract, including esophagus (eosinophilic esophagitis), stomach (eosinophilic gastritis), small bowel (EE), and colon (eosinophilic colitis) . AlthougThe term “eosinophilic enteritis” was first introduced by Kaijser in 1937 and has often been used interchangeably with eosinophilic gastroenteritis in literature, as the nomenclature is under review. It is defined by the presence of GI symptoms associated with eosinophil-rich infiltration of the intestinal mucosa, without secondary intestinal eosinophilia . The exaThe exact pathogenesis of this disorder remains elusive. While believed to have an allergic origin, this hypothesis is still debated due to the fact that only 70% of cases may exhibit a personal or family history of atopy and/or allergy . In thisIn 1970, Klein et al. described three different forms of EE based on the intestinal layer involved: the mucosal, muscular, and subserosal. Of note, the clinical presentation can vary depending on the degree and depth of GI tract involvement. The mucosal form, often involving duodenum, is the most common variant. It typically presents with overlapping symptoms such as abdominal pain, vomiting, diarrhea, blood in stools, iron-deficiency anemia, malabsorption, protein-losing enteropathy, and failure to thrive. The muscularis form is characterized by thickening of the bowel wall, leading to symptoms related to GI obstruction. The serosal form, characterized by the exudative ascites, is rare . Our pat3), seen in nearly 20%-80% of patients [The diagnosis of EE relies on a detailed history, thorough physical examination, and diagnostic investigations. The main abnormal finding in blood is an elevated eosinophilic count , mast cell stabilizers , anti-histamines , and medications such as azathioprine and biologics  , 20. OurEE is a rare disorder characterized by a constellation of non-specific GI symptoms, laboratory findings, and imaging abnormalities. Our case presents a unique manifestation of EE, marked by intractable vomiting and diarrhea upon presentation, imitating the symptoms of acute gastroenteritis. In a case such as ours, it is essential to remember that EE can exhibit an acute flare and create diagnostic and therapeutic challenges for physicians. A high index of suspicion becomes imperative in such cases. Given the scarcity of literature on the acute flare aspect of EE, we emphasize the need for further extensive research to comprehend the diverse facets of this intricate disorder.


Specifically, the recent, albeit mostly of low-to-moderate evidence, authoritative guideline by the European Society of Gastrointestinal Endoscopy suggest EUS characterization and potential puncture for gastrointestinal stromal tumor (GIST)-suspective lesions, at least so if estimated at >20 mm. Alternatively, tissue diagnosis by mucosal incision–assisted biopsy (MIAB), for which small-scale systematic studies are available, is recommended. By contrast, the presence of a lipoma with an unequivocal endoscopic presentation as the most common and harmless type of SEL in the stomach may not warrant specialized diagnostics. While its endoscopic appearance, such as a yellowish aspect and/or a “naked fat sign” on standard and/or bite-on-bite biopsy, is appreciable in many cases, an atypical endoscopic presentation is not uncommon and mandates a clear-cut differentiation, in particular, from gastrointestinal stromal tumors (GISTs) at the other spectrum of the malignant potential. However, in equivocal cases, real-time characterization of benign subepithelial lipoma with or without tissue diagnosis may be beneficial in terms of reduced economic  and, albeit essentially no patient-reported outcome (PRO) data are available to this end, psychological burden on the patient part. Resectional treatment of gastrointestinal lipomas is only indicated in a minor fraction with symptoms of, for example, obstruction and/or hemorrhage.2 Therefore, and in extension of a singular case reported elsewhere, pioneering an innovative endoscopic SEL tissue acquisition technique, a more straightforward approach to unmask gastric lipoma, relying on resecting the overlying mucosa by a standard cold snare is presented herein in three consecutive patients with somewhat uncharacteristic standard endoscopy.3 Table  and Fig. naked fat sign” becomes appreciable. Albeit not mandatory by itself, biopsies from visible fat are obtained under direct endoscopic vision and sent for histopathological confirmation of lipoma as tissue diagnosis. As a final step, in this series, the defective lesions underwent complete clip closure to mitigate residual risk of bleeding.Gastric subepithelial lesions (SELs) are common in endoscopy practice. Clinical management depends on multiple factors including tissue diagnosis and thus assessment of malignant potential.The authors declare no conflicts of interest.


Rosai-Dorfman-Destombes disease (RDD) was first described in 1965 as a benign histiocytic proliferative disorder of unknown cause. Cases of RDD limited to cutaneous tissue have been reported over the past few decades, but single cutaneous RDD of the scalp is rare.We report a 31-year-old male with a lump on the parietal scalp without extranodal lesion lasting 1 month with gradual enlargement. The surgical incision ruptured with purulent after the first resection. Then the patient was treated with plastic surgery after disinfection and antibiotic treatment. Finally, he recovered well and discharged after 20 days.RDD of the scalp is rare. Surgical incision can cure the lesion but it may become infected because of increased lymphocytic infiltration. Early diagnosis and differential diagnosis of RDD are necessary. For treatment, individualized therapy is critical to patient prognosis. Rosai-Dorfman-Destombes disease (RDD) is a non-neoplastic, benign, idiopathic non-Langerhans cell histiocytosis presenting in children and young adults. It mostly affects cervical lymph nodes, but approximately 40% of cases have extranodal lesions . DestombThere are few reported cases of RDD of the scalp, so we here report a case with RDD of the scalp the lesion was resected and xanthogranuloma was found in it.A 32-year-old man was found a lump on his parietal scalp. He sought medical treatment after 1 month, during which the lump gradually grew larger. He claimed the mass produced no pain. Clinical examination found that the mass was hard, smooth, and not easily moved under the skin. It measured 6 cm × 5 cm in area.A CT scan of the skull was performed, revealing a cutaneous mass above the parietal bone with a potential lytic abnormality of the bone window. MRI showed that the lesion had not infiltrated the dura or penetrated the central nervous system (CNS). The images are shown in We designed an 8 cm surgical incision in the parietal center of the scalp. During the procedure, we found that the lump had an abundant blood supply and poorly defined boundaries between the scalp and the lump, but there were