Pancreatic pseudocyst is a common complication that can occur following acute or chronic pancreatitis. Commonly, they are peripancreatic in location. Rarely, they can extend to the mediastinum, and further extension to the neck is even rarer. A 55-year-old man who presented with neck stiffness and dysphagia and on imaging, was found to have a cystic lesion in the neck. Aspiration of the lesion revealed raised amylase levels suggestive of a pancreatic pseudocyst. Rarely, they can extend into the mediastinum through anatomical defects in the diaphragm.6 Further extension to the neck is even rarer and has been described in a few case reports only.9 These can present with neck swelling or mass effect on the adjacent structures, and patients may present with complaints of dysphagia, chest pain or shortness of breath. Due to the varied clinical presentation, diagnosis is often challenging. Cross-sectional imaging such as CT is an excellent tool to establish the diagnosis.Pancreatic pseudocyst is a very common complication that can occur following either acute or chronic pancreatitis. It is seen in about 30% – 40% of patients with chronic pancreatitis.This report describes the case of a 55-year-old man with cervical extension of a pancreatic pseudocyst.A 55-year-old male patient presented to the Emergency Department with complaints of neck stiffness and dysphagia for one week. There was no history of trauma or fever. Occasional alcohol intake was documented. At examination, the patient had limited neck movements along with tachypnoea. However, the lung auscultation findings were normal. The abdomen was soft and non-tender with no obvious palpable lump.A radiograph of the neck and cervical spine was obtained , which iA contrast-enhanced CT scan of the neck and chest was conducted. It revealed a hypodense peripherally enhancing cystic lesion involving the prevertebral and retropharyngeal spaces of the neck, extending from the base of the skull to the level of the thoracic inlet . FurtherOn subsequent probing, the patient provided a history of acute pain in the epigastric region eight weeks prior, which gradually subsided over a few days. Hence, the diagnosis of pancreatic pseudocyst extending to the mediastinum and neck was made.As the patient was symptomatic, the cervical cyst was drained intraorally. Analysis of the aspirate indicated leucocytes and raised amylase levels (55 043 U/L), confirming the diagnosis. The pseudocyst resolved and the patient’s recovery was uneventful.1 In the paediatric population, additional causes include genetic abnormalities such as cystic fibrosis, pancreatic anomalies, ingestion of medications such as antiepileptics and metabolic disorders.10 Following an episode of acute pancreatitis, up to four weeks from the onset of pain, fluid collections associated with interstitial oedematous pancreatitis are defined as acute peripancreatic fluid collections according to the revised Atlanta classification for pancreatitis. If the fluid fails to resorb after four weeks and develops a mature wall, the term pseudocyst is used.11 Pathologically, a pseudocyst of the pancreas consists of a fluid collection surrounded by fibrous tissue with no epithelial lining.12 The fluid is rich in amylase, lipase and other enzymes produced by the pancreas. The symptoms are usually non-specific, but the possibility of pseudocyst formation should be considered in patients with persistent abdominal pain or a palpable mass in the abdomen following an episode of pancreatitis.1 Serum analysis is not reliable, as patients may or may not have elevated serum lipase and amylase levels and/or raised serum bilirubin levels.1Pancreatic pseudocysts are common complications of both acute and chronic pancreatitis. The aetiology of pancreatitis and hence pseudocyst includes excessive alcohol consumption, biliary tract pathologies and trauma.13 One case report also describes a pseudocyst presenting as an inguinoscrotal swelling.14 These atypical locations cause a diagnostic dilemma due to the varied clinical presentations. Hence a thorough clinical history along with cross-sectional imaging is required to make the diagnosis.Pancreatic pseudocysts are commonly peripancreatic in location, involving the lesser sac. Occasionally, pancreatic pseudocysts can occur at atypical locations like the splenic hilum or within the spleen and mediastinum. Extension further cranially to the neck resulting in retropharyngeal collections is fairly uncommon and has only been described in a few reports.6 A case of an isolated mediastinal pseudocyst with no obvious communication with the pancreas has also been reported.15 The most common location for a mediastinal pseudocyst is the posterior mediastinum, although they can be seen in the anterior or middle mediastinum. It occurs when there is extension of the fluid from the retroperitoneum through the aortic or oesophageal hiatus and sometimes through the diaphragmatic crura.13 Patients may present with shortness of breath, chest pain, fever, palpitations or sometimes gastroesophageal reflux due to hiatal widening.5 Occasionally, they may cause life-threatening complications due to mass effect or rupture of the pseudocysts in the pleural or pericardial cavity resulting in cardiac tamponade, circulatory failure or acute respiratory distress.6 Pseudocysts extending to the neck can result in dysphagia or can present as a neck swelling. In the presented case, mediastinal extension of the pseudocyst was seen through the oesophageal hiatus with further extension to the neck. The patient presented with complaints of neck stiffness and dysphagia due to mass effect on the oesophagus along with retropharyngeal and prevertebral extension.Mediastinal extension of a pancreatic pseudocyst although previously reported, is a relatively rare entity.16 along with its extension into the neck, when present. It is useful to evaluate the complete extent of the lesion and its relation to the visceral and vascular structures. Magnetic resonance imaging is useful to demonstrate pancreatic ductal anatomy and its communication with the pseudocyst. Ultrasonography can be used to guide the aspiration of fluid from the cyst for both diagnostic and therapeutic purposes. The presence of raised amylase levels in the fluid is highly sensitive and specific for the diagnosis of a pseudocyst of pancreatic origin.1Cross-sectional imaging plays an important role in the diagnosis of a mediastinal/retropharyngeal pseudocyst. Computed tomography demonstrates a hypodense thin-walled cystic lesion usually in the posterior mediastinum,17 Other differentials for mediastinal pseudocysts include neurofibroma, cystic schwannoma and other posterior mediastinal tumours, lymphoma, aortic aneurysm and extramedullary haematopoiesis.6 Cervical pseudocysts may mimic other cystic lesions of the neck including cystic lymph nodes, cystic lesions of the thyroid or salivary glands and lymphatic malformations.13A mediastinal pseudocyst must be differentiated from a paravertebral abscess. Pseudocysts extending to the neck can be misdiagnosed as a retropharyngeal or prevertebral abscess at the preliminary examination. The clinical history, patient presentation and imaging findings aid in the diagnosis. Tuberculosis of the spine can cause paravertebral abscess formation, and patients present with long-standing history of fever, loss of appetite, weight loss, backache and localised tenderness. Typically, there is the involvement of the vertebrae adjacent to the paravertebral collections.18 Surgical drainage or excision is limited to pseudocysts that fail to resolve by the above techniques or when they become complicated.6Management depends on several factors including the symptoms of the patient, location, extension of the lesion and its relation to major structures. In asymptomatic patients and patients with small lesions, medical management with octreotide may be useful. Drainage of the fluid can be performed using percutaneous or endoscopic techniques. It has been reported that the recurrence rate is lower following endoscopic drainage compared with percutaneous drainage techniques. Complications following percutaneous drainage include haemorrhage, infection and fistula formation.In any patient presenting with dysphagia along with a cystic lesion in the neck, cervical extension of a pancreatic pseudocyst must be considered as a differential under appropriate clinical settings.


Multiple extramedullary plasmacytoma (EMP) of the head and neck is rare and a definitive first-line treatment has not been established. EMP successively as a benign mass occurring in multiple areas of the head and neck is rare. This report describes an atypical case with EMP occurring in multiple areas of the head and neck.A 35-year-old woman was admitted due to complaints of sore throat discomfort accompanied by hoarseness. The patient had undergone surgical excision of the thyroid gland and parotid gland excision several years ago. Postoperative pathological examination both indicated EMP. This time, the woman suffered EMP in head and neck who was treated with a simple surgery.Postoperative pathological examination of the tumor indicated EMP, and histopathological findings revealed the tumor to be a plasmacytoma. Immunopathological examination were consistent with the diagnosis of EMP.The patient underwent surgical resection without radiotherapy.Histopathological and immunopathological examination findings revealed the tumor to be EMP. The patient was recurrence-free and did not progress to multiple myeloma (MM) during 19 months follow-up.Increasing the awareness of EMP of head and neck is warranted. Our case confirmed that surgical excision is beneficial in the treatment of small, localized EMP. Burgos-Blasco et al suggested that EMP accounts for only 3% of all plasma cell neoplasms. Christoph et al revealed that EMP belonging to the category of non-Hodgkin lymphoma is a rare entity. EMPs account for 4% of all plasma cell tumors. Guowei Li et al showed that EMP makes up about 5% of all plasmacytomas. EMP reveals a favorable prognosis compared with other plasma cell dyscrasia, such as solitary plasmacytoma of bone and multiple myeloma (MM). Most cases of EMP are seen in older men , with a peak incidence in the 50 to 60-year-old group, with no racial predilection. EMP patients characteristically present with localized disease, and the incidence of lymph node involvement fluctuates around 10% to 20%. Owing to the rarity of EMP, most studies had been retrospective series and case reports, with no randomized trials. Experience in the diagnosis and treatment of EMP is limited, and EMP of the larynx has not been thoroughly studied. The multiplicity has been rarely presented. To provide references for clinical therapy, we describe the rare case of EMP arising from the larynx.Plasmacytomas are essentially characterized by abnormal