The purpose of this study was to report on a surgical treatment for the cases of GCT . In the cervico-dorsal spine, the incidence is extremely low and has been reported very less in the literature.•Local recurrence is high when total resection is not achieved, especially when the tumor extends around the neural elements or adjacent vascular structures. In the upper cervical spine, the recurrence rate is slightly higher because complete excision is not always feasible.•Giant cell tumour of cervico-dorsal spine is a rare entity and should be managed Surgically with en bloc/extralesional resection but due to risk of surrounding neurovascular structures damage they are managed by marginal resection.•Also radiotherapy plays a pivotal role to prevent recurrence in cases where extralesional/en bloc excision is not feasible if used in controlled dosage. Giant cell tumor (GCT) of the spine is uncommon but most aggressive benign tumor of the spine with unpredictable outcome. The purpose of this study was to report on a surgical treatment for the cases of GCT . The spine is not a common site for a Benign GCT, with a 2.5% incidence in the sacrum and 2.9% in the vertebrae above the sacrum. In the cervico-thoracic spine, the incidence is extremely low and has been reported very less in the literature.This study was conducted on 3 cases of GCT of the spine and evaluated the outcome of different treatment modalities retrospectively. All the cases were treated with intralesional surgical resection but only one developed recurrence.Cord compression and neurological deficits of varying grades was observed in all the cases. All patients also presented with clinical as well as radiological instability. Overall results were satisfactory, as all patients were symptom-free postoperatively. One out of the three cases had tumour recurrence and needed repeat intervention.Giant cell tumour of cervico-thoracic spine is a rare entity and should be managed Surgically with en bloc/extralesional resection but due to risk of surrounding neurovascular structures damage they are managed by marginal resection therefore since total resection is not possible there are high chances of recurrences, Hence require close monitoring and follow up for early diagnosis and appropriate management. It was Giant cell tumor (GCT) of bone is a rare neoplasm that accounts for approximately 5% of all primary bone tumors in adults. GCT most frequently occurs at the end of long bones, and the sacrum is the fourth most common site, accounting for between 1.7–8.2% of cases. Giant cell tumor also occurs in the mobile spine, but this location accounts for only 2–4% of cases . In a reCommon symptoms of patients with spinal GCT include back pain, neurological deficit due to compression of spinal cord, bladder and bowel dysfunction, and structural deformity of the spine. Radiologically, spinal GCTs also present as expansile lytic lesion that most often involves the vertebral body  and soften bloc surgical excision is the treatment of choice in long bones as well as spine. The treatment of long bones GCT is curettage, sclerotherapy, and filling of the defect with bone cement [Various modalities of treatment used for spinal GCTs are surgery, radiotherapy, embolization, cryosurgery, cementation, and chemical adjuvant like phenol or liquid nitrogen. Total e cement . This ise cement .We are presenting a case series of THREE cases of GCT of cervico-thoracic spine and sharing our experience regarding their clinical outcome.2We retrospectively analyzed cases of spinal GCT that had been operated between 2015–2018. From the approximately 300 spinal surgeries done during this period 35 were reported to have tumor and only three were for GCT and formed our study. All the data was extracted from hospital records, including preoperative and sequential postoperative clinical findings, radiological details and pictures of the status at last follow-up. Patient was assessed by plain radiographs and CT-Scans for recurrence of tumour, instability and the status of fusion and spinal implants. There were one male and two females in our group of three patients. Age ranged from 22 to 30.All the Three patients Presented as fresh cases and only one case had a recurrence after their first surgery with us. Two patients presented with cord compression and neurological deficit while one just had associated pain symptoms. All patients had clinical as well as radiological evidence of spinal instability judged on the basis of factors such as Mechanical pain, Location of lesion, Bone lesion type, Radiographic spinal alignment, Vertebral body collapse, Postero-lateral involvement used for calculating Spinal Instability Neoplastic score (SINS) . All casOne of our pt had GCT in cervical spine (C2 level), One in Thoracic spine (T4 level) and third in cervico-thoracic junction (C7-T1 level) . All theIn our first case study of 24-year-old male who presented to author institute Hospital OPD with the complaint of pain in the neck of 2 months duration. Onset was insidious and the pain was worsening. Pain was located in the mid cervical-spine radiating bilaterally to the upper limbs. Neck movements were grossly restricted and painful along with difficulty in daily activity. NO history of loss of appetite/weight with NO history of fever. No neurological deficit was present in the upper and lower limbs. Marked tenderness was present in the mid cervical region. Patient was advised plain radiograph cervical spine. Plain radiograph showed destruction C2 vertebrae body. Patient was advised MRI  and corrIn one of the case of 24 year old female with complete paraplegia from one month with complains of pain in neck region from last three months. Power in the lower limbs was grade – 0/5 and in the upper limbs was grade – 4/5 with Plantor-Extensor. Patient was advised Plain radiograph of the cervico-thoracic spine which showed compression