no adhesions to the parietal bone. We decompressed the mass into blocks, and a total mass of 5 cm × 4 cm × 4 cm was resected with bleeding of approximately 750 ml. The patient suffered subcutaneous fluid 1 week after the operation. We aspirated 10 ml of blood-colored fluid by puncture. Then the surgical incision was dressed with a pressure bandage. The stitches were removed 10 days after surgery. The incision ruptured 20 days after initial procedure, and we observed xanthogranuloma in the fluid. Then the patient was referred to our plastic surgery department for resection of the granuloma and suturing of the incision. The diagnosis of RDD can be made by hematoxylin-eosin (H&E) staining section and specific immunohistochemical markers. Usually, there can be observed pale histocytes and emperipolesis of plasma cells and lymphocytes. Besides, S100, CD68, CD163 and CD1a are RDD's classic markers in staining. The immunohistochemical report showed that CD68 and CD163 in tissue cells, S100 in partial tissue cells, and LCA, CD20, CD79α, CD45RO, CD3, and IgG in partial tissue cells were positive; Igλ was weakly positive; Igκ and IgG4 were negative; EMA, CD38, and CD138 in plasmacytes and VIM and CD34 in capillary walls were positive; CK, GFAP, CD1a, PAS, and antacid staining were negative, The Ki-67 staining result is 3%.Originally, RDD was considered as a reactive and histioproliferative disorder of unknown cause, patients mostly being young males  who presenting with painless lymphadenopathy and systemic symptoms of fever and weight loss . The etiHowever, our patient exhibited an exclusive cutaneous RDD without systemic symptoms. CRDD is easy  to be diIn previously reported cases, CRDD observed in scalp cases are rare. Eran et al. reported a 10-year-old boy presenting with multiple Langerhans cell histiocytosis of the bone and CRDD. In addition, the patient showed amelioration after prednisone and vinblastine therapy . SimilarSome clinical data from cases of RDD in cutaneous tissue of the scalp with bony involvement are summarized in There are still limits in this study. Result from the limited objective conditions, we did not perform generation sequencing of the lesion, which might be of great help to following treatment after surgical procedure. Moreover, we report a single case, which potential provided limited treatment methods of RDD.RDD of the scalp is rare. Surgical incision can cure the lesion but it may become infected because of lymphocytic infiltration. As a consequence, early diagnosis and differential diagnosis of RDD are necessary. For treatment, individualized therapy is critical to patient prognosis.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding authors.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.


Dental clinicians and physicians should be careful in differential diagnosis of facial cutaneous nodules, since they might have an odontogenic origin.Odontogenic cutaneous sinus tracts are commonly misdiagnosed and mismanaged; thus, they are prone to recurrence. Herein, a 21‐year‐old female patient is reported with a red fluctuant nodule on her right cheek which had been misdiagnosed as an epidermoid cyst, cystic acne, and parotid gland fistula. The odontogenic origin of the lesion was first suspected when the patient presented to the Department of Endodontics, Faculty of Dentistry for a routine dental check‐up. Multiple‐visit non‐surgical root canal retreatment of the maxillary right first molar, without any additional treatment, resulted in shrinkage of the lesion. After 1 year, the lesion was resolved completely, the respective tooth and the cheek were asymptomatic, and the patient had no complaint. Healing of a cutaneous sinus tract associated with maxillary first molar after non‐surgical root canal retreatment. Several previous studies have highlighted the role of sodium hypochlorite activation in intratubular and intracanal microbial load reduction.Despite impossibility of sinus tract tracing due to the distance between the lesion and the suspected tooth; the pattern of bone destruction on CBCT images, failure of the previous treatment plans, and the onset of the healing process of cutaneous lesion during the treatment sessions, supported our clinical diagnosis and indicated the direct relation of the suspected tooth and the lesion. Although short‐term treatment outcomes were favorable, long‐term evaluation is required to ensure treatment success.4The present case report highlights the necessity of thorough extra‐ and intra‐oral examinations before any intervention. It also highlights the role of multidisciplinary approach and background knowledge in management of cutaneous sinus tracts.Pegah Sarraf: Conceptualization; methodology; supervision. Mehrfam Khoshkhounejad: Conceptualization; investigation; writing – review and editing. Maryam Babaahmadi: Methodology; validation; writing – original draft; writing – review and editing. Fatemeh Hamidzadeh: Methodology; writing – original draft.This research received no specific grant from any funding agency in the public, commercial, or not‐for‐profit sectors.The authors deny any conflict of interest.For clinical cases, the local ethics committee considers that the patient's consent is sufficient.Written informed consent was obtained from the patient to publish this report in accordance with the journal's patient consent policy.


Head and neck rhabdomyosarcoma (HNRMS) is a rare type of soft tissue tumor that affects both adults and children with an overall incidence of 0.041 per 100,000 people. Adults make up approximately 31.2% of all HNRMS diagnoses and have an overall survival rate between 20% and 40%. We present a case of a 46-year-old male who initially presented with nasal congestion and vision changes. Maxillofacial computed tomography and magnetic resonance imaging of the brain showed involvement of the orbital apex, abutment of the planum sphenoidale, and extension to the foramen rotundum (FR). Nasal endoscopy with biopsy confirmed the diagnosis of T2aN0M0 parameningeal HNRMS. The patient underwent induction chemotherapy, followed by endoscopic resection, which resulted in negative intraoperative margins. Subsequently, he underwent adjuvant concurrent chemotherapy and proton beam radiation after positive microscopic positive margins were found on the optic nerve. The patient did not experience any significant complications, and he is currently without radiographic or clinical recurrence 18 months after the treatment. He was able to maintain his vision throughout the treatment. In adults, HNRMS is usually treated with chemoradiotherapy based on pediatric protocols, since there are limited data available for adult treatment protocols and outcomes. Although surgery has been associated with positive outcomes in adult patients, there are no previous reports of its use with either neoadjuvant or adjuvant treatment. This type of treatment protocol has never been described for adult HNRMS. We hope that our report can add more data to the growing body of literature on HNRMS treatment protocols. Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor composed of immature mesenchymal cells with the potential to differentiate into striated muscle tissue. It is a rare tumor that accounts for only 350 cases annually, yet it comprises about half of soft-tissue sarcomas in children and adolescents . ApproxiHead and neck RMS (HNRMS) has been reported to the Surveillance, Epidemiology, and End Results (SEER) registry 558 times from 1973 to 2007, with an overall incidence of 0.041 cases per 100,000 population [Here, we present a 46-year-old male with a T2aN0M0 parameningeal HNRMS with involvement of the orbital apex, abutment of the planum sphenoidale, and extension to the foramen rotundum (FR) who underwent induction chemotherapy, followed by endoscopic resection and then by adjuvant concurrent chemotherapy and proton beam radiation. The treatment course was modeled after pediatric protocols for similar parameningeal RMS. To the authors’ knowledge, there are no previous reports of this type of operative and post-operative treatment regimen for parameningeal RMS in an adult, and the patient is currently without radiographic or clinical recurrence 18 months out and has maintained his vision.A 46-year-old male presented to an outside hospital emergency department with five days of progressively worsening diplopia and nasal congestion. Ophthalmology evaluation noted right papilledema. Non-contrast maxillofacial CT showed a 4.5 cm x 4.1 cm x 4.0 cm soft tissue mass centered in the right ethmoid sinus involving both ethmoid sinuses, both sphenoid sinuses, and the right sphenopalatine foramen with thinning of the planum sphenoidale and erosion into the right orbital apex Figure . OtolaryThe patient underwent an endoscopic image-guided biopsy. The frozen section was not definitive. Permanent histological analysis revealed a small, round, blue-cell tumor, with immunohistochemistry that was positive for desmin, myoD1, and myogenin consistent with RMS. Confirmatory testing with reverse transcription polymerase chain reaction (RT-PCR) was positive for PAX/FOX01 translocation, confirming the alveolar subtype RMS.After tissue diagnosis, a positron emission tomography (PET) scan redemonstrated the mass centered in the right ethmoid cavity with a maximum standard uptake value (SUV) of 8.6. There was also nonspecific borderline activity of bilateral level 2 lymph nodes with an SUV of 2.8, but no clinical cervical lymphadenopathy on physical exam.The patient was presented at a multidisciplinary tumor board as a T2aN0M0 alveolar RMS of the right ethmoid sinus. The tumor board recommendation was for induction neoadjuvant chemotherapy, followed by endoscopic resection and then adjuvant concurrent chemotherapy and proton beam radiation therapy. The patient was strongly opposed to an orbital exenteration. It was felt that this induction chemotherapy (IC) technique, modeled after pediatric protocols, would help facilitate achieving surgical clear margins or at least only microscopic disease at the orbital apex to be followed by adjuvant concurrent chemoradiation therapy. The combination of vincristine, actinomycin D, and cyclophosphamide was chosen according to the D9803 Children’s Oncology Group (COG) protocol. Upon completion of four cycles of induction, both post-treatment CT of the sinuses Figure  revealedOne month after completion of IC, image-guided endoscopic resection, including a right medial maxillectomy, bilateral ethmoidectomy, bilateral sphenoidotomy, right frontal sinusotomy, near total septectomy, extended right PPF dissection, and right orbital/optic nerve decompression, was performed. Final intraoperative frozen section margins of the posterior periorbita, posterior septum, lateral sphenoid wall/optic nerve sheath, ethmoid/sphenoid skull base, and PPF/vidian canal were negative. Vision remained intact after surgery and no cerebrospinal fluid leak was encountered. Final pathology from the resection again revealed parameningeal alveolar RMS, FOX01 translocation positive. Of note, the lateral sphenoid wall/optic nerve sheath did show evidence of microscopic disease on the permanent section. Four weeks after recovery from surgery, the patient started concurrent chemoradiotherapy (CRT), which included vincristine, cyclophosphamide, actinomycin D, and 23 fractions of proton beam therapy with a total dose of 41.4 Gy.Three-month post-treatment PET-CT Figure  revealedRMS is a malignant tumor composed of immature mesenchymal cells with the potential to differentiate into striated muscle tissue and typically presents with small blue round cells on histological examination . The twoOverall, RMS treatment relies on the TNM classification, disease site, FOX01 translocation factor presence, and clinical group findings. Due to the rare occurrence of RMS in adults, the prognosis is based on data identified by the Intergroup Rhabdomyosarcoma Study Group Outcomes I and II, which included only those diagnosed in the first two decades of life -10. The Regarding prognostication and survival of HNRMS, the reported 5-year overall survival (OS) ranges from 33% to 45% . Wu et aThe parameningeal subsite of HNRMS is also associated with a poorer prognosis and earlier recurrence. This is likely because these tumors tend to extend into meningeal and intracranial sites, as seen in our patient . In a daIn general, the treatment guidelines for adults are similar to those for children due to limited data available, which consists primarily of chemotherapy with the addition of radiation and/or surgical resection -13. AlthWhile there was no direct extension into the cranial fossa in our patient, there was the involvement of the PPF and orbital apex. With the patient opposed to an orbital exenteration, a pterygopalatine fossa dissection was a feasible option. Our patient underwent this procedure without any orbital injury, cranial nerve injury, or CSF leak. Craniofacial approaches have been reported, but these result in transfacial incisions or facial osteotomies, which can be disfiguring and have an increased risk of neural and vascular injury secondary to poorer visualization -17.We presented a 46-year-old male with a T2aN0M0 parameningeal alveolar RMS with involvement of the orbital apex, abutment of the planum sphenoidale, and extension to the FR who underwent IC followed by endoscopic resection and adjuvant concurrent chemotherapy with proton beam radiation. The treatment course was modeled after pediatric protocols for similar parameningeal RMS. Currently, the patient is 18 months disease-free without significant comorbidities or side effects from their treatment. To the authors’ knowledge, there are no previous reports of this type of operative and post-operative treatment regimen for parameningeal RMS in an adult, specifically with adult endoscopic resection with proton beam therapy.