monoclonal proliferation of mature B-cells, producing monoclonal globulin by clonal expansion. Extramedullary plasmacytoma (EMP) is an uncommon plasma cell neoplasm.A 35-years-old female patient suffering from sore throat discomfort accompanied by hoarseness lasting for a month was diagnosed with a laryngeal tumor in February 2021. The patient did not present dysphagia or dyspnea, and she had never smoked in her life. Her medical history did reveal an abnormality. The particularity is that she had undergone surgical excision of the thyroid gland in another institution and excision of the parotid gland in stomatology department of our hospital after several months. Postoperative pathological examination both indicated EMP. The patient was admitted with swelling in the left parotid area and had a fever. The palpable mass in the left parotid gland area was 3.0 cm × 2.0 cm × 2.0 cm in size, with no tenderness. Ultrasonography revealed a solid lesion on the left parotid gland, and multiple swollen lymph nodes were detected bilaterally on both parotid glands. A biopsy was performed before surgery, and the mass was revealed to be benign lesions of the salivary glands or inflammation. The patient underwent an excision of the parotid gland mass and facial nerve dissection. Intraoperative rapid pathological examination of the lesion indicated lymphoproliferative lesions, and the postoperative pathological examination consultation results indicated plasmacytoma. Immunopathological examination revealed that CD56 lambda was negative and that CD138 kappa was positive. No neck lymph nodes enlargement was palpable. Electronic video laryngoscopy revealed a mass with a smooth surface and normal mucosal color in the nasopharynx, the right wall of the oropharynx, aryepiglottic fold, and ventricular zone Fig.  revealedThe patient underwent a resection of the tumor with a suspension laryngoscope, with no administration of further therapy. The mass was excised with a plasma cutting machine Fig. . The masPostoperative pathological examination of the tumor indicated EMP, and histopathological findings revealed the tumor to be a plasmacytoma Fig.. ImmunopAfter follow-up for 20 months, the patient was in good condition following surgery. The thyroid and parotid gland tumor had been excised for 6.5 years. After surgery, the patient underwent further investigations. The tumor was recurrence-free and did not progress to MM during follow-up. Bone marrow aspiration performed at another hospital revealed to be normal with no evidence of tumor deposits. A renal function panel, a complete blood count, electrolyte, and calcium measurements, were all within normal limits. Moreover, the laryngoscope showed normal postoperative changes Fig. . Our patient successively found EMP masses in the thyroid gland, parotid gland, larynx, nasopharynx, and lateral pharyngeal wall, which is a rare occurrence. However, the patient’s first thyroidectomy was performed in another hospital, and we remain unaware of their management protocols. In both of the surgeries performed in our hospital, preoperative biopsies were used to confirm the diagnosis of EMP, and the results of postoperative cases were obtained through outpatient consultation. The patient did not undergo radiotherapy or chemotherapy on the 3 occasions, and the tumors did not invade the surrounding bone and cartilage.A plasma cell tumor is a malignant tumor caused by abnormal proliferation of the plasma cell system. Plasma cell neoplasms entail several chronic diseases, such as solitary plasmacytoma of bone, EMP, and MM. EMP occurs outside the bone marrow and can appear in any organ system. and tumor size, EMP of the larynx often presents with hoarseness or dysphagia, without special clinical manifestations, due to the non-specificity in the clinical symptoms, laryngoscope findings, and imaging examinations, it is easily misdiagnosed. The diagnosis of EMP is based on the exclusion of systemic plasma cell proliferative disorders and immunohistochemistry results. Histological diagnosis of EMP requires the presence of clonal plasma cells. Immunohistochemical markers include CD79a, CD38, and CD138, and monoclonal cytoplasmic lambda or kappa light chain expression. José Antônio Pinto et al suggested that imaging tests rule out lytic lesions. MRI is the test of choice to highlight the lytic lesions. CT usually demonstrates a well-defined or infiltrative soft tissue mass, appearing with a mild-to-moderate contrast enhancement. The MRI scans of most EMPs is iso/hypointense to skeletal muscle on T1 and iso/hyperintense on T2-weighted image. Diffusion weight imaging demonstrates restricted diffusion, indicating high cellularity of the lesions. Caers J et al demonstrated that CT might be helpful for an adequate loco-regional staging to EMP as regional lymph node recurrences occur in 7% of EMP cases. It can help with the observation of the size and location of the mass and determine the site of tumor invasion. Although the sensitivity of MRI to detect lytic bone lesions is lower than that of CT, MRI can detect soft tissue. The use of diffusion-weighted MRI, which is a new, highly sensitive technique to detect and monitor tumor lesions in the soft tissues, could improve the ability of MRI to discern between active and inactive lesions. Caers J et al recommended that in addition to the skeletal survey or CT, MRI or PET-CT scans are supposed to exclude the presence of additional lesions, and the use of at least one of these examinations is mandatory. Salaun et al reported that PET-CT outperforms MRI in the diagnosis and follow-up of plasmacytoma patients, although this study was performed in the context of extramedullary spread during MM. Although, over the past decade, PET-CT has increasingly been used in the prognosis of MM; however, its routine use is still hampered by several factors, including high cost, disparities in insurance policies between regions, and limited availability. Ryu SW et al suggested that the use of ultrasonography is limited by the location of the plasmacytoma, such as in the abdomen, breast, subcutaneous tissue, and skeletal muscles. In our case, we performed CT, MRI, and biopsy before surgery, and no malignant tendencies were noted. Therefore, we did not perform bone marrow puncture or radiation therapy after surgery.Although the clinical presentation varies according to the involved organ Ge et al suggested that surgical excision, either alone or with adjuvant radiotherapy, has been proposed and proved to be more beneficial than radiotherapy alone. Takahiro et al showed that surgical excision offers better survival outcomes in the therapy of small and localized EMP of the larynx. Alexiou et al showed that surgery suffices provided the mass is localized and resectable. We did not find any cases of successive EMP of the head and neck during the literature review. The prognosis of patients is no recurrence or progression of MM. However, imaging investigations in our patient did not reveal an aggressive and invasive tumor. On the contrary, they were isolated and were surgically excised without radiotherapy. No local recurrence or deterioration has been observed since the first surgery. Hence, the treatment regimen should be devised with caution, and laryngeal function should be preserved.The optimal therapy of EMP remains controversial. Due to the rarity of EMP, most studies had been retrospective series and case reports. It is generally considered that treatment strategies included surgical excision, systemic chemotherapy, radiotherapy, or a combination of those. suggested that the 5-year local overall survival rates are between 78.4% and 87.4% for EMP. Guowei Lu et al indicated that cumulative 5-year survival rates are 80%, but about 20% of patients of EMP are prone to recurrence, and 15% of patients can progress to MM. Kamijo et al suggested that cumulative 5-year survival rates for EMP fluctuate between 50% and 60%. Susnerwala et al indicated that patients with lambda light chains have a worse prognosis than patients expressing kappa light chains. Pratibha et al revealed that EMP prognosis does not depend on local staging. The evolution of EMP to MM is the determinant factor for survival. As a result, an EMP differential diagnosis should be considered in cases presenting laryngeal lesions. All laryngeal masses have to be biopsied prior to therapy to establish an accurate diagnosis, thereby leading to proper management protocols. Therefore, long-term follow-up is necessary to monitor local recurrence and the progression of MM, including local control and systemic observation via blood counts and immunoglobulin measurements. Definitive first-line treatment in EMP is based on small-scale studies due to the low incidence of EMP. Therefore, larger-scale studies are supposed to further establish and refine the current diagnosis and management guidelines, entailing major improvements in the quality of life of patients and prognosis. The specialness of our case was that the patient presented EMP masses in thyroid gland, parotid gland, larynx, the right wall of the oropharynx and nasopharynx successively, all of which were treated surgically without postoperative adjuvant radiotherapy and chemotherapy. There were no signs of progression to MM during follow-up. Unfortunately, the patient’s previous 2 surgeries were not performed in our department, and the patient’s medical history was not fully grasped. And due to insufficient understanding of the disease, the patient did not undergo bone puncture in time. When we realized that, the patient had done it in other hospital. Another special feature is the plasma that was used in this time. Low-temperature plasma radiofrequency ablation has been used in the treatment of various benign and malignant diseases of otolaryngology since 2000, but EMP resection has not been reported. We do not know what will happented to our patient, but we will continue to follow up.EMP is a localized entity. However, in 16% of the cases, the EMP can deteriorate to MM, and the progression usually affects the prognosis. Therefore, the diagnosis of EMP is based on the exclusion of MM. Systemic examinations rule out bone involvement. In addition to tumor biopsies, comprehensive laboratory investigations including complete blood cell count, urinalysis, abdominal ultrasonography, bone X-ray examination, immunoglobulin test, and bone marrow cytology should be performed. EMP has good prognosis, Glasbey et alIncreasing the awareness of EMP of head and neck is warranted. Our case confirmed that surgical excision is beneficial in the treatment of small, localized EMP. Plasma is also a possible way to treat diseases.Data curation: Detao Ding, Xu Ma.Project administration: Juxing Sun, Hui Zhang.Resources: Yungang Wu.Writing – original draft: Jin Zhang.Writing – review & editing: YunBing Dai, Xiaoying Li, Xiaoyu Li, Yungang Wu.