at C7-T1 with decrease in height of C7 and T1. Patient MRI showed “Areas of altered signal intensity involving C7 and T1 vertebrae and C7-T1 intervertebral disc involvement with Anterior wedging, Focal Kyphosis at C7-T1 level with epidural mass causing thecal sac indentation and cord compression at these levels with cord edema at C6 level with pre and paravertebral abscess at C7-T1 level”, compressing/encasing respective exiting Nerve roots . PatientIn other case of 30 year old female presented in OPD with complete paraplegic from one month with Bladder Bowel involvement along with sensory deficit below T4-T5 with Planter extensor reflex. History of loss of appetite present but NO history of fever/loss of weight.ESR levels were 45 mm/hr. Patient was advised Plain radiograph of the thoracic spine which showed collapse of T4 with decrease in height of the same. Patient on MRI showed Compressive Myelopathy with collapse at T4. MRI  reportin3In Our 4 interventions we achieved goals of spinal decompression and stabilization. All three returned to neurologically stable state with adequate bladder bowel control at the last follow up. The patient with recurrence showed signs of improvement of power upto grade 4/5 with bladder bowel continence.We had no major complications, though one patient in a long term follow-up presented with recurrence after 6–8 months of normal interval. Posterior decompression was planned in view of MRI showing involvement of posterior spinal elements which underwent successfully and is free from symptoms on further follow-up till date.Out of the three cases two underwent postoperative radiotherapy for which doses varied depending on type of lesion and previos radiotherapy.All the three patients on subsequent follow ups after getting plain radiographs and MRI done postoperatively at long term follow-up showed Good alignment and no recurrence of GCT . Overall4The spine is not a common site for a Benign GCT , with a Extensive previous studies in the literature show paucity of data regarding incidence and management of GCT cervico-thoracic spine. GCT of sacrum and lumbar spine has been well reported ,29. A stThe gross appearance of a GCTB is described as having a vascular, friable tissue with a surface that is reddish brown, yellowish brown or yellowish gray . DiffereSpinal GCTs are usually expansile lesion with bone destruction that affects the vertebral body and can cross disk spaces and extend into the posterior elements, but involvement in the posterior elements was observed with other spinal bone tumors, as aneurismal bone cyst and osteoid osteoma or osteoblastoma . PosteriLocal recurrence is high when total resection is not achieved, especially when the tumor extends around the neural elements or adjacent vascular structures. The overall recurrence rate in the spine is between 25 and 45% . In the In the spine, the initial surgery of GCT should be as aggressive as possible within neurological preservation and spinal stability. In case of partial removing of tumors, the radiation therapy is a useful added treatment with less risk of recurrence even though it gives a chance of malignant transformation . Its higSimilar to our inference extensive study of the literature showed Giant cell tumors of the spine including sacrum should be managed with en bloc resections whenever possible as this provides the greatest chance for cure but When the risk of post-operative neurologic deficit after en bloc excision is high they should undergo intra lesional marginal resection followed by controlled local radiotherapy ,37.The recommended follow up for spinal GCTs in view of high recurrence rate is three monthly for initial two years followed by six monthly for three years and then yearly for 5 years making it completely around 10 years along with CT Scans and MRI at corresponding follow ups. The previous reported studies in the past had a mean follow up of 5 years . SimilarUsually, some microscopic tumor tissue is expected to stay behind, therefore postoperative radiotherapy is recommended. Though earlier literature seemed to suggest that irradiation converts benign GCTs to malignant ones, this is no longer true with modern radiotherapy techniques, especially by keeping the total radiation dose under 50 Gy .This Res5Giant Cell Tumour of the cervico-thoracic spine is a rare entity which has not been previously extensively reported. Early diagnosis and proper surgical excision using extralesional/en bloc resection is required but due to the fear of injury to the extremely close neurovascular structures Marginal resection is done which leads to higher chances of recurrence as the total resection is not feasible. A close follow-up and monitoring for signs and symptoms with periodic imaging are excellent tools to identify the recurrent lesion and plan out the necessary treatment. Also radiotherapy plays a pivotal role to prevent recurrence in cases where extralesional/en bloc excision is not feasible if used in controlled dosage.The authors declare that they have no conflict of interest.There was no sources of funding for our research.Since we are not reporting the first case of this type in the literature, hence no approval was taken from the relevant ethics committee but written informed consent was taken from the patient to publish his details and X-ray photographs.Written informed consent was obtained from the patients for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.SG and JS were involved in Conceptualization and Data curation. JS, SG and KR did Formal analysis and Investigation. SG, RB and JS formulated Methodology. JS and KC did project administration. SG and KR developed Resources and Software. RB and JS Supervised. SG, JS and KR Validated and Visualized. SG involved in Writing - original draft. JS helped in Writing - review & editing.NA.Dr. Sorabh Garg.Not commissioned, externally peer-reviewed.