Background: The typical imaging findings of hairy polyps have been described mostly in case reports. This study was conducted to describe the CT and MRI features of hairy polyps and their common associated abnormalities. Methods: Medical records of 14 patients with pathological diagnosis of hairy polyps were collected for this study. For each patient, the medical records, including demographics, clinical manifestations, and imaging findings were reviewed. Results: The female-to-male ratio was 3.7:1. The age at first episode varied from birth to 2.7 years. The masses were derived from the back side of the soft palate in seven (50.0%) cases, from the lateral pharyngeal wall in four (28.6%) cases, from the soft palate in one (7.1%) case, from the nasal vestibule in one (7.1%) case, and from the parapharyngeal space in one (7.1%) case. A total of 11 (78.6%) cases presented with pedicled masses containing fat and a central core of soft tissue, there were 3 (21.4%) cases whose imaging findings were atypical, and there were 6 (42.9%) patients who had other pathologies. Conclusions: Hairy polyps typically presented as pedicled masses containing fat and a central core of soft tissue, but sometimes their imaging findings can be atypical and they can be associated with other congenital abnormalities. CT and MRI are reliable methods for the diagnosis of hairy polyps and their associated abnormalities. Hairy polyps are rare mature bi-germinal masses and are usually found in the oronasopharyngeal region . HistopaHairy polyps are nonmalignant lesions with limited growth potential and mostly occur in newborns, infants, and young children, with a higher prevalence in females . The incTwenty-four cases of hairy polyps confirmed by pathology between January 2013 and March 2020 were retrospectively reviewed; however, ten cases without preoperative CT or MRI scans were ruled out. This work was approved by the local ethics board. All 14 patients with medical records, including demographics, clinical manifestations, CT, and/or MRI findings, were reviewed. A total of 3 patients had undergone MRI, 6 patients had undergone CT, and 5 of them had received both MRI and CT. Out of the 14 patients in this study, 3 patients received contrast. One patient received contrast-enhanced CT scans. One patient received contrast-enhanced MRI scans. One patient received both contrast-enhanced CT and MRI scans. The location, origin, morphology, size, density/signal characteristics, and contrast enhancement pattern of the masses were analyzed on the CT and MRI images.2 was used for DWI. Additional sequences were acquired according to the patient’s condition.MRI scans were performed with a 3.0T MR scanner  with a 16-channel phased-array receiver coil in the referral area. All cases received transverse T1-weighted or T2-weighted, sagittal, or coronal T2-weighted scan, and transverse, sagittal, and coronal T2-weighted spectral presaturation and inversion recovery (T2W-SPIR). Gadolinium-enhanced T1W-SPIR was not standard and was used in two patients. Diffusion-weighted imaging (DWI) was used in five cases. A gradient strength of b = 1000 s/mmCT scans were performed using a 256-row CT scanner  from the base of the skull to the level of the thoracic inlet. The scan was performed once before and once after the injection of the contrast medium. Iodixanol-enhanced CT scans were not standard and were used in two patients. The tube voltages of the plain scan, arterial phase, and venous phase were 120 KVp, 80 KVp, and 120 KVp, respectively. The tube current was set by automatic tube current modulation.Clinical and imaging data of 14 patients are shown in There were 11 females and 3 males with a female-to-male ratio of 3.7:1. There were 11 neonates. The age at first episode varied from birth to 2.7 years with a median age of 1 day. The clinical manifestations included dyspnea (42.9%), polypnea (35.7%), snoring (21.4%), nasal obstruction (21.4%), mouth breathing (21.4%), perioral cyanosis (21.4%), choking on milk (21.4%), hearing impairment (14.3%), difficulty feeding (7.1%), otopyorrhea (7.1%), inspiratory stridor (7.1%), gurgling with sputum (7.1%), and heart rate reduction (14.3%). Three patients underwent cardiopulmonary resuscitation (CPR) due to mass-induced asphyxia. One patient was asymptomatic, and the pharyngeal mass was detected by accident when being treated for a cold.Of the 14 patients, the mass occurred on the left side in 9 (64.3%) patients, on the right side in 4 (28.6%) patients, and on the midline in 1 (7.1%) patient. Intraoperative findings showed that the lesions were derived from the back side of the soft palate in seven (50.0%) cases; from the lateral pharyngeal wall in four (28.6%) cases, including the pharyngeal opening of auditory tube, the eustachian tube, the pharyngeal recess, and the inferior side of the pharyngeal opening of the auditory tube; from the soft palate in one (7.1%) case; from the nasal vestibule in one (7.1%) case; and from the parapharyngeal space extending to external the auditory canal and middle ear in one (7.1%) case. The main body of the mass was located in the pharyngeal region in 11 (78.6%) cases, in the nasal cavity and nasopharynx in 1 (7.1%) case, in the nasal vestibule in 1 (7.1%) case, and in the parapharyngeal space, the external auditory canal, and the tympanum in 1 (7.1%) case. There was one case where the lesion was stretched to the oral cavity during a severe cough.The size of masses varied from 0.6 to 4.3 cm. There were 11 (78.6%) cases with the largest diameter of the mass greater than 2 cm. The shapes of the masses were varied. Eleven (78.6%) cases presented with typical pedicled masses containing fat and a central core of soft tissue, including sausage-like masses in five (35.7%) cases, tongue-like masses in four (28.6%) cases, and pear-like masses in two (14.3%) cases. Of the eight patients who underwent MRI scans, there were five (62.5%) patients whose masses had high signal intensity on T1-weighted and T2-weighted sequences with a low-signal central core whose signal intensities were similar to those of muscles. The high signal intensities on all sequences were similar to the fat signals and attenuated by fat suppression. The central core demonstrated mild enhancement following intravenous gadolinium administration, while the surrounding components of the fat signals demonstrated no enhancement in one case . There wIn addition, three (21.4%) cases presented with irregular shapes the MRI or CT findings that were atypical. One case presented with a sausage-like mass in the right nasal vestibule and nasal limen that was mainly high-signal with multiple filament-like low signals inside on T1-weighted and T2-weighted MRI . One casSix (42.9%) patients had other abnormalities. Five of them were incidental findings within the imaging scope of their investigations and one was in the other area. One infant had ossicular chain disruption showing the absence of the left stapes head and part of stapes arch , one hadNone of the patients were associated with syndromic disorders.Complete resection was performed in all 14 cases, which were followed up for more than 1 year without recurrence.The volumetric CT dose index (CTDIvol) was 2.98 ± 2.21 mGy, and the dose–length product (DLP) value was 62.50 ± 57.38 mGy-cm.Hairy polyps are rare congenital benign lesions, which were first described in 1784 . They moHairy polyps can occur anywhere in the body . PharyngClinical symptoms depend on the location and size of the mass. In this paper, the size of masses varied from 0.6 to 4.3 cm, which is comparable to a previous study reporting the size of lesions ranging from 0.5 to 6 cm . They tyHairy polyps are not definitively associated with any syndromic disorder but have been reported to be associated with other congenital abnormalities such as cleft palate, uvula