Aneurysmal bone cysts (ABC) are rare in the paranasal sinuses. They are benign expansile multicystic masses containing blood-filled spaces which typically occur in the long bones of pediatric patients. The lesion often produces symptoms due to the compression of adjacent structures or pathological fracture and depends on localization. In this case report, we discuss a 28-year-old female who presented with left-sided headache, left eye proptosis, and diplopia. Radiologic evaluation revealed a left paranasal sinus expansile multicystic mass with internal blood fluid levels displacing and thinning the left medial orbital wall which suggested the diagnosis of ABC. Radiologists should be familiar with and comfortable diagnosing ABC in the head and neck, and be able to differentiate this entity from others, such as telangiectatic osteosarcoma. Biopsy can be challenging since blood products may be the only material identified and may produce tissue that is difficult to interpret or misdiagnosed. Aneurysmal bone cysts (ABC) are described as benign cystic osseous lesions containing blood-filled spaces separated by connective tissue septa A 28-year-old female stationed in Guam as a military dependent presented virtually via tele-health to her physician due to COVID-19 pandemic limitations complaining of a 1-month history of worsening left-sided headaches, vision changes, and left eye proptosis. Due to limited physical exam capabilities virtually, she was treated for migraines with outpatient referral to ophthalmology. She presented 2 weeks later to the emergency department with worsening symptoms where imaging was performed.Initial unenhanced CT  performeFollowing discharge, and while still awaiting biopsy results, an MRI was obtained . This stThe biopsy results revealed the lesion to be consistent with an ABC. Though benign, the mass already proved to be locally destructive in a short time, and given concern for worsening orbital and intracranial complications, surgical removal was recommended. Given the complex surgical requirements necessary for management, the patient was immediately transferred to the United States for specialty care.Given the destructive appearance of the mass radiographically, and clinical evidence of invasion outside of the sinonasal cavity, a diagnostic surgery with partial tumor removal inferiorly and centrally was performed to allow for reconfirmation of pathology and to exclude a separate underlying process prior to definitive treatment.Intraoperatively the mass was not highly vascular, but there were areas of hemorrhage with interspersed bony fragments. The peripheral and central portions of the mass had slightly different characteristics so multiple excisional biopsies were taken. The nasal septum was still intact despite concern for erosion preoperatively. The lamina papyracea on the left was completely eroded and the mass was abutting the orbital contents. Despite our concerns for a more sinister process, ABC was again confirmed on all repeat biopsies. The case was discussed at the institution's multidisciplinary tumor board and definitive surgical extirpation was planned. A staged surgery allowing for complete removal of the mass followed by definitive orbital reconstruction to correct the proptosis and extraocular muscle impairments was carried out successfully. Postoperatively, the patient recovered without significant complication and the final pathology again confirmed ABC.ABCs are relatively rare benign bone lesions, which typically occur in the long bones of pediatric patients. They typically arise in the first 2 decades of life, with a mean age of 11.6 years and a 2:1 female to male predilection Presentation of ABC is somewhat variable, and as an expansile lesion will cause symptoms of compressive mass effect and depends on localization. When occurring in the head and neck, the most common presenting symptom is pain and swelling of the affected area ABC were first described in 1942, by Lichtenstein and Jaffe, as “blood-containing osseous tumor” and were said to have similar radiomorphologic characteristics to aneurysms Histopathologic diagnosis is essential in confirming an ABC and ensuring there is no malignancy. Excisional biopsy is preferred over fine needle aspiration and incisional biopsy. Fine needle aspirations tend to only return blood products whereas incisional biopsy is not as accurate and may cause significant hemorrhage Radiologic evaluation with CT and MR is essential in providing extent of involvement and suggesting the diagnosis. The presence of an expansile lesion, internal septa, and fluid-fluid levels is characteristic of ABC; however, these findings can also be seen in a variety of other lesions such as chondroblastoma, giant cell tumor, and telangiectatic osteosarcoma. CT is useful in delineating a cortical rim and evaluating the amount of bony remodeling that is affecting adjacent structures MRI is, therefore, very useful in suggesting the diagnosis and evaluating for the presence of an underlying lesion/secondary etiology. Fluid-fluid levels are characteristic of ABC and are the result of internal blood products of different ages which will vary in signal intensity and will be most prominent on the fluid sensitive sequences Although ABC are benign, treatment is usually pursued because of mass effect symptoms and discomfort. Surgical resection is usually pursued and depending on the location a gross total or subtotal resection may be pursued, although recurrence is higher with a subtotal resection Sinonasal ABC are rare lesions and may present a diagnostic challenge. Radiological features are crucial to predict the diagnosis which should be considered in a patient presenting with an expansile mass with internal fluid-fluid levels. The presence of a soft tissue mass or enhancement of the cystic components suggests a different diagnosis and histopathological diagnosis is necessary to exclude malignant lesions.


Bilateral ureteroinguinal hernia is a rare presentation with only five cases previously presented in the literature. We describe a 60-year-old male who was diagnosed with large bilateral hernias extending into the scrotum containing redundant ureters. Both hernias were repaired in staged fashion with retrograde temporary ureteral stent placement and modified Lichtenstein technique. Follow-up computerized tomography imaging with >5 years follow-up revealed intact repairs with no evidence of urinary obstruction. Inguinal hernia repair is one of the most common operations performed in the United States with over 800 000 surgeries annually . UreteroMost patients diagnosed with herniation of urologic organs have no symptoms of urologic pathology. The most common diagnosis is in obese men in their 50s or 60s . Some paWe report a case of large bilateral ureteroinguinal scrotal hernia identified pre-operatively and repaired successfully in a staged fashion.This is a 60-year-old male with a body mass index of 45.4 who was referred to the emergency room with a complaint of acute onset left flank pain. Past medical history was significant for hypertension and hyperlipidemia. Past surgical history was notable for appendectomy and multiple right lower extremity operations as a child to correct deformities from polio. He continued to require right lower extremity bracing. At the time of diagnosis, he had no additional gastrointestinal or urinary symptoms. Examination was notable for bilateral groin bulge suggestive of hernia versus hydrocele. The complete blood count, basic metabolic panel and urinalysis were unremarkable. A computerized tomography (CT) scan of the abdomen was performed and revealed bilateral inguinal hernias containing ureters and fatty tissue . Mild riThe patient was taken to the operative suite where he underwent cystoscopy, retrograde pyelogram and temporary ureteral stent placement . Right iThe patient was allowed to fully recover over the next 4 months. He then returned to the operating suite and a nearly identical procedure was performed on the left side. His post-operative course was uneventful. At a 6-month follow-up, the patient exhibited no recurrence of his hernias, markedly decreased size of the scrotum and significant improvement in urinary soiling due to retraction of the phallus.CT abdomen performed >5 years after repair revealed intact bilateral inguinal hernia repair and no evidence of hydronephrosis.Bilateral ureteroinguinal hernia has rarely been described in the literature and there are not clear guidelines for best management. There are two variations of ureteroinguinal hernias, paraperitoneal and extraperitoneal. Paraperitoneal comprises 80% and has an indirect sac that pulls on the ureter. Extraperitoneal occurs without a peritoneal sac, and the ureter moves with the retroperitoneal fat into the scrotum and is thought to be a congenital defect in which the Wolffian duct fails to separate from the ureteric bud .Due to the high volume of inguinal hernia surgery and low incidence of herniated genitourinary organs, it is difficult to recommend preoperative imaging for all patients. Performing a quality preoperative history may be beneficial inquiring about two-stage micturition, hematuria, acute urinary obstruction or ipsilateral flank pain. Patients with suspicious history may warrant preoperative imaging with ultrasound or CT scan . InteresIt has been consistently reported that ureteroinguinal hernia is associated with large cord lipomas and sliding fat, especially in obese patients in their 50s and 60s. It is important to consider this possibility to limit iatrogenic ureteral injury.In patients who have ureteroinguinal hernia identified pre- and post-operatively management options that have been reported include anterior repair with reduction into the retroperitoneum, percutaneous nephrostomy and stenting, retrograde stenting, nephrectomy, exploratory laparotomy and resection of redundant ureter with re-implantation , 11. TheIn our patient, we elected to perform repair in a staged fashion to ensure the patient did not develop obstruction prior to proceeding with contralateral repair. Given the large size of the hernias, risk of ureter injury and the decision to perform a staged approach, we chose to perform open repair rather than minimally invasive. Retrograde stenting was beneficial to facilitate identification of ureters and decrease the risk of injury. Retrograde pyelogram after completion of repair confirmed no obstruction prior to leaving the operating suite. This approach resulted in a successful outcome with a greater than 5-year follow-up.


Arising from the autonomic paraganglia of the neuroendocrine system, paragangliomas are rare neoplasms that are derived from the embryonic neural crest. Primary paragangliomas of the lung are exceedingly rare, with little known about their origin. Here we present a 47-year-old female presenting in 2021, one year after a COVID-19 infection, with symptoms of tachycardia, shortness of breath, and palpitations which she associated with presumed long COVID-19. An imaging workup noted a 1.5 cm nodule in the lingula of the left lung. She then had surgical resection of the nodule, which was found to be a primary lung paraganglioma. A follow-up dotatate positron emission tomography (PET) CT noted no residual disease, and genetic testing was negative for known mutations. This case demonstrates the need for close monitoring with follow-up for incidental findings in order to provide a timely and accurate diagnosis in accordance with guideline criteria. Paragangliomas are neuroendocrine tumors derived from the embryonic neural crest . They maParagangliomas are rare tumors with an incidence rate combined with pheochromocytomas of 0.8 per 100,000 person-years . PrimaryIn October of 2021, a 47-year-old female with a past medical history of follicular thyroid adenoma with surgical removal in the year 2000 and a 4 mm nodule in the lingula of the lung noted on chest CT in the year 2014 presented to the pulmonology clinic for evaluation of tachycardia, shortness of breath and dyspnea on exertion for the past four to six months. She had previously been evaluated with an electrocardiogram (ECG) in the emergency department and a stress test that showed no evidence of myocardial ischemia. She reported a previous history of COVID-19 in 2020, but since that period, she has had no symptoms of tachycardia or shortness of breath. Because of her new-onset dyspnea and tachycardia, she presented to the pulmonology clinic for evaluation. Initial work-up included a computed tomography pulmonary angiogram (CTPA) of the chest to rule out pulmonary embolism, which found no acute findings but did note an increase in the size of the known 4 mm noncalcified nodule in the lingula of the left upper lobe Figure . This noA discussion between pulmonology and cardiothoracic surgery determined that because of the increase in the size of the pulmonary nodule, it would be better to be resected at that time rather than wait for it to further increase in size. The patient underwent a video-assisted thoracoscopic surgery with a left upper lobe lingular wedge resection in December of 2021. The patient did well in the postoperative period with no immediate recurrence of shortness of breath or chest pain, or any symptoms of infection. The pathology report noted a 15 x 12 x 12 mm nodule with negative borders described as tan to gray-white with diffusely positive chromogranin and synaptophysin Figure . It alsoThe patient was then referred to hematology/oncology for evaluation, who ordered plasma-free metanephrines and plasma catecholamines, of which all were within normal limits. She was referred to medical genetics and underwent genetic testing with no genetic mutations noted, including multiple endocrine neoplasia type 2 (MEN2). A referral was placed to a regional academic medical center hematology/oncology department for further evaluation, and the recommendations were to organize follow-up surveillance with repeat CT of the chest at six-month intervals along with plasma free metanephrines. She also was referred to cardiology for further evaluation of her ongoing palpitations and intermittent tachycardia.A review of the literature up to this point seems to indicate that a majority of known paragangliomas are sporadic, though it has a predilection to appear in middle-aged females . FunctioPPP is still exceedingly rare in literature, and there are no pathognomonic radiology findings that would have led to consideration for this disease preoperatively. With our patient, there has been no indication of local invasion or metastasis, which is consistent with literature citing that only about 10% of paragangliomas are malignant . MetastaFleischner Society guidelines are the standard for the management of most incidentally found pulmonary nodules found on imaging . In genePrimary pulmonary paragangliomas are exceedingly rare tumors of the neuroendocrine system. The majority of these are non-secreting and slow-growing, as presented in this case. Many are found incidentally on imaging studies and should be followed up based on Fleischner Society pulmonary nodule guidelines. In this case, the option to perform a wedge resection of the lingula to remove the nodule was elected based on the increase in the size of the nodule over a seven-year period. After resection of the nodule, genetic testing should be performed to rule out disorders, such as MEN2. This case demonstrates the need to have a broad clinical diagnosis in the management of incidental imaging findings and close follow-up in accordance with guideline criteria.