Eosinophilic colitis is an inflammatory condition in which the wall of the colon becomes infiltrated by eosinophils which stain densely pink on microscopy. It is an uncommon clinical entity which has a long list of differential diagnoses. We present a case of a patient whose biopsy at colonoscopy revealed eosinophilic colitis which led to a delay in the diagnosis and subsequent treatment of colon cancer. A 35-year-old male presented with a six-week history of right lower quadrant abdominal pain associated with diarrhoea and weight loss. Colonoscopy showed an inflamed cecum; computed tomography revealed a small ascitic fluid collection in the right iliac fossa. Biopsy showed eosinophilic colitis, and he was treated conservatively with albendazole and mesalamine. The patient failed to improve over the following month with continued weight loss. A repeat CT scan showed a right iliac fossa mass. A right hemicolectomy was performed with histopathology from the specimen showing mucinous adenocarcinoma. Eosinophilic colitis can mask colon cancer and should be considered a diagnosis of exclusion. The term red herring originates from William Cobbett who described the use of a smoked red herring to distract hunting dogs from their pursuit of a rabbit . The phrWorldwide colon cancer is the third most common cancer, accounting for the 2nd greatest number of cancer related deaths . It is tThe diagnostic algorithm for colon cancer incorporates history, examination, and imaging studies. Treatment is embarked upon after histological confirmation from a specimen obtained via endoscopy , 6. RegrEosinophilic colitis is an inflammatory condition in which the wall of the colon becomes infiltrated by eosinophils which stain densely pink on microscopy. This case is that of a patient whose biopsy from colonoscopy revealed eosinophilic colitis which led to a delay in the diagnosis and treatment of colon cancer. Like a red herring, eosinophilic colitis was a distracting diagnosis which masked a more sinister pathology.This report highlights potential pitfalls in diagnosing a patient with eosinophilic colitis.Mr. B.C. is a 35-year-old male patient who presented with a six-month history of right lower quadrant pain and weight loss of 20 pounds. He also complained of constipation with a decrease in bowel motion frequency from once per day to two per week.On examination, B.C. was noted to be cachexic. Palpation of the abdomen revealed tenderness in the right iliac fossa; however, no signs of peritonitis were elicited. Complete blood count and renal and liver function tests were normal.Computed tomography (CT) of the abdomen and pelvis with intravenous contrast showed a thick-walled (6.3 mm) caecum with fat stranding and a small volume of adjacent free fluid see . These fThe patient subsequently had a colonoscopy which showed a mass at the caecal pole with edematous, inflamed tissue and pus see . Random Considering the CT and histology findings, the patient's age, and the absence of a family history of malignancy, the findings at colonoscopy were interpreted as an inflammatory mass secondary to eosinophilic colitis. Stool microscopy showed no ova, cysts, and parasites. In conjunction with a gastroenterologist, the patient was started on an empiric course of albendazole and mesalamine.One month after his first presentation, Mr. B.C. represented reporting no improvement in symptoms, further weight loss of 10 pounds, and intractable abdominal pain. At this time, the patient was tender in the right lower quadrant of the abdomen with guarding. Repeat CT scan showed a fluid collection surrounding the caecum suggestive of an intra-abdominal abscess. Taking into account the new CT findings and continued clinical deterioration despite medical therapy, the decision was made to perform a laparotomy. Intraoperatively, a large caecal mass was found with an associated abscess and multiple enlarged mesenteric lymph nodes see . A rightHistology of the surgical specimen revealed a moderately differentiated mucinous adenocarcinoma, pT3 pN1b. Of note, the tumour involved a relatively small area of the mucosa with the bulk