dysplasia, auricular deformities  such as In the case of tracheal intubation, hairy polyps might be easily overlooked, as they are mobile and soft masses. One case of pharyngeal hairy polyp was obscured by oral examination and endotracheal intubation, but it was diagnosed by MRI . ImagingUpon MRI, most of the masses were found to have heterogeneous high signal intensity on T1-weighted and T2-weighted sequences with a low-signal central core whose signal intensities were similar to those of muscles. The high signal intensities on all sequences were similar to the fat signals and attenuated by fat suppression. The central core demonstrated mild enhancement following intravenous gadolinium administration, while the surrounding components of the fat signals demonstrated no enhancement. The central core helps us trace the origin of the mass, which is important for the choice of surgical method. Characteristic imaging features on CT include well-circumscribed masses containing fat and linear soft tissue–density components centrally that correspond to the fibrovascular stalk . These fHowever, there were three cases whose imaging findings were atypical. All masses contain fat and soft tissue, but their distribution and proportions vary. In rare cases, the mass has a thick capsule.The high fat content of hairy polyps demonstrated on CT and MRI scans helps to exclude some vascular and neurologic lesions, such as neuroblastoma, hemangioma, encephalomeningocele , and embConsidering that a large proportion of patients with hair polyps are associated with other congenital malformations that are mostly found within the imaging scope of their investigations, we recommend that all patients clinically suspected of this disease undergo CT and/or MRI examination. In general, it is not necessary to use contrast media for lesions with typical imaging features on non-contrast enhanced images. However, for lesions with atypical imaging findings or in rare locations such as the middle ear or parapharyngeal space, contrast enhanced scanning plays an important role in differential diagnosis and determination of the extent and blood supply of the lesion.Surgical excision of the mass at the pedicle base is an effective way to treat hairy polyps . Two casThis study had several limitations. The first was the insufficient sample size for statistical measurements. Second, there was no consistent scanning protocol for each patient in this study. Third, there was no available genetic test result to provide evidence for the pathogenesis of associated malformations. Hence, further investigation with a larger cohort is required to understand the full disease spectrum among children with hairy polyps. Standard CT and MRI scanning procedures should be developed for head and neck masses, too, especially in those with fat. Future research should carry out genetic testing on patients with hairy polyps so as to have a more comprehensive understanding of the disease and its associated malformations.Pharyngeal hairy polyps are sometimes associated with other congenital abnormalities that can be easily missed at an initial visit. Imaging is useful for delineating the size, origin, and extent of the mass, and it is essential for determining intracranial extension and evaluating associated comorbidities. There are some distinctive imaging features for pharyngeal hairy polyps, but sometimes their imaging findings can be atypical. For lesions with atypical imaging findings or in rare locations, we recommend more comprehensive imaging examinations, such as contrast enhanced CT and MRI.


Pancreatic cystic lesions (PCL) have a wide range of demographical, clinical, morphological and histological characteristics. The distinction between these lesions is of paramount importance due to the risk of malignancy in specific categories of PCL. Considering the malignant potential for pancreatic cystic neoplasm (PCN) lesions, guidelines have been made to balance unnecessary treatment and manage the progression to malignancy. Various surgical procedures can be done for PCN depending on the location and size of the cyst; pancreatoduodenectomy is done for PCN located in the head of the uncinate process, whereas distal pancreatectomy is done for PCN in the body or tail. In the neck and proximal body of the pancreas, less extensive resections such as central pancreatectomy can be performed. Active surveillance of PCN is typically offered to asymptomatic PCNs of subtype intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms (MCN) without any concerning features. In recent years, numerous guidelines have been created to augment PCN diagnosis, classification and management. Despite this, the management of PCNs remains complex. Thus, discussions with multidisciplinary teams involving surgeons, gastroenterologists, pathologists, and radiologists are required to ensure optimum care for the patient.  Pancreatic cystic lesions (PCL) are a comprehensive group of pancreatic pathology and are broadly classified into non-neoplastic lesions and cystic neoplasms. These lesions have a wide range of demographical, clinical, morphological and histological characteristics . The incClassificationUntil recently, the spectrum of PCL was restricted to mucinous and serous neoplasms. However, since the 1980s, there has been an exponential advancement in radiology and other diagnostic techniques, leading to a drastic increase in awareness and subsequent research on PCLs. Thereafter, new classification systems were created, leading to more studies on the pathogenesis, morphology, clinical spectrum, and management of these new entities of PCL . CurrentNon-neoplastic cysts consist of pancreatic pseudocyst, congenital cysts, lympho-epithelial cysts, mucinous non-neoplastic cysts, enterogenous cysts, retention cysts, periampullary duodenal wall cysts, and endometrial cyst. Of the following, pseudocysts are most commonly seen in clinical practice and will be discussed in detail in the following sections. Neoplastic cysts or PCN are broadly classified according to the World Health Organisation classification of pancreatic tumours Table  into BenPCNs are a heterogenous group of lesions comprising 5-15% of all PCLs [Pseudocyst of the pancreasPancreatic pseudocysts are the most common PCL accounting for approximately 75% of all PCLs. These are inflammatory cysts consisting of fluid rich in pancreatic enzymes or protease-free serous fluid enclosed by a wall of fibrous or granulation tissue. They are named so because of the lack of epithelial lining. They are formed due to autodigestive tissue necrosis secondary to acute or chronic pancreatitis. Pseudocysts occur when an acute post-pancreatic fluid collection fails to resolve spontaneously and walls off; this usually occurs four weeks after the onset of acute pancreatitis. Pseudocysts may occur anywhere in the pancreas and can even be extra-pancreatic. They vary in size from 3 to 20 cm; larger sizes are associated with alcohol-induced pancreatitis and are most commonly located in the lesser omental sac. Other common locations include retroperitoneum, stomach, liver, and perirenal or subdiaphragmatic space. They may or may not communicate with the ductal system and are usually single but may be multiple in 10% of cases . The mosThe initial investigation of choice of pseudocysts is a transabdominal ultrasound (US) . They arHigh attenuation areas may be noted within the cavity, indicating the presence of debris, blood, or infections. CT scans also provide additional information about anatomy and pathology. The difficulty arises in cases where there is no evidence of pancreatitis when pseudocysts may be confused with pancreatic mucinous cysts. In these cases, cyst fluid analysis may help diagnose the lesion. Besides CT scans, MRI abdomen and magnetic resonance cholangiopancreatography (MRCP) is also used