Desmoid tumors are extremely rare borderline benign and malignant tumors that do not exhibit accumulation on fluorodeoxyglucose positron emission tomography–computed tomography. In the present study, we report a rare case of a desmoid tumor with fluorodeoxyglucose accumulation at the anastomotic postoperative gastric cancer site.A 68-year-old Japanese man underwent robot-assisted laparoscopic distal gastrectomy for early-stage gastric cancer in 2019. The pathological diagnosis was stage IA cancer, and no adjuvant chemotherapy was administered. Two years after surgery, a soft mass appeared on the greater curvature side of the anastomosis on computed tomography. Fluorodeoxyglucose positron emission tomography–computed tomography revealed fluorodeoxyglucose accumulation, which suggested a malignancy; therefore, surgery was performed for diagnostic treatment. The histopathological findings led to the diagnosis of a desmoid tumor. The patient has not experienced recurrence to date.In the present study, we encountered a desmoid tumor arising from the anastomotic site of a postoperative gastric cancer. This case is rare as fluorodeoxyglucose positron emission tomography–computed tomography showed fluorodeoxyglucose accumulation in the desmoid tumor, and a preoperative diagnosis could not be reached. We hope that further studies will improve the accuracy of preoperative diagnosis. Desmoid tumors are extremely rare benign or malignant borderline tumors, and mechanical stimuli such as surgery are considered triggers for their development. Preoperative diagnosis with standard imaging modalities is difficult, and desmoid tumors do not exhibit any accumulation of fluorodeoxyglucose on positron emission tomography–computed tomography (FDG-PET/CT). In the present study, we report the case of a desmoid tumor with fluorodeoxyglucose (FDG) accumulation at the anastomotic site following robot-assisted laparoscopic distal gastrectomy, along with a review of the literature.A 68-year-old Japanese man was found to have an extensive type 0–IIc lesion in the upper gastric body extending up to the hypogastric lesser curvature on upper gastrointestinal endoscopy in September 2019. Biopsy revealed a poorly differentiated adenocarcinoma; the patient was referred to our department for surgery. He had hypertension and hyperlipidemia. However, he had no family history of malignancy. The patient underwent robot-assisted laparoscopic distal gastrectomy, D1+ lymph node dissection, and Roux-en-Y reconstruction in November 2019. The patient had a good postoperative course and was discharged 6 days postoperatively.The initial surgical specimen findings were as follows Fig. : a 50 × The patient was followed-up without adjuvant chemotherapy; however, a 38 × 36 mm soft tissue mass was noted on the greater curvature side of the residual stomach during computed tomography (CT) 2 years after the procedure. Although partial thickening of the gastric wall was seen retrospectively in a 1-year postoperative CT scan, it was reviewed within normal limits by a radiologist at that time , 1.8 ng/mL; carbohydrate antigen 19-9 (CA19-9), 8.5 U/mL; and soluble IL-2 receptor, 396 U/mL. Colonoscopy had been performed prior to the first surgery; hence, it was not performed again.The FDG-PET/CT findings were as follows Fig. : The sofContrast-enhanced magnetic resonance imaging (MRI) of the abdomen revealed the following findings Fig. : the sofUpper gastrointestinal endoscopic findings were as follows Fig. : no massThe biopsy results were as follows: the tumor contained multiple spindle-shaped cells, and immunostaining for CD34, c-kit, desmin, S-100, and β-catenin was performed to distinguish spindle-shaped tumors; however, no significant positive findings were obtained. The biopsy specimen did not allow us to estimate the histological type of the tumor as it did not conclusively suggest a GIST, smooth myxoid, schwannoma, or desmoid tumor.Based on the above-mentioned examination results, local recurrence of gastric cancer, lymph node metastasis, a GIST, a smooth myxoid, schwannoma, and a desmoid tumor were regarded as possible differential diagnoses. Although the possibility of a benign tumor remained, a definitive diagnosis could not be made histopathologically, and moderate FDG accumulation could not rule out a malignant lesion; thus, we decided to operate for diagnostic treatment. Preoperative CT revealed a suspicious finding of splenic arteriovenous invasion; therefore, total residual gastrectomy and splenectomy were planned.The surgical findings were as follows: the abdomen was opened via a median upper abdominal incision. A 50 × 40 mm soft tissue mass originating from the gastrojejunal anastomosis involving the spleen and splenic arteriovenous was observed on the greater curvature side of the anastomosis. Additionally, a partial invasion of the pancreas was observed. The remaining stomach, spleen, splenic artery and vein, and part of the pancreas, including the gastrojejunal anastomosis, along with the soft tissue mass, were removed as a single lump. Regarding reconstruction, only esophago-jejunal anastomosis was performed, sparing previous jejuno-jejunal anastomosis.The operation time was 279 minutes, and blood loss was 1100 mL; however, no blood transfusion was required. Pathological findings of the resected specimen were as follows Fig. : a 45 × The patient developed a postoperative pancreatic fistula that resolved after drainage. He was discharged from the hospital on postoperative day 16 and has not experienced any recurrence for 6 months.Desmoid tumors are a type of fibromatosis that are on the borderline between being benign and malignant, with an annual incidence of 2–4 per million people . Patholoet al. have reported 12 cases of intra-abdominal desmoid tumors with FDG uptake on PET/CT. They have reported that only one case showed high accumulation; however, the remaining 11 cases showed only mild accumulation with the maximum standardized uptake value (SUVmax) ranging between 1.95 and 3.9 [MRI has excellent tissue resolution and is the most useful imaging technique for diagnosing desmoid tumors, with T1-weighted images showing equal signals and T2-weighted images showing high or heterogeneous signals . However and 3.9 , 7. TherAs our patient was a postoperative gastric cancer patient, the moderate accumulation on FDG-PET/CT could not be ruled out as a recurrence of gastric cancer or lymph node metastasis. We decided to perform EUS-FNA in this case because some studies have mentioned that preoperative EUS-FNA should have been performed and some have reported that the diagnosis was made using needle biopsy , 9. In JThe local recurrence rate of desmoid tumors is extremely high, ranging between 56% and 86% . TherefoIn the present study, we encountered a desmoid tumor arising from the anastomotic site of a postoperative gastric cancer. The present case is rare in that FDG-PET/CT showed desmoid tumor accumulation, and a preoperative diagnosis could not be made. We hope that further studies will improve the accuracy of preoperative diagnosis.


CYP4V2 mutation and typically presents with crystalline retinal deposits in the posterior fundus.Bietti crystalline dystrophy is primarily a retinal dystrophy caused by a We present the case of an otherwise healthy 39-year-old Iranian woman with no family history of ocular disease who suffered with progressive vision loss that had started 2 years prior to presentation. Ocular examination revealed blurry optic nerve head margin and diffuse retinal crystalline deposit in both eyes. Spectral domain optical coherence tomography images showed retinal crystals, located mostly in outer retinal layers, with some areas of outer retinal tubulation and attenuation of outer retinal layers. Crystalline deposits were better visualized on near-infrared images as hyperreflective spots. Fundus autofluorescence images showed hyperautofluorescence areas on optic nerve head consistent with optic nerve head drusen and large hypoautofluorescence areas in posterior retina consistent with retinal pigment epithelium atrophy. Cystinosis was ruled out by blood testing.Bietti crystalline dystrophy may be associated with optic nerve head drusen. CYP4V2 gene that result in defective ocular fatty acid metabolism in retinal pigment epithelium and the formation of crystalline deposits [Bietti crystalline dystrophy typically occurs in the second to fourth decades of life, with symptoms of reduced visual acuity with or without nyctalopia , 2. Oculdeposits –7. In thA 39-year-old Iranian woman suffered from progressively reduced vision in both eyes that had started 2 years prior to presentation. Her parents were relatives, but there was no family history of ocular disease in the patient’s first- and second-degree family. The patient’s medical history and drug history were unremarkable. Blood testing and urine analysis were performed to rule out cystinosis and crystalluria. Written informed consent was obtained from the patient. A complete ocular examination—including optical coherence tomography (OCT), OCT angiography (OCT-A), infrared (IR) imaging, fundus autofluorescence (FAF) imaging, and electroretinography (ERG)—was performed.The best-corrected Snellen visual acuity in both eyes was 7/10. Anterior segment examinations were normal. A posterior segment examination revealed a bilateral blurry optic nerve head margin, a bilateral diffuse crystalline deposit at the posterior pole through mid-periphery, and reduced foveal reflex (Fig. SD-OCT revealed patchy atrophy of the outer retina Fig. B, E. OCTOn NIR images, crystalline deposits were better visualized as hyperreflective spots throughout the posterior retina in both eyes Fig. E, F. FAFThe foveal avascular zone was irregular, especially in the right eye in superficial and deep capillary plexuses Fig. . Large cERG showed moderate to severe reductions in both photopic and scotopic responses Fig. .Fig. 4NoCYP4V2 mutation was not performed for the patient owing to economic restraints.The most probable diagnosis according to the clinical examination and multimodal imaging was Bietti crystalline dystrophy with bilateral optic nerve head drusen. However, the confirmation of the diagnosis requires genetic testing. Genetic testing of CYP4V2 mutation and is presumed to affect fatty-acid omega-hydroxylase activity in retinal pigmented epithelium and accumulation of yellow-white crystalline-like deposits [Bietti crystalline dystrophy is primarily a retinal dystrophy caused by a deposits , 2, 8. Tdeposits , 5, 7. Tdeposits , 10. In deposits . Outer rdeposits . The sendeposits , 11. Retdeposits . Fluoresdeposits . The cliOptic nerve head drusen is a clinical condition that is usually asymptomatic and found incidentally but may be associated with retinal hemorrhage and visual field defects . Optic nTo the best of our knowledge, this is the first report on the association between optic nerve head drusen and Bietti crystalline dystrophy.