of the tumour extending in the other layers of the bowel wall. The majority of the tumour was observed to be deep to mucosa, which showed no evidence of malignancy, and dense eosinophilic infiltrate of more than 40 per high powered field . This caEosinophilic gastrointestinal disease (EGID) is a rare condition of unknown aetiology which is characterized by eosinophil-rich chronic inflammation of the gastrointestinal tract, in the absence of any known cause of eosinophilia. Initially described by Kaijser in 1937 , it can When EGID affects the colon, it is termed as eosinophilic colitis (EC). EC is the rarest form of EGID, with a prevalence of 2·3 per 100000 in adults . Cases hThe diagnosis of EC is made when a patient satisfies the following three criteria: (1) the presence of gastrointestinal symptoms, (2) histologically confirmed eosinophilic infiltration of the colon, and (3) other causes of colonic eosinophilia which have been excluded .EC typically presents with vague abdominal complaints such as abdominal pain, nausea, vomiting, diarrhoea, constipation, and weight loss. Less frequently, patients may present with obstruction, perforation, intussusception, volvulus, and ascites .Histological evaluation for EC is done via biopsy, typically obtained through endoscopy. There is no consensus in the literature regarding the level of eosinophils considered to be diagnostic; however, most authors used a threshold of 20 eosinophils per high powered field (h.p.f.) . Using aFew published cases of EC include quantification of eosinophilic infiltrates. Bates' review highlighted that out of 34 cases published in the English literature since 1959, just 7 cases included measurements of eosinophil density. Of these 7 cases, only 5 were genuinely idiopathic with the remaining cases due to a secondary cause. The author went on to question whether EC was an actual nosological entity and concluded that it should be regarded as a nonspecific reaction pattern . With thNumerous secondary causes of eosinophilic infiltration of the bowel wall have been identified in the literature. These include drugs , parasitic infestation, scleredema, polymyositis, vasculitis, inflammatory bowel disease, radiotherapy, lymphomas, and carcinomas .Eosinophils have a role in the host's reaction to tumours and are thought to have tumouricidal effects. In mice with tumour eosinophilia, there are decreased tumour progression and tumourigenicity. As it relates to colon cancer in humans, tumour eosinophilia is associated with an improved prognosis , 19. SpeColon cancers can contain large numbers of eosinophils, and they are often observed in tumour specimens. Harbaum et al.'s histological review of colon cancer specimens revealed intratumoural eosinophils in 86% of specimens and peritumoural eosinophils in 75% of specimens . Given tThis case highlights the importance of a multidisciplinary approach to the management of patients. With improved communication between surgery, gastroenterology, and pathology, perhaps, this patient may have had a repeat biopsy following his colonoscopy. This could have led to an earlier diagnosis of colon cancer and avoided a delay in surgery. Earlier surgery would have afforded the surgeons the opportunity to operate on a patient with better nutritional status and thus reduced the risk of postoperative complications . More imAs a consequence of the nonspecific nature of the presentation of EC, there have been many reports of it being confused preoperatively with other diagnoses including inflammatory bowel disease, acute appendicitis, and small bowel obstruction . It is, Eosinophilic colitis is an uncommon clinical condition whose diagnostic criteria are not elucidated in the literature, and it may not be a true nosological entity. Increased numbers of eosinophils can be observed in the colon in a number of disease conditions, including colon cancer. Given the potential for adverse outcomes because of a misdiagnosis, an extensive search for secondary causes of eosinophilia should be undertaken employing a multidisciplinary approach before a patient is diagnosed with eosinophilic colitis.