selectively in cases where communication with the biliary system cannot be accurately determined .EUS can differentiate pancreatic cysts from other PCLs. In EUS, pseudocysts appear as anechoic fluid-filled structures surrounded by a thick hyperechoic rim. Debris may be noted in the dependent portion of the cavity, and colour Doppler often reveals multiple prominent vessels . EUS-guiSpecial note is that evidence of epithelial cells in the fluid analysis is more suggestive of a PCN than a pseudocyst. Elevated amylase levels are suggestive of a connection with the main pancreatic duct (MPD) and help with the diagnosis of a pseudocyst. Tumor markers such as carcinoembryonic antigen (CEA), on the other hand, are relatively low compared to MCN and IPMN .The management of pancreatic pseudocysts includes conservative management, image-guided drainage, and surgical intervention. The majority of simple small uncomplicated pseudocysts (<4cm) are known to resolve spontaneously; hence simple observation with periodic follow-up is sufficient. However, with the increase in the cyst size, most patients become symptomatic and may develop complications. The presence of symptoms is also indication for treatment, not only presence of complications . DrainagSurgical management includes internal drainage and pseudocyst resection. Internal drainage is preferred in uncomplicated mature pseudocysts. Cystogastrostomy can be done in cases where the cysts are adherent to the posterior wall of the stomach, whereas cystoduodenostomy can be done in small cysts in the head and uncinate process of the pancreas. On the other hand, cystojejunostomy can be done for all other cysts, including large cysts or cysts more than 15cm .Cystogastrostomy is a simple and fast procedure; infectious complications are less common, but it is associated with a higher incidence of upper gastrointestinal bleeding. There is no significant difference noted in terms of recurrence rate, morbidity, or mortality between cystogastrostomy and cystojejunostomy. However, cystogastrostomy was reported to have fewer complications in terms of blood loss and duration of surgery .Pseudocyst resection can be done in certain cases of chronic pancreatitis, including chronic pain associated with chronic pancreatitis, multiple cysts, hemorrhage from pseudoaneurysm, obstruction, or cysts located in the uncinate process which are technically difficult to drain. Different resections can be performed depending on the location of cysts, including enucleation, partial left-sided pancreatectomy, Whipple procedure, pylorus-preserving pancreatoduodenectomy, Beger operation, and Frey procedure. Laparoscopic approaches can be done with studies showing low complication rates, good outcomes postoperatively, decreased hospital and postoperative recovery time and decreased incidence of bleeding complications .Pancreatic cystic neoplasmsPCNs are a heterogenous group of cystic lesions that range from SCN, MCN, and IPMN to other rare lesions such as cystic neuroendocrine tumors (cNET) and solid pseudopapillary neoplasms (SPN). This diverse group of pancreatic cysts has varied clinical, radiological, and pathological features Table  and diffPrevalence of PCN in abdominal ultrasound was noted to be 0.21%, in CT 2.6%, and in MRI with MRCP 2.4 to 49.1%. However, in autopsy studies, PCN was reported to be as high as 50% of patients. There is no gender predilection, but increasing age had a strong correlation .Serous cystic neoplasms are focal well-demarcated lesions that consist of multiple small cysts (<1-2cm) with dense fibrous septations, which gives it its honeycomb appearance. A pathognomonic feature seen is sunburst calcification caused by central fibrosis or calcification. However, this is seen in only 10% of the patients. Less common variants include a macrocystic variant (>2cm cysts) and a solid variant that consists of multiple microcysts, which appear as a hypoechoic mass on CT scan. Cytology with findings of glycogen-positive staining cells established the diagnosis in 50% of the patients .Mucinous cystic neoplasms are septated thin-walled fluid-filled cystic lesions. Diagnostic features include epithelial cells . Pathognomonic features include eccentric calcifications but are seen in only 15% of cases. Ductal communication is rare, but there is a 25% chance of malignancy which is suggested by larger sizes, solid regions, or a solid mass. Elevated CEA or Mucin is indicative of malignancy .IPMNs originate from the main pancreatic duct or side branches. Characteristic features include the production of mucin, ductal dilatation, and papillary epithelial growth. A fish mouth of gaping papilla may be present. EUS findings include dilated main or side ducts with mucin and mural nodules. Imaging features on CT scan suggestive of IPMN include focal hypoechoic mass, mural nodules, or unilocular cystic lesions . The difClinical FeaturesThe majority of PCNs are incidentally diagnosed on imaging studies; typical pancreatic symptoms are usually absent. Jaundice may be seen secondary to external compression of the common bile duct (CBD) by the PCN or a mucin plug. MD-IPMN with mucin production may be associated with acute pancreatitis as the mucin plugs may occlude the MPD causing acute pancreatitis and abdominal pain with elevated amylase levels. Endocrine/exocrine function disruption or insufficiency may also occur with progressive inflammation. Jaundice and pancreatitis may also occur in the setting of advanced neoplasia .Risk of MalignancySCN is most commonly benign with no need for active surveillance. On the other hand, IPMN, MCN, SPN, and cNET are considered to have malignant potential, and hence surgical resection or active surveillance is required. Amongst PCN, IPMN is considered to be the most common . This isDiagnosisDifferentiation between the various subtypes of PCN is the crucial step in diagnosis as the management varies according to the subtype and its related malignant potential. The typical workup of PCN includes a pancreatic protocol CT and gadolinium-enhanced MRI with MRCP and EUS .MRI with MRCP is used to determine the involvement of the pancreatic duct and is more sensitive to detect mural nodules or internal septations as compared to a CT scan. MRI with MRCP is also used for follow-up studies to reduce exposure to ionizing radiation . IPMN isEUS is used as an adjunct if specific clinical or radiological features are present or if cytological examination or biochemical analysis is required to differentiate between the lesions. EUS can determine the cyst size, location, thickness, focal wall irregularity, mural nodule or mass, septations, debri, mucus or dilatation of MPD. The indications for EUS are as described in the American Gastroenterological Association (AGA) guidelines 2015), International Association of Pancreatology (IAP) guidelines (2017), and European guidelines (2018) and are summarized in Table , InternaEUS can also be augmented with FNA to get samples for cytopathological examination and other biochemical examinations to distinguish between the different subtypes of PCN Table . HoweverCyst Fluid AnalysisCyst fluid can be analyzed for tumour markers such as CEA, mucin, amylase, cytology and other molecular markers. Cyst fluid CEA is the most useful for differentiation between mucinous and non-mucinous PCN and has a higher accuracy for detection of MCN when compared to EUS morphology or cytology. CEA is used because mucinous cysts are lined by endoderm-derived columnar epithelial cells, which secrete CEA in contrast to non-mucinous cysts. Amylase is also routinely used in the biochemical analysis of cyst fluid and elevated amylase supports more the diagnosis of pseudocyst. Elevated CEA levels are indicative of a connection with the pancreatic duct; however, it can also be raised in MCN. A third marker used is fluid glucose level which is elevated in mucinous PCN. However, evidence is still lacking; hence further studies are required. Deoxyribonucleic acid (DNA) tests have also been introduced as a promising adjunct to differentiate between mucinous subtypes and premalignant and advanced lesions. DNA biomarkers such as KRAS mutations, guanine nucleotide binding protein, alpha stimulating (GNAS) mutations are sensitive and specific for IPMN. They are less sensitive for MCN but not sensitive or specific for SCN. However, the use of these tests is expensive and time-consuming and is currently not integrated into management guidelines -33.Management and guideline recommendationsConsidering the malignant potential for PCN lesions, guidelines for either close monitoring or management are needed to balance unnecessary treatment and manage the progression to malignancy. Five guidelines that offer recommendations on PCN surveillance and surgical resection based on the risk of malignancy are American Gastroenterology Association (2015), International Consensus Guidelines (2017), European guidelines (2018), American College Gastroenterology (2018) and Radiology White paper (2017) ,35.Active Surveillance of PCNActive surveillance of PCN is typically offered to asymptomatic PCNs of subtype IPMN and MCN without any concerning features Table .Surveillance protocols vary between guidelines, and no definite protocol is agreed upon for surveillance. However, all guidelines agree that the risk of malignancy, life expectancy, and comorbidities of the patient should be taken into account in the decision-making .In the case of IPMN, for asymptomatic patients without concerning features, surveillance interval is determined by the cyst size according to IAP guidelines, whereas AGA and European guidelines do not consider cyst size. For MCN, AGA and IAP guidelines recommend resection for all cases. On the other hand, European guidelines recommend follow-up in MCN with a size <40mm without mural nodules. Active surveillance is not needed in asymptomatic SCN patients as the risk for malignant transformation is very rare .European Neuroendocrine Tumour Society consensus guideline and European guidelines advise surveillance for patients with asymptomatic cNET tumors of size <20mm. Studies have shown that in asymptomatic tumors <20mm, conservative management is safe .The modality of choice for surveillance is MRI with MRCP. EUS is used in patients who cannot undergo MRI with MRCP. Surveillance is indefinite as long as the patient is surgically fit and willing for surgery if warranted. Table Surgical interventionSurgical resection of PCN is recommended to be performed by an experienced multidisciplinary team in a high-volume center. Various surgical procedures can be done for PCN depending on the location and size of the cyst; pancreatoduodenectomy is done for PCN located in the head of the uncinate process, whereas distal pancreatectomy is done for PCN in the body or tail. In the neck and proximal body of the pancreas, less extensive resections such as central pancreatectomy can be performed. Central pancreatectomy is a parenchyma-sparing technique that entails removal of the neck and proximal body and sparing the head and tail of the pancreas. However, despite its excellent long-term pancreatic functional outcomes, there is a high risk of pancreatic fistula, thereby increasing the morbidity of the procedure. Hence careful patient selection is of paramount importance ,41.IPMNResection is indicated in MD-IPMN according to IAP and European guidelines. On the contrary, AGA guidelines only recommend resection if main duct dilatation is present along with a nodule or positive cytology for malignancy. In the case of SB-IPMN, active surveillance or surgical intervention is determined by the presence of malignant features or symptoms. Pancreatic enucleation can also be done in cases of larger SB-IPMN as an alternative to lifelong surveillance. Advantages include decreased pancreatic parenchyma resection and decreased endocrine and exocrine insufficiency. However, in cases of unanticipated malignancy, lack of lymphadenectomy may lead to inferior oncological outcomes.MCNResection is recommended for all lesions according to AGA and IAP guidelines, whereas European guidelines recommend resection of symptomatic MCN if size >-40mm or if a nodule is noted. European guidelines advocate conservative management for MCN <40mm as studies have shown that small-sized asymptomatic indeterminate MCN with no at-risk features have a low risk of malignancy.SPNResection is indicated in patients with SPN according to the AGA, IAP, and European guidelines.cNETEuropean guideline and European Neuroendocrine Tumor Society consensus guideline recommend resection of cNETs > 20mm/any malignant behaviour .EUS-Guided Pancreatic Cyst AblationCyst ablation using ethanol or paclitaxel is an experimental approach for PCN. Advantages include organ preservation and preservation of endocrine and exocrine function. Disadvantages include the inability to obtain a specimen for histopathological evaluation ,43.Postoperative SurveillancePatients who underwent resection for MCN do not require surveillance. However, resected MCN with evidence of invasive carcinoma in the histopathological specimen have a 25% risk of recurrence. IPMN cases post partial pancreatectomy must undergo life-long follow-up until the patient is not fit or is unwilling to undergo surgery. European guidelines recommend that follow-up is indicated with cross-sectional imaging every six months for the first two years, followed by yearly imaging for HGD or main duct involvement post resection. IAP guidelines recommend biannual follow-up in patients with a family history of pancreatic malignancy, HGD present in margin, and nonintestinal histological subtype; for the rest, six to 12 monthly follow-up is adequate -46.Recent advances in diagnosis and investigations in PCNSecretin-Enhanced MRCPSecretin-enhanced MRCP is used to enhance the visualization of a connection between the PCN and pancreatic duct. It is based on the mechanism of action of secretin which stimulates the acinar cells to release pancreatic juice into the duct, subsequently causing an increase in size and diameter of the duct. The advantages of the use of secretin-enhanced MRCP have been shown in several studies, but long-term studies are yet to be done to justify its use in standard clinical practice. Disadvantages include its cost and increase in scanning time by five to 10 minutes .Contrast-Enhanced EUSContrast-enhanced EUS is used to distinguish between mural nodules and clots by determining the vascularity present in mural nodules. It is done by detecting signals from microbubbles in vessels secondary to the contrast agent. Disadvantages are similar to EUS, including its operator dependence and need for specialists experienced in advanced endoscopy .Confocal Laser Endomicroscopy (CLE)CLE is used as an adjunct to differentiate between PCN types. It uses an endoscopic probe introduced through a 19-gauge needle to display real-time visualization of the PCN with microscopic detail. Characteristic findings for SCN include 'superficial vascular network' or 'fern pattern', whereas for IPMN finger-like papillae are characteristic. Epithelial bands  suggest MCN. Diagnostic accuracy in differentiating between PCN types ranges from 71-94% ,50.Pancreatic cystic neoplasms are being diagnosed at an increased pace primarily due to improvements in radiological and endoscopic imaging modalities. In recent years, numerous guidelines have been created to augment PCN diagnosis, classification and management. Despite this, the management of PCNs remains complex. Thus, discussions with multidisciplinary teams involving surgeons, gastroenterologists, pathologists, and radiologists are required to ensure optimum care to the patient.


Inflammatory fibroid polyp (IFP), initially considered a reactive process, is now recognized as a benign mesenchymal neoplasm of the gastrointestinal tract. We report a case of a 68-year-old woman with medically refractory Crohn disease that presented with intussusception requiring surgical intervention. The resection revealed a jejunal mass consisting of a submucosal proliferation of bland spindle cells in a fibrous stroma infiltrated by numerous eosinophils. By immunohistochemistry, the lesion was positive for vimentin and negative for desmin, smooth muscle actin (SMA), S-100, CD117, DOG1, ALK (D5F3), Melan-A, HMB-45, CD34, and STAT6. Ki-67 proliferative index was low (<1%). The mass was classified as IFP by its characteristic morphology and associated eosinophilia. IFP should be considered in the differential diagnosis of adults with intussusception or bowel obstruction. Definitive treatment typically requires surgical resection of the involved bowel segment. PDGFRA) gene, similar to a subset of gastrointestinal stromal tumors and inflammatory myofibroblastic tumors. It is now recognized as a benign mesenchymal neoplasm indigenous to the GI tract [Inflammatory fibroid polyp (IFP) is a rare benign mesenchymal neoplasm that can be found throughout the gastrointestinal (GI) tract but is more frequent in the gastric antrum, followed by the small intestine, in particular the ileum, and is rare at other locations –4. Most GI tract –12.A 68-year-old woman with history of recurrent small bowel obstruction secondary to medically refractory Crohn disease presented with abdominal pain, nausea, and vomiting. Computed tomography (CT) of the abdomen and pelvis demonstrated diffuse wall thickening involving the mid small bowel through the terminal ileum with abnormal dilatation including a 23 cm long segment of distal ileum and mesenteric stranding located about 8-10 cm proximal to the ileocecal junction. A developing fluid pocket and surrounding phlegmon approximately 7 mm in size was noted, suggestive of possible abscess or early fistulous tract formation. The decision was made for ileocolic resection, which showed evidence of a bowel obstruction with dilated proximal small bowel and an unanticipated intussusception in the mid jejunum with a palpable mass. Additionally, there was active ileal Crohn disease involving the last 20 cm of the terminal ileum with associated stricture. Gross examination revealed a 4.2 cm well-circumscribed, gray-white, lobulated mass with a fleshy cut surface in the jejunum. Histologic sections showed a submucosal lesion with focal extension into the mucosa consisting of bland plump spindle cells embedded in a loose fibrous stroma and infiltrated by numerous eosinophils . ImmunohTo our knowledge, there have been 41 cases of IFP reported to occur in the jejunum to date, the majority of which are individual case reports and a few case series, summarized in PDGFRA, they are considered to represent variations of the same tumor and not separate entities [PDGFRA mutations, IFPs are common in individuals with germline mutations in the PDGFRA gene, and most IFPs express PDGFRA by immunohistochemistry [IFPs resemble other spindle cell tumors of the GI tract presenting as submucosal or mucosal masses: gastrointestinal stromal tumor (GIST), inflammatory myofibroblastic tumor (IMT), hamartomatous polyps, solitary fibrous tumor (SFT), leiomyoma, and nerve sheath tumors –48. Immuentities , 49. A pentities . There ihemistry , 49–51. hemistry , 52, 53.PDGFRA mutations may lack CD117 expression and are more likely to have epithelioid morphology [GISTs typically present as well-circumscribed gastric masses arising from the muscularis propria . In the rphology . There irphology –59. ConcIMTs may also present with intussusception, and the most common sites include the liver and biliary tract, spleen, peritoneum, stomach, and colon-rectum while the jejunum-ileum, duodenum, pancreas, and esophagus are less commonly affected sites , 61. IMTSFTs are exceedingly rare in the gastrointestinal tract –82. HistAmong CD34 negative tumors, immunoreactivity for SMA and desmin helps to establish a diagnosis of leiomyoma/leiomyosarcoma. Schwannomas and neurofibromas diffusely express S-100 and SOX10, and perineuromas are positive for EMA, Glut-1, and claudin-1. Perivascular epithelioid cell neoplasms (PEComas) are exceedingly rare in the GI tract, consist of a mixture of epithelioid and spindle cells, and can be recognized by the coexpression of melanocytic  and myogenic markers (SMA and desmin). Similar to SFT, these tumors may be overtly malignant.PDGFRA rearrangements [Inflammatory polyp of Crohn disease may be considered in the differential diagnosis of IFP when encountered at specific segments of the GI tract, most notably the distal pylorus and distal ileum . Hamartongements .PDGFRA appear to define this lesion molecularly and are responsible for the recruitment of eosinophils. PDGFRA mutations have been detected in both CD34 positive and negative cases suggesting they represent variants of the same entity. Immunohistochemical staining and mutation analysis for PDFGRA were not available and a limitation of this case study. Surgical excision is curative for symptomatic IFPs with little risk of recurrence.IFP is a distinct histologic entity that should be distinguished from other mesenchymal tumors of the GI tract, particularly those consisting of spindle-shaped tumor cells. Immunohistochemical studies performed on the surgical specimen should aid in the final diagnosis. Although the majority of IFPs express CD34, negative cases have been described as atypical IFPs. Activating mutations of