Superior mesenteric artery syndrome (Wilkie’s syndrome) is a rare cause of intestinal obstruction caused by a congenital or acquired reduction of the aorto-mesenteric angle leading to duodenal compression. We present the case of a 51-year-old female patient with a previous history of breast cancer. She was admitted to the Emergency Department with acute onset of recurrent vomiting, intense abdominal pain especially in the epigastric region, and abdominal distension. The ultrasound showed an absence of lower abdominal quadrants with an enlarged and distended stomach reaching the pelvis. The computer tomography scans confirmed the diagnosis of superior mesenteric artery syndrome. Conservative management was implemented, and using a nasogastric tube, and upped endoscopy approximately 4000 mL of fluid were aspirated with clinical improvement shortly after. The patient resumed a high-calorie diet and five months later, the patient was completely asymptomatic. Superior mesenteric artery syndrome (SMAS) is a rare gastrointestinal (GI) and vascular medical condition, with a prevalence of less than 0.5% in the general population . The sym2 and a distended, diffusely painful abdomen  of 18.1 kg/m abdomen . Her weiImmediate exploration of the abdomen was performed in the Emergency Department using X-rays, ultrasound, and a computed tomography (CT) scan of the abdomen. The abdominal ultrasound revealed an enlarged stomach , and the2.The abdominal X-ray showed significant gastric and duodenal distension and a nasogastric (NG) tube was inserted to prevent aspiration. A total of 4000 mL of fluid were drained after insertion of the NG tube. An upper endoscopy was performed and showed no associated or obscure lesions in the upper GI tract . The surSMAS is a rare entity and can affect both genders and all age groups . Even thAs previously mentioned, the third portion of the duodenum is positioned at the angle formed by the aorta and the superior mesenteric artery. A narrow-angle would enable the obstruction of the third segment by the arterial vessels due to a loss of retroperitoneal and mesenteric fat deposits which are surrounding the vascular structures ,14. The The loss of body fat is associated with a higher risk of developing SMAS . That isCurrently, the exact prevalence of vascular compression syndromes is unknown but is generally considered very rare, still poorly understood, and difficult to diagnose. Noninvasive tests such as standard ultrasound examination of the duodenum are insufficient for a positive diagnosis, but Doppler ultrasound offers detailed information concerning the venous and arterial flow, aorto-mesenteric angle, and distance enabling a quick and accurate diagnosis. Ultrasound is a sensitive method, and it has been shown to have a good correlation with CT .Our patient had a history of malignancy with unremarkable but progressive weight loss and mild digestive symptoms before the diagnosis. A correct and early diagnosis would have been difficult to state given the patient’s discrete and unspecific symptomatology which could easily overlap other more common medical conditions. The patient had signs of partial intestinal obstruction such as intermittent nonvolitional vomiting, postprandial epigastric pain, and early satiety shortly after food intake which was confused with other anatomical or motility-related disorders. The acute onset of epigastric pain, plenitude, vomiting, and abdominal distension enabled physicians to adequately explore the digestive tract and diagnose intestinal obstruction secondary to SMAS. Additionally, the patient presented with asymptomatic mild hematuria which was a valuable argument for the diagnosis of left vein compression, in the absence of other causes such as masses, infection, and kidney stones.The majority of the cases with this vascular-anatomic syndrome are treated conservatively by medical management  ,10. ThisVascular compression syndromes are rare medical conditions. However, a clinical diagnosis requires a high index of suspicion and should be kept in mind in cases where patients have recurrent symptoms of vomiting, epigastric pain, postprandial abdominal distension, and recent history of weight loss. The diagnosis is confirmed by a CT scan. Unfortunately, there are no standardized treatment protocols, but conservative medical treatment is the first-line choice. Further studies are necessary to identify characteristics of patients with SMAS  that fail conservative management and undergo complex surgical procedures. Based on our experience, we feel that it is important for physicians to remain alert to the possible association between SMAS and malignancy. Early recognition may allow appropriate management by interrupting the cycle of weight loss and secondary upper gut obstruction from SMAS.


Biting fibroma, an irritation fibroma or traumatic fibroma associated with a history of a prior lesion-related tooth bite or biting injury at the site, is a commonly acquired benign reactive lesion of the oral cavity. It is usually an asymptomatic, small, mucosa-colored, smooth, pedunculated or sessile papule. A biting fibroma is most commonly located on the buccal mucosa, followed by, in decreasing frequency, on the tongue or lip or hard palate or gingiva. It typically presents as a solitary lesion; however, it can appear as multiple lesions. Excision is the treatment of choice for a biting fibroma; however, the resolution of the lesion-associated chronic inflammation is also necessary to prevent a recurrence. The clinical history, lesion morphology, and pathology findings of an illustrative patient with a biting fibroma were included in this case report. An 80-year-old woman was described who developed a biting fibroma at the site of a tooth bite on her lower lip. An excisional biopsy not only confirmed the suspected diagnosis of a biting fibroma but also successfully treated her condition by removing the lesion; there was no recurrence. In conclusion, the diagnosis of a biting fibroma should be considered when a patient presents with a new intraoral lesion, particularly if associated with an acute injury or chronic inflammation of the site. Since the clinical differential diagnosis of a biting fibroma includes various other benign conditions and less common malignant neoplasms, a biopsy that removes the lesion may not only establish the diagnosis but also potentially provide adequate treatment. Reactive conditions can present as an oral mucosal lesion . An irriBiting fibroma is a common lesion of the oral mucosa ,6. AlthoA patient with a biting fibroma is described: an 80-year-old woman with an acquired papule on the mucosa of her lower lip. The location of the mucosal papule corresponded to a prior tooth bite site; the removal of the lesion, performed during the excisional biopsy, healed completely without recurrence of the oral lesion. The features of biting fibroma are summarized.An 80-year-old woman presented for the evaluation of an asymptomatic acquired lesion on her lower lip of two-year duration. Prior to the development of the new lesion, she recalled the nonintentional biting of her lip at this location. The site of the tooth bite, which resulted in an open wound, subsequently healed.The patient assisted in the cutaneous examination of her lower lip. She gently everted the lip with the index and middle fingers from both of her hands. There was a painless, smooth, firm, flesh-colored, 3 × 3 millimeter papule on the mucosal portion of the lower lip, just left of the midline Figure . An exciThe microscopic evaluation of the tissue specimen showed collagenous fibrous tissue with vessels in the lamina propria. The arrangement of the collagen fibers had a radiating pattern, and some of the vessels contained erythrocytes. The overlying stratified squamous epithelium was slightly acanthotic Figure .The correlation of the clinical history, lesion morphology, and pathology findings established a diagnosis of biting fibroma. The biopsy site completely healed by second intention. During a follow-up period of 17 months, there was no recurrence of the lesion.Biting fibroma is the most frequently acquired benign soft tissue lesion of the oral mucosa . It can Several terms have been used to describe a biting fibroma Table  1-20]. -20. 1-20The incidence of biting fibroma ranges from 1% (12 lesions per 1000 patients) to 15% (193 lesions per 1290 patients) ,8. InforThere was a female predilection in two large retrospective studies that only included patients with biting fibromas. In one study of 124 patients with 129 lesions), 77 were women, and 47 were men resulting in a women-to-men ratio of 1.6:1.0 . The sec lesions,Biting fibroma has been observed in children ,5,8,10. The duration of time that the biting fibroma was present prior to diagnosis was evaluated by one group of investigators; they had data from 112 of the 129 lesions in their study. The average duration was 19 months; the lesions had been present from one week to 13 years. However, the duration of the biting fibroma had been one year or less for nearly 75% 82 of 112) of the lesions [2 of 112 Although the definition of a biting fibroma implies that it was preceded by prior trauma or irritation to the affected site, researchers are not always able to elicit a history of the causative event. In one study of biting fibroma, over 90% (175 of 193) of the patients related a history of local trauma . SimilarA biting fibroma usually occurs as an isolated event. A mucocele is an intraoral lesion that results from trauma to the minor salivary glands. Albeit rare, a nine-year-old girl with coexisting biting fibromas and a mucocele on her lower lip has been described .Biting fibroma is typically asymptomatic. However, a study observed that 7.8% 10 of 129 lesions) were painful . In addi lesions The greatest diameter of a biting fibroma is usually less than 1 centimeter ,9. Yet, Similar to the patient in this report, the biting fibroma is usually a pedunculated or sessile growth with a smooth surface and mucosa-colored or yellowish-white papule. However, the surface can be hyperkeratotic or ulcerated ,9,17,20.Biting fibroma may appear on any location within the oral cavity. The biting fibroma site observed in six studies is summarized in Table Most individuals only have a solitary biting fibroma. However, albeit less frequently, patients with multiple biting fibromas have been described. In a retrospective study of 129 biting fibroma in 124 patients, multiple lesions were observed in three of the individuals. Two lesions were noted on either the tongue  of a 55-year-old woman or the hard palate of an 84-year-old woman. The third patient, a 79-year-old man, had three lesions on his buccal mucosa [Multiple biting fibromas were also noted to suddenly appear on the tongue of a middle-aged Chinese man one week after an excisional biopsy of a benign lesion on his dorsal tongue. The biopsy of one of the five new lesions showed a biting fibroma; the site healed without the development of a subsequent lesion. Four months after the second biopsy, the investigators noticed a tendency toward gradual spontaneous regression of the new lesions .Two concurrent biting fibromas were also reported on the lower lip of a nine-year-old girl who had a witnessed habit of lip biting; in addition, she also had a mucocele. All the lesions were excised; microscopic evaluation confirmed the diagnoses. After the surgical sites had healed, treatment was provided to ensure that recurrent injury to the location of the lesions did not occur; specifically, a lip bumper was placed on the woman’s lower lip for the management of the lip-biting habit .The clinical differential diagnosis of a biting fibroma is diverse. Other reactive conditions of the oral cavity can have a similar morphology; these include fibroma, inflammatory fibrous hyperplasia, inflammatory gingival hyperplasia, peripheral giant cell granuloma, peripheral ossifying fibroma, and pyogenic granuloma ,11,12,17The microscopic examination of a biting fibroma shows a connective tissue lesion in the mucosa that consists of dense collagen and a proliferation of mature fibroblasts. Chronic inflammatory cells (such as lymphocytes) may also be present; in addition, giant stellate cells may rarely be observed. The surface of the lesion can be hyperkeratotic or ulcerated or both secondary to the chronic irritation ,12,13,17Two patterns of collagen arrangement in biting fibroma have been described: circular and radiating. The collagen pattern is influenced by the location of the lesion and the degree of irritation that the lesion has received. The circular pattern has been more commonly observed; it was usually associated with a mobile cheek lesion that was not fixed to the underlying bone and therefore less traumatized. In contrast, the radiating pattern was less frequently noted; it tended to appear in a lesion affecting mucosa overlying the bone that was immobile and received more trauma ,12,13,17A group of researchers performed a light fluorescent microscope study of deparaffinized and unstained sections of 40 biting fibroma from either the buccal mucosa (27 lesions) or the lip (13 lesions) to determine the blue autofluorescence of the lesion’s collagen. They made two important observations. The intensity of the fluorescence increased with the age of the patient. Also, there was a positive correlation between the fluorescence intensity and the lesion duration .In a follow-up investigation, the research group performed a polarized microscope study of formalin-fixed and paraffin-embedded sections stained with picrosirius red of 43 biting fibromas to determine the characterization of the collagen fibers of these lesions. They observed that there was not only an increased number of yellowish-orange and orange fibers but also fewer blue-green and green fibers both in patients greater than age 30 years and in biting fibromas of longer duration. The changes that they noted in the older lesions correlated microscopically with tighter packing and better alignment of the microfibrils, similar to that observed in mature collagen .Based on their polarized microscope findings, the researchers concluded that younger biting fibroma primarily contained unpacked, poorly organized collagen whereas older lesions were composed of packed, well-organized collagen. Their observations also prompted them to recommend management based on the age of the biting fibroma and thereby its corresponding collagen characteristics. In a patient with an older lesion, not only stopping the chronic trauma but also surgical excision of the biting fibroma was necessary; however, in an individual with a younger biting fibroma, it was potentially possible for the lesion to spontaneously regress with only conservative treatment consisting of eliminating the chronic trauma .The pathogenesis of a biting fibroma involves trauma or chronic inflammation or both. The trauma can be acute and isolated, such as a tooth bite of the lip. Alternatively, the trauma can be recurrent, such as chronic biting of the buccal mucosa of the cheek. Other potential causes of mechanical injury include calculi, dental prosthetics, dentures, foreign bodies, overextended borders of an oral appliance, overhanging margins of a dental restoration, and sharp spicules of the bones ,9,11-13.One group of investigators postulated that female hormones might be a cofactor in the pathogenesis of biting fibroma. In their study, they noted that a biting fibroma predominantly occurred in women (70%). They also mentioned that other reactive lesions of the oral cavity, similar to a biting fibroma, most commonly appeared during the first five decades when hormonal changes were most prominent. Therefore, they hypothesized that, in the presence of chronic intraoral injury in a woman, female hormones stimulated fibroblasts resulting in increased collagen production and accumulation .A biting fibroma is a benign lesion; the malignant transformation of the lesion has not been described ,20. The psoriasin  and alpha-defensin  in the biting fibroma compared to the healthy gingiva. They also showed reduced gene expression of matrix metalloproteinase-3  and numerous inflammatory markers: interleukin 1 beta (IL-1β), interleukin 6 (IL-6), interleukin 8 (IL-8), tumor necrosis factor-alpha (TNF-α), and cyclooxygenase-2 (Cox-2). Finally, they noted that profound downregulation of deleted-in-oral-cancer-1  was missing. They concluded that the lack of malignant transformation of a biting fibroma may be attributable to the concurrent missing downregulation of the tumor suppressor gene DOC-1 and the overexpression of S100A7 [The researchers demonstrated significant gene expression elevation of The management of a biting fibroma has two key components: the excision of the lesion and the prevention of recurrence by eliminating the etiology of chronic inflammation. The excision is usually performed using a scalpel. After hemostasis has been achieved, the surgical wound is often allowed to heal by second intention ,3,4,8. EBiting fibroma may develop at the site of the patient’s denture. A 46-year-old woman developed a large, 3.5 × 2.5 × 1.0 centimeter, biting fibroma  that originated at the posterior border of her maxillary denture . In a stThere is a possibility of spontaneous regression of a biting fibroma in a patient who has a younger lesion if the source of irritation is removed; supporting evidence for this occurrence is based on fluorescence and polarized microscopy evaluation of the collagen arrangement in a biting fibroma. The collagen in an older lesion is more mature and organized; therefore, an older biting fibroma is unlikely to resolve spontaneously. However, in contrast to the older lesion, the collagen in a younger biting fibroma is poorly organized and may resolve without additional treatment if the source of irritation is eliminated. Hence, conservative management consisting of complete removal of the biting papilloma-associated irritation might be considered as an initial intervention if the lesion is newly acquired ,18.The recurrence of a biting fibroma is rare. However, several factors can contribute to provoking the recurrence of a biting fibroma. The lesion can persist and continue to grow if it has not been completely excised. Also, if the biting fibroma-initiating trauma persists or a new source of injury occurs at the site, the lesion can recur ,11,17.In a large study of 129 biting fibroma, there were no recurrences . A 1% reA 13-year-old boy presented with a recurrence of the same lesion (which was located on the papillary region adjacent to the upper incisors) that had been surgically excised less than one year earlier; the proposed causes for the recurrent biting fibroma were the incomplete excision of the initial lesion or the nonremoval of the irritant  or both. The recurrent lesion was completely excised; after the surgical site healed, the orthodontic management of the patient’s deep bite was successfully initiated by having the patient wear a removable anterior bite plane. After three months, the overbite decreased from 7.5 to 5.5 millimeters, and the intraoral appliance was discontinued; during the next nine months of follow-up, there was no recurrence of the biting fibroma .The excisional biopsy site of the woman in this report had excellent healing by second intention. There was no recurrence of her biting fibroma.An irritation fibroma or traumatic fibroma is a commonly acquired benign reactive lesion of the oral cavity. The lesion is referred to as a biting fibroma, incorporating the causative etiology of the lesion in its diagnosis, when there is an established history of a prior tooth bite or biting injury at the lesion site. Most biting fibromas (nearly 40%) are located on the buccal mucosa; other intraoral sites include the tongue (18.4%), lip (16.5%), hard palate (10.1%), and gingiva (10.0%). The lesion typically presents as an asymptomatic, small, mucosa-colored, smooth, pedunculated or sessile, solitary papule. The treatment of a biting fibroma usually requires not only the removal of the lesion but also the resolution of the associated chronic inflammation; when the lesion is appropriately managed, recurrence is rare. An illustrative case of an 80-year-old woman with a biting fibroma that occurred at a tooth bite site on her lower lip is described. Her suspected diagnosis was confirmed, and the lesion was successfully treated with an excisional biopsy; the site healed by second intention, and there was no recurrence. In summary, if the diagnosis of a biting fibroma is suspected, a biopsy that removes the lesion may not only establish the diagnosis but also adequately treat the lesion.


Purpose: To report a case of central retinal artery occlusion associated with sildenafil intake and briefly discuss its causative pathogenesis.Methods: A 50-year-old man with no premorbidities presented with symptoms of sudden severe visual field constriction in the left eye (LE). Best-corrected visual acuity in the LE was 20/25. Fundus examination and fluorescein angiography of the LE were suggestive of central retinal artery occlusion (CRAO) with cilioretinal artery sparing. Further investigation revealed that 100 mg of sildenafil had been taken for the first time three hours before the onset of symptoms.Results: The patient was treated promptly with intravenous acetazolamide, sublingual isosorbide dinitrate and ocular massage, but without visual recovery. No other associated systemic or local risk factors were found, and the case was classified as a potential complication of sildenafil.Conclusion: Although no direct link could be established, the aim of this report is to highlight the incidence and to consider this issue when evaluating any case of central retinal artery occlusion. We report herein a case of central retinal artery occlusion (CRAO), which occurred a few hours after oral sildenafil intake.Sildenafil is a specific phosphodiesterase V inhibitor which is a widely used treatment for erectile dysfunction. Many reports have highlighted ischemic ocular side effects associated with sildenafil.A 50-year-old Tunisian man, otherwise healthy and unemployed, presented to the ophthalmology department with sudden severe visual field constriction in the left eye (LE) of 48 hours duration preceded by severe headaches. On ophthalmic examination, visual acuity was 20/20 in the right (RE) eye and 20/25 in the left eye (LE). LE fundus examination revealed diffuse faint retinal whitening, except for central area of normal retinal color along the distribution of a perfused cilioretinal artery (Anterior segment examination of both eyes as well as fundus examination of theRE were unremarkable. LE fluorescein angiography (FA) showed no filling of the central retinal artery, regular filling of the cilioretinal artery and late retrograde filling of the central retinal vein  with cilioretinal artery sparing was diagnosed. Echocardiogram, carotid artery imaging and blood tests were unremarkable. Further investigation revealed that a 100 mg dose of sildenafil had been taken for the first time three hours before the onset of symptoms.Ocular massage was performed as well as sublingual isosorbide dinitrate and intravenous acetazolamide were administered. Two weeks later, reduction in retinal oedema was evident on left eye fundus examination, although there was no improvement in either visual acuity or visual field. acute macular neuroretinopathy, anterior ischemic optic neuropathy,, central retinal vein occlusion and cilioretinal artery occlusion.Various ischemic ocular events related to sildenafil have been reported. The most notable are branch retinal artery occlusion,,Only two cases of CRAO with sildenafil have been reported in the literature.The patient presented here reported taking sildenafil (100 mg) a few hours before the onset of ocular symptoms and headaches. In fact, ocular side effects are directly proportional to the blood concentration of the drug, which usually appears between 15 and 30 minutes after administration, reaches a peak one to two hours later and clears halfway in 3 to 5 hours. it may decrease cerebral blood flow leading to severe headaches as experienced by our patient. Similarly, numerous clinical studies have demonstrated that sildenafil induces retinal veinous vasodilatationin vivo.As sildenafil has a high systemic vasodilator effect that reduces systemic blood pressure,Both the absence of risk factors for retinal vascular occlusion and the timeline of events indicates that oral sildenafil was probably a contributing factor in the development of CRAO, but its pathogenesis remains speculative. We suggest that central retinal artery occlusion occurs in the region of the lamina cribrosa where the central retinal vein and artery share a common adventitia. We speculate that sildenafil-related vasodilation of the central retinal vein causes central retinal artery compression, resulting in secondary changes, including blood flow changes, endothelial damage and platelet thrombi, leading to CRAO. This case underlines the importance of a careful drug intake investigation in cases of CRAO without obvious cause. This incident should not be overlooked by physicians and must be seriously discussed with patients requiring sildenafil, especially since most of them are at risk for ocular ischemic events. Nevertheless, this association remains poorly explained and requires further documented cases.In summary, CRAO secondary to sildenafil is extremely rare and has only been reported in the literature twice previously. Clinicians should be aware of this risk and should avoid prescribing sildenafil in patients with a history of ischemic ocular events.All data underlying the results are available as part of the article and no additional source data are required.Written informed consent for publication of their clinical details and clinical images was obtained from the patient. The author reported an original case of a young adult patient with no personal history who presented with a central retinal artery occlusion following the intake of Sildenafil. The retinal imaging is interesting, moreover, it would be necessary to add the early times of the retinal angiography and a B mode OCT,  as well as it should be specified that the imaging was not done at an acute stage of the arterial occlusion seen the blood flow recovery exceeding the cilioretinal artery area. It may be relevant to detail the biological radiological and clinical explorations done to rule out other etiologies of retinal arterial occlusion like cardiovascular pathologies, dysimmune diseases with vasculitis, and hematological disordersAre enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?YesIs the case presented with sufficient detail to be useful for other practitioners?YesIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?YesIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:Ophtalmology Ocular Surface Disease , glaucoma, retinal diseases, anterior segment surgery, contactologyI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The authors report a case of central retinal artery occlusion as a potential complication of Sildenafil. they conclude that Clinicians should take into account this complication while prescribing this treatment.  In this case, multimodal imaging was well detailed with high quality images. It would be also interesting if the authors could add structural OCT imaging of the posterior pole. On LE fluorescein angiography, are there more earlier phases that could better describe the retrograde filling of the posterior pole vessels? Did the general examination of the patient reveal some characteristics that could be possible markers predicting this potential complication when prescribing this treatment Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?YesIs the case presented with sufficient detail to be useful for other practitioners?YesIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?YesIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:retinal imaging, peridatric ophthalmology, cataract surgery, oculogeneticsI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


Choledochal cysts are rare congenital cystic dilatations of the biliary tree. They are most commonly present in female infants and young children, and their pathology remains unclear. The triad of intermittent jaundice, abdominal mass, and pain is found only in a minority of patients. Diagnosis and delineation of accurate biliary anatomy are crucial for surgical planning. This is most often successfully achieved with ultrasound and magnetic resonance cholangiopancreatography. The definitive treatment is cyst excision which decreases the risk of biliary carcinoma. We present an unusual case of a choledochal cyst in an adolescent boy with a review of the literature and emphasis on multi-imaging modalities, including the role of liver-specific gadolinium contrast agents in challenging cases to confirm the diagnosis. Choledochal cysts (CC) are rare congenital cystic dilations of the biliary tract ,2. They The triad of intermittent jaundice, abdominal mass, and pain is found only in a minority of patients ,2,4. AduCholedochal cysts are predominantly benign but can be associated with serious complications, including malignant transformation, recurrent cholangitis, pancreatitis, and cholelithiasis ,2.We present an interesting clinical scenario of a choledochal cyst in an adolescent boy with a review of the literature. We discuss multi-imaging modalities and highlight the role of liver-specific gadolinium contrast agents.A 16-year-old male with no significant past medical history presented to the emergency room for recurrent episodes of epigastric pain for three months radiating to the interscapular region with nausea and vomiting. The patient was not jaundiced, and the physical exam was unremarkable. Pertinent laboratory tests demonstrated elevated gamma-glutamyl transferase (657 U/L), alkaline phosphatase (493 U/L), alanine aminotransferase (233 U/L), and aspartate aminotransferase (79 U/L). Total and direct bilirubin were normal , as well as lipase (50 U/L). Subsequently, an ultrasound was performed and showed cystic dilatation of intrahepatic bile ducts with normal gall bladder and pancreatic duct Figures , 1B.Further work-up with magnetic resonance cholangiopancreatography (MRCP) was performed to exclude the possibility of intrinsic (such as a stone) versus extrinsic obstruction. MRCP revealed, in addition to dilated intra- and extrahepatic hepatic ducts, a 5.0 x 4.0 cm round lesion in the topography of the common bile duct (CBD) in the vicinity of the duodenum, medial to the pancreatic head. The lesion showed an increased signal on T1 and T2 -weighted images and a thick low-intensity rim on T2-weighted images Figures -2C.An abdominal radiograph Figure  showed nThe overall appearance favored the accumulation of mucin and proteinaceous material in a pre-existing CC, type IVA. However, the possibility of an extrinsic abnormality, such as duodenal diverticulum or old hematoma compressing the CBD, could not be completely excluded. Repeat imaging employing liver-specific hepatobiliary gadolinium contrast agent (Eovist) Figures -4D showeThe CC was successfully removed surgically, without complications.Choledochal cysts can be classified based on their imaging characteristics. The first imaging classification system was established in 1959 by Alonso-Lej et al. ,6. This Type V cysts must be differentiated from Caroli’s Syndrome, which occurs when type V CC are accompanied by congenital hepatic fibrosis .The most commonly encountered types are I and IVA ,6, and tSelecting the appropriate imaging test is always more challenging in the pediatric population. Multiple points need to be taken into consideration, starting with avoiding ionizing radiation as much as possible. However, the radiograph was performed in our case to help exclude more common etiologies, including kidney stones, bowel obstruction, and bowel dilatation. In addition, young pediatric patients or older pediatric patients with special needs may be unable to tolerate long exams, hold still or hold their breath, and may require sedation and anesthesia. However, our case was a 16-year-old patient who was cooperative with the imaging procedures. Furthermore, the smaller size of the anatomic structures in the pediatric population poses challenges in identification and imaging, which results in the need for higher resolution imaging .Ultrasound is usually performed as the first line of imaging. It is quick, non-invasive, non-radiating, and the least costly. However, it can be limited by the skill level of the users. It can demonstrate biliary ductal dilatation and can often help narrow the differential diagnosis, but it is least likely to provide a definite diagnosis, especially since visualization of the distal CBD may be limited by overlying bowel gas , as in oEndoscopic retrograde cholangiopancreatography (ERCP) was traditionally the gold standard for the diagnosis and accurate classification of CC and associated anomalies of the biliary tree ,8,9. HowContrast agents are not usually necessary for diagnosis. However, in certain equivocal cases such as ours, the use of liver-specific contrast agents can be of important diagnostic value . In liveGadolinium ethoxybenzyl dimeglumine  is the most commonly used FDA-approved liver-specific MRI contrast agent with up to 50% hepatobiliary excretion in the normal liver ,13. ThisA disadvantage of this technique is poor or even absent Gd-EOB-DTPA-enhancement in patients with advanced diffuse parenchymal disease or bile duct obstruction . AnotherTotal cyst excision is the definitive treatment of CC due to the risk of malignant transformation, which is most commonly associated with types I and IV cysts ,16. CompCholedochal cysts are rare and predominantly congenital in nature, but this case sheds light on the importance of keeping choledochal cysts among the differential diagnoses, even in older children. Diagnosis and delineation of accurate biliary anatomy are crucial for surgical planning. This is most often successfully achieved with ultrasound and MRCP. Liver-specific gadolinium-based contrast agents are reserved for the most challenging cases, especially in the pediatric population, where the anatomic structures may be much smaller than in adults. This technique is most likely to prove problem-solving, such as in our case, allowing prompt diagnosis and surgical resection.


Gastrointestinal stromal tumors (GISTs) account for 1% of GI neoplasms in adults, and epidemiological data suggest an even lower occurrence in pregnant women. The majority of GISTs are caused by KIT and PDGFRA mutations. This is not the case in women of childbearing age. Some GISTs do not have a KIT/PDGFRA mutation and are classified as wild-type (WT) GISTs. WT-GIST includes many molecular subtypes including SDH deficiencies. In this paper, we present the first case report of a metastatic SDH-deficient GIST in a 23-year-old pregnant patient and the challenges encountered given her concurrent pregnancy. Our patient underwent a surgical tumor resection of her gastric GIST as well as a lymphadenectomy a week after induction of labor at 37 + 1 weeks. She received imatinib, sunitinib as well as regorafenib afterward. These drugs were discontinued because of disease progression despite treatment or after side effects were reported. Hence, she is currently under treatment with ripretinib. Her last FDG-PET showed a stable disease. This case highlights the complexity of GI malignancy care during pregnancy, and the presentation and management particularities of metastatic WT-GISTs. This case also emphasizes the need for a multidisciplinary approach and better clinical guidelines for offering optimal management to women in this specific context. Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms originating from the interstitial cells of Cajal. GISTs are the most common soft tissue sarcoma of the GI tract but remain fairly uncommon, with an incidence of 10–15 cases per million per year. The median age of diagnosis is 64 years old [To date, there have been less than twenty reported cases of GISTs during pregnancy and there is currently no guideline for management in this specific context. We hereby present the first case of an SDH-deficient GIST in a pregnant patient and present our approach to this complex case.A 23-year-old female was referred to our GI clinic at 23 weeks of pregnancy for a palpable epigastric mass. She first noticed this mass a year ago after her first pregnancy. She was otherwise asymptomatic. Her personal as well as familial past medical histories were unremarkable, and her pregnancy was otherwise eventless. Her physical examination was normal except for the palpable epigastric mass. Laboratory work-up showed a hemoglobin level of 110 g/L (N 120–160) with an MCV of 66.5 (N 80–100). The patient had already been on optimal iron supplementation for 3 months. She was not taking any other medication.An abdominal unenhanced MRI showed a large well-defined submucosal polylobate antral gastric mass of the size 7.6 × 4 × 5 cm. The mass was solid, with cystic components. Lymph node involvement was suspected. These lymph nodes measured up to 1 cm on the short axis. These findings were consistent with a GIST.An EUS confirmed that the pre-pyloric solid mass originated from the muscularis mucosa. The mass had two mucosal ulcers on its surface. The endoscopic appearance was suggestive of a GIST, but a neuroendocrine tumor could not be excluded. Chromographin A was mildly elevated at 118 ug/L, and urinary 5-HIAA was normal. The patient had no symptoms suggestive of carcinoid syndrome.A multidisciplinary team (MDT) including surgeons, gastroenterologists, oncologists, radiologists, obstetrician-gynecologists (OBGYNs) and maternal–fetal medical (MFM) specialists discussed the case to offer the best medical options to the patient. After assessing the maternal/fetal surgical risk and the risk of tumor malignity , a decision was made to proceed to surgery after an induction of labor between 34 and 37 weeks to maximize fetal viability and to avoid bleeding complications. Performing a tumor biopsy was not considered given the potential risk of dissemination and hemorrhage. An abdominal ultrasound planned 3 weeks later showed no tumor growth. The patient was induced at 37 + 1 week. The baby was born with no immediate complications with an Apgar score of 9-9-10. She was discharged 2 days postpartum and was readmitted again a week later for her elective surgery. A preoperative abdominal CT with contrast was performed . The mas2. Immunohistochemical studies were highly positive and diffuse for CD117. Surgical margins were positive, and there was a lymphovascular invasion. Metastasis was found in 5 of the 11 excised lymph nodes, including 2 lymph nodes localized on the greater omentum. The tumor was classified as a pT3(m)N1 according to the AJCC Cancer Staging Manual 8th edition. Molecular studies were negative for KIT and PDGFRA mutations.Pathology was consistent with a multifocal, invasive, gastric and duodenal GIST with a predominant spindle cell component . The sizThree weeks later, a post-operative FDG-PET scan was performed and showed a hypermetabolic 9 mm lymph node at the aortic bifurcation with an SUV of 5.9 and a discretely hypermetabolic mass adjacent to the distal duodenum, with an SUV of 2.4 that was equivocal for metastasis. There were no other suspicious findings.A diagnosis of metastatic GIST was confirmed, and treatment was initiated with imatinib at a standard dose of 400 mg. The patient was also started on contraceptives. A genetic and molecular referral was requested to assess for wild-type (WT) GISTs. The genetic panel revealed a heterozygote mutation in the SDHC gene. Even though a WT-GIST was confirmed, imatinib was continued. In fact, her 4-month follow-up FDG-PET scan showed a stable disease while receiving imatinib.Considering the known association between SDH deficiencies and pheochromocytomas and paragangliomas (PPGLs), a neck and thorax MRI and a 68Ga DOTATE PET scan were performed and showed no evidence of PPGLs. Urinary and plasmatic catecholamines were normal.A 9-month follow-up FDG-TEP scan  after thA molecular diagnosis of GIST can be made based on immunohistochemistry studies. In fact, the expression of CD117 and DOG1 allows for the diagnosis of approximately 99% of GISTs . FurtherSome GISTs do not have a KIT/PDGFRA mutation and are consequently classified as WT-GISTs. WT-GIST includes many molecular subtypes including SDH deficiencies, SDHx mutations, NF1 mutations and BRAF mutations. They can appear sporadically or as part of a syndrome. A large study by the National Institutes of Health (NIH) GIST clinic including 95 patients with WT-GISTs showed that the majority of these tumors 88%) had an SDH-related anomaly  . Clinica8% had anSurgical excision remains the mainstay of treatment for localized WT-GISTs. Lymphadenectomy is often required because of tumor invasion. Quality of surgical resection (presence of negative margins) and surgery type  do not seem to impact the event-free survival (EFS) in these patients . More inAlthough imatinib is very effective at treating KIT/PDGFRA-mutant GISTs, SDH-deficient GISTs are largely resistant to TKIs due to the absence of gain-of-function tyrosine kinase mutations . The resAlthough the majority of GISTs appear to be sporadic, SDH-deficient GISTs can be syndromic in around 5% of cases . Carney–Patients with germline mutations of an SDHx gene are also susceptible to developing pheochromocytomas and paragangliomas (PPGLs). It is responsible for 30–40% of hereditary PPGL cases . StudiesMalignancy during gestation involves major management implications that should be anticipated by clinicians. First, the optimal timing of delivery should be discussed with a multidisciplinary team including surgeons, oncologists and MFMs. Delivery should be delayed to the 35th–37th weeks, when possible, to avoid premature and neonatal complications. In a systematic review of 12 cases of GISTs occurring during pregnancy, the median gestational week at delivery was 36 weeks. A total of 11/12 patients were delivered by C-section, including one case due to fetal distress . All theMoreover, using imatinib in advanced disease should be carefully assessed given experimental data showing teratogenic effects . Pye et To our knowledge, this is the first case report describing the management of a metastatic and refractory SDH-deficient GIST diagnosed during pregnancy. Considering the preponderant incidence of WT-GISTs in young women of childbearing age, a diagnosis of SDH-deficient GISTs should be considered in pregnant women presenting with a gastric mass, particularly when investigations support a diagnosis of GIST and do not identify KIT or PDGFRA mutations. This case report also highlights that, in the case of progression, doubling the dose of ripretinib is an interesting option to try. Finally, this paper emphasizes the importance of a multidisciplinary approach and the need for better clinical data and guidelines for offering optimal management of malignancy in this specific context.


Medulloblastoma is well known as the most common malignant brain tumor identified in children, frequently found at an intra-axial location in the posterior cranial fossa. Extra-axial medulloblastoma is uncommon and often misdiagnosed. We believe that a thorough understanding of atypical medulloblastoma cases is important in daily practice.−3 mm2/second was detected on magnetic resonance imaging. The initial diagnosis suggested schwannoma or hemangioblastoma. However, the postoperative histopathologic findings indicated medulloblastoma . Pre- and postoperative magnetic resonance imaging revealed no drop metastasis, but adjuvant radiation therapy was still required as a standard treatment therapyWe present the unique case of a 39-year-old woman of Asian descent who suffered from headaches and right-sided hearing impairment. A right extra-axial medulloblastoma with an extremely low apparent diffusion coefficient of 0.404 × 10Extra-axial medulloblastoma is an uncommon tumor that is often mistaken for other cerebellopontine angle neoplasms. We describe a rare example of extra-axial medulloblastoma, characterized by a low apparent diffusion coefficient. When evaluating an atypical cerebellopontine angle neoplasm, the apparent diffusion coefficient should be considered a relevant indicator. At the most basic histological level, the human brain consists primarily of two components: neuronal cells and glial cells. Glial cells are typically viewed as supportive cells able to undergo cell division to replenish themselves after injury. By contrast, neuronal cells are more stable and often do not divide after differentiation. Therefore, the vast majority of brain tumors have glial cell components; medulloblastoma is an unusual exception owing to its neuronal cell origins. Medulloblastoma is thought to derive from primitive neuronal cells and is expected to be found in intra-axial locations of the brain and spine . On extrClinical symptoms also vary on the basis of location, and patients often complain of headaches and persistent vomiting . Truncal9/L, red blood cell count of 5 × 109/L, hemoglobin of 12.8 g/dl, and hematocrit of 42%).A 39-year-old Asian woman presented to our hospital with symptoms including headache, dizziness, and right-sided hearing impairment, which she had experienced for some time but described as worsening starting 2 months prior to presentation. The patient did not adopt any specific medications until hospital admission. She was a multi-para woman . The family members did not have any specific diseases and did not suffer from the same symptoms. She lives in Hanoi and worked as an accountant with normal medical profile prior to this admission. On physical examination, the patient’s weight and height were 50 kg and 162 cm, respectively [body mass index (BMI) of 19]. Vital signs were within normal range, with body temperature of 37 °C, pulse rate of 80 beats per minute, blood pressure of 120/70 mmHg, and respiratory rate of 16 breaths per minute. Upon neurological examination, no neurological defects of sensorium, cognition, cranial nerves, motor, sensory, cerebellar, gait, reflexes, meningeal irritation, or long tract signs were observed. Blood test findings were normal in her report  sequence was ordered, which revealed the preservation of the right internal acoustic meatus with slightly anteriorly displaced nerves  and fluid-attenuated inversion recovery. A very low ADC of 0.404 × 10The patient underwent a retrosigmoidal suboccipital craniectomy for tumor resection. According to the operative report, this CPA tumor was located close to the dural matter and was soft with hypervascularity. The preoperative specimen confirmed a medulloblastoma case, and gross total excision was performed.The postoperative histopathological result revealed that this neoplasm was composed of multiple cells with carrot-shaped nuclei and scant cytoplasm. The tumor cells were arranged in sheets, rows, or nodules. Immunohistochemical stains were positive for oligodendrocyte transcription factor 2, synaptophysin, and INI1 but negative for glial fibrillary acidic protein, beta-catenin, and p53  . The mecThe clinical symptoms associated with medulloblastoma are often nonspecific, but some hallmarks can be used for locational diagnosis. In our case, long-lasting hearing impairments were significant indicators of a CPA lesion affecting cranial nerve VIII. CPA neoplasms represent approximately 10% of all intracranial tumors . Vestibuet al. reported a CPA medulloblastoma case with a “dural tail sign” and hyperostosis, which mimicked a posterior petrous meningioma [et al. [−3 mm2/second. In 2020, Mert Doğan et al. described a study of 56 pediatric patients with brain tumor. The mean ADC for hemangioblastoma was reported as 1.72 ± 0.25 × 10−3 mm2/second, which is significantly lower than the ADC for medulloblastoma (0.63 ± 0.22 × 10−3 mm2/second), and the cutoff value they suggested for differentiating between low- and high-grade pediatric brain tumors was 1.1 × 10−3 mm2/second [−3 mm2/second [The differentiation between CPA medulloblastoma and other CPA tumors can be extremely difficult , 11. In ningioma . Cystic  [et al. . Hemangi2/second . In a pr2/second . Our finTreatment options include observation, radiation therapy, and microsurgery. Observation was not considered in our case owing to the patient’s young age and cranial nerve function problems, which can typically be addressed through microsurgery. Several microsurgery approaches have been introduced , and theThe recurrence rate of adult medulloblastoma is roughly 50–60% . In contExtra-axial medulloblastoma is a rare entity that can easily be misdiagnosed as other CPA tumors. We report a rare case of extra-axial medulloblastoma characterized by low ADC. ADC should be carefully considered for all atypical CPA neoplasms.