To investigate the imaging features of hemangiomas in long tabular bones for better diagnosis.Twenty-four patients with long bone hemangiomas confirmed by pathology were enrolled. Nineteen patients had plain radiography, fourteen patients had computed tomography (CT) and eleven had magnetic resonance imaging (MRI). The hemangioma was divided into medullary , periostAmong 19 patients with plain radiography, eleven patients were medullary, three periosteal, and five intracortical. In the medullary type, the lesion was primarily osteolytic, including five cases with irregular and unclear rims and one lesion having osteosclerotic and unclear rims. In three patients with the periosteal type, the lesion had clear rims with involvement of the cortical bone in the form of bone defect, including two cases with local thickened bone periosteum and one case having expansile periosteum. Five intracortical hemangiomas had intracortical osteolytic lesions with clear margins. Among 14 patients with CT imaging, 8 cases were medullary, three periosteal, and three intracortical. Among 8 medullary hemangiomas, one had ground glass opacity, and seven had osteolytic, expansile lesions like soft tissue density with no calcification. In three periosteal cases, the lesion was osteolytic with thickened periosteum and narrowed medullary cavity. In three intracortical hemangiomas, the lesion was of even soft tissue density with no calcification. Among 11 patients with MRI imaging, seven were medullary, two periosteal, and two intracortical. Among 7 medullary lesions, six were of hypointense signal on T1WI and hyperintensesignal on T2 WI. In two periosteal cases, the periosteum was thickened, with one case being of equal signal, and the other having no signal. Two intracortical hemangiomas were both of slightly low signal on T1WI but hyperintense signal on T2WI.The long bone hemangiomas had characteristic cystic honeycomb-like presentations in plain radiograph. CT and MRI imagings are helpful for diagnosis of hemangiomas in long bone. Osseous hemangioma accounts for 1% of all primary skeletal neoplasms with about 75% in the vertebra or skull and 15%-20% in the scapula, ribs, clavicle and pelvic bones . OsseousThis study was approved by our hospital ethics committee with all the patients given their signed informed consent. Between 2000 and 2019, twenty-four patients with hemangiomas in the long tubular bones were confirmed by histopathology including 13 males and 11 females with an age range of 11–67 years (median 35). The presentation symptoms and signs were pain in 20 cases and local masses in six patients with a history of one month to 10 years. Plain radiography was performed in 19 cases, CT in 14, and MRI in 11.All the images were analyzed by two senior imaging physicians with 15 years of experience, and when in disagreement, a third radiologist with 20 years of experience was involved to reach a consensus. The lesions were divided into medullary (the lesion was located in the medulla or at the cancellous bone), periosteal (the lesion was located at the surface of bone), and intracortical (the lesion was inside the cortex). The long bone hemangioma was at the femur in 9 patients, tibia in 7, fibula in four, humerus in 3, and radius in the remaining one, including medullary hemangioma in 13 cases (54.2%), periosteal in 6 (25%) and intracortical in the remaining 5 (20.8%).Nineteen patients had plain radiography with 11 patients presenting as medullary, three as periosteal and the rest five as intracortical hemangiomas. In medullary hemangioma, the lesion was lytic accompanied with coarse trabecula, with a beehive-like appearance in five patients, a sclerotic rim in three patients Fig. a. One caFourteen patients had CT scanning with a medullary type in eight patients, periosteal in three, and intracortical in the remaining three. Among eight patients with medullary hemangioma, a ground-glass appearance was in one while a lytic expansile lesion without apparent calcification was demonstrated in the other seven cases including four cases having a sclerotic rim with varying thickness, one with incomplete sclerotic rim, and two without sclerotic rim. Among eight medullary lesions, four cases had bone septa inside the lesion like a beehive Fig. a, one haEleven patients had MRI scan including seven cases of the medullary type, two of the periosteal type, and two of the intracortical type. Among the seven cases with the medullary type, four cases had well-defined lesions of hypointense signal on T1WI but hyperintense signal on T2WI compared with normal cancellous bone, of which two cases had honeycomb-like septations. In one case, the lesion broke through the bone cortex and had uneven T1WI slight hypointense signal and high signal on T2WI, with the adjacent medullary cavity being of hypointense signal on in T1WI and hyperintense signal on T2WI compared with normal medullary cavity. In one case, the lesion was oval at the medulla of the neck of femur, which had slight hypointense signal on T1WI but hyperintense signal on T2WI, with honeycomb-like septations. The abnormal signal extended downwards to the upper and middle segment of the femur, with clear margins and uneven thinned cortex at the internal side Fig. . In one Hemangioma is a rare bone lesion with a preponderance in females, with most patients being young adults and female patients younger than their male counterparts . ContrarThe lower extremities are the predominant location for skeletal hemangioma (73%), with the tibia and femur being the commonest site, similar to our case series. It has been reported that most hemangiomas were meduallary (55%) followed by periosteal (33%) and intracortical 12%) [% [2]. ThRadiological presentations of hemangiomas in the long bones are highly variable and non-specific and are difficult for a correct diagnosis  because CT appearance of hemangioma in long bones correspond to that on radiographs as a lytic lesion with internal trabecula , and in − 3mm2/s vs. 1.54 ± 0.03 × 10− 3mm2/s). Diffusion-weighted echo-planar MRI is a promising non-invasive modality which may be helpful for differentiating malignant from benign soft tissue tumors and for grading malignancy.Intramedullary hemangiomas need to be differentiated from other chondrogenic or dilative dissolved tumors, such as giant cell tumor, aneurysmal bone cyst, or fibrous dysplasia. In these cases, reticular or honeycomb lesions can also be seen, with thick internal trabecula. Sometimes, it is difficult to make a correct diagnosis only by X-ray radiography and CT. Intra-hemangioma cyst is easy to be misdiagnosed as bone cyst or fibrous dysplasia, which needs MRI to identify. In periosteal hemangioma, thickening of cortical bone indicates that the lesion has involved the periosteum. With enrichment of periosteal blood supply, osteoblasts become more active, leading to periosteal hyperplasia to gradually manifest as cortical bone thickening. This type of hemangioma needs to be distinguished from osseous structural changes, because soft tissue hemangioma can also readily invade adjacent bones, resulting in cortical thickening. MRI is helpful to determine the location and scope of the lesion, which is conducive to differential diagnosis. Cortical hemangioma is easy to be confused with osteoid osteoma. MRI is superior to X-ray and CT in determining the location, boundarys and extent of lesions , 17. WheIn our study, the plain radiography, CT, and MRI images of hemangioma in long tubular bones were investigated. The advantages of plain radiography are convenient and fast, with low medical cost but high spatial resolution, which can be used to evaluate the location and scope of lesions, but the disadvantage is its inability to clearly show the lesion details and density differences. Thus, plain radiography should be used as the first level examination method, which is suitable for small lesions with slight bone changes and for follow-up of these patients. CT provides the clearest description of cortical destruction and is an excellent imaging method for characterizing tumor matrix, exact location, expansion, and bone changes. However, the display of soft tissue on CT imaging is weaker than that of MRI, and it can be used as a secondary examination method for the diagnosis of large lesions and severe bone destruction. MRI can be used to observe periosteal reaction, bone marrow edema, and soft tissue edema. Moreover, MRI can be used to quantitatively evaluate the composition of lesions and the characteristics of blood flow signals, but it is expensive and may serve as the third level examination method for diagnosis. Understanding the advantages and disadvantages of each imaging technique is helpful to establish the correct imaging examination procedure for a correct diagnosis.For correct diagnosis of hemangioma in long tubular bones, plain radiography should be performed firstly when local symptoms in the extremities appear, and small benign lesions can be diagnosed and followed up for close monitoring. For larger expansile lesions with unclear margins, CT scanning should be conducted for differentiation and for further decision of follow-up observation or biopsy. If CT imaging cannot determine the malignancy or benign nature of a lesion, or when surgery is needed, MRI scanning should be performed to determine the nature of the lesion and to provide detailed information of the lesion.In conclusion, long bone hemangiomas are rare and may, although not-life threatening, pose a challenging task in diagnosis because of variable radiological appearances, and classical radiological presentations including coarse trabecular bone pattern or soap-bubble appearances may suggest this disease.


Ampullary adenocarcinoma is a malignant tumor that arises from the ampullary complex, distal to the confluence of common bile duct and pancreatic duct. It is a rare tumor and pathologically differentiated into intestinal or pancreaticobiliary in origin. Management is surgical resection. We report a case of a 67-year-old male who presented with abdominal pain, vomiting, and constipation. Computed tomography scan showed a cystic mass compressing the duodenum and causing small intestinal obstruction. Pathologic evaluation was consistent with ampullary adenocarcinoma. Ampullary adenocarcinoma is a rare malignant tumor that constituting only 0.2% of all gastrointestinal tumors . These tA 67-year-old male patient with a history of tobacco use and alcohol dependence presented with two weeks of abdominal pain, distention, vomiting, and a 30-pound weight loss over the course of three months. On presentation, he was afebrile with normal blood pressure and heart rate. On exam, he appeared cachectic, with left upper quadrant abdominal tenderness on palpation. 3/μl), potassium 2.5 , bicarbonate of 43 , anion gap of 20 (reference range 5 - 15 mmol/L), creatinine 2.1 , calcium 12.4  and lipase of 849 . Cancer antigen 19-9 (CA 19-9) was normal. Liver enzymes were within normal range. Given his acute kidney injury, computed tomography was ordered only with oral contrast. The study revealed massive gastric distention concerning for outlet obstruction  revealed large irregular hyperattenuating area within the left lobe of the liver that may represent hepatic ischemia. Subsequently, the patient underwent mesenteric angiogram showing hepatic artery thrombosis and subsequently, thrombolysis was performed. Afterwards, the patient had an uncomplicated hospital course and was discharged with close surgical and oncology follow up for chemotherapy evaluation. The ampulla of Vater is the point of confluence where the common bile duct and pancreatic duct drain prior to emptying into the duodenum. In the majority of individuals, it serves as the single conduit to the duodenum, and in roughly 42% of individuals, the ampulla drains the common bile duct separate from the pancreatic duct. The area surrounding the ampulla is known as the periampullary region, which is defined as the area within two centimeters of the ampulla of Vater. This is an important anatomical landmark as multiple structures  exist within this complex anatomic area, all of which can harbor distinct pathology. In terms of malignancy, periampullary cancers account for 5% of all gastrointestinal cancers, however, ampullary cancer itself is a rare entity. Ampullary cancer makes up only 0.2% of all gastrointestinal tumors Ampullary adenocarcinoma may have early clinical findings that are non-specific such as abdominal pain, lethargy, and weight loss. The early biliary obstruction that clinically manifests as jaundice can prompt a more detailed workup that can result in earlier diagnosis and treatment with surgery and chemotherapy .It has been shown in prior literature that ampullary cancer, as well as other malignancies originating from the periampullary region, can be considered when cross-sectional imaging shows a “double duct sign” in the setting of mass lesion. This finding denotes simultaneous dilatation of the common bile duct and pancreatic duct and has been found in 52% of patients with ampullary adenocarcinoma, and 62% of patients with pancreatic adenocarcinoma [The majority of ampullary cancers are adenocarcinomas, with a smaller diversity of tumors being papillary, adenosquamous or mucinous . FurtherAmpullary adenocarcinoma is a rare tumor that most commonly present with jaundice and is detected on radiologic imaging. Initial evaluation requires endoscopic ultrasound with fine needle biopsy. The tumor can be divided into two subtypes, intestinal or pancreaticobiliary based on immunohistochemical staining. Surgery is the mainstay of management with excellent prognosis.


Bilateral diffuse uveal melanocytic proliferation (BDUMP) is an extremely rare paraneoplastic syndrome, with most cases reported as secondary to female urogenital and male lung malignancies. We reported this case of BDUMP patient whose primary malignancy was gastric adenocarcinoma verified with gastroscopy and subsequent pathological test.A patient complaining blurred vision was suspected of bilateral diffuse uveal melanocytic proliferation (BDUMP), due to bilateral round oval patches at the posterior pole and cardinal signs in retinal angiography. Malignancy screening was suggested, and pathological report from gastroscopy confirmed the primary lesion as gastric adenocarcinoma. The patient chose palliative care due to late stage and unresectable nature of the malignancy.Identifying BDUMP warrants further investigation of a primary malignancy. Our case provided evidence for the link between gastric adenocarcinoma and BDUMP. Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic syndrome (PNS) affecting the eye, with around 60 cases reported . There aA 50-year-old Chinese male presented with bilateral blurred vision for 3 months. Nine months earlier, he experienced pulmonary embolism and lower limb venous thrombosis, and was diagnosed with antiphospholipid antibody syndrome (APS). He had lost 10 kilograms in the past 9 months. No other gastric or constitutive symptoms were reported.On examination, his best corrected visual acuity (BCVA) was 20/200 OU. Anterior chamber was basically normal except for moderate cataract in both eyes. Fundus examination showed bilateral diffuse oval yellow patches in the posterior pole  positron emission tomography–computed tomography (PET/CT)  factor . HepatocTreatment of BDUMP primarily targets the primary malignancies, including local resection, radiation and systemic chemotherapy. Since systemic factors elicited by primary malignancies is considered involved in the pathogenesis of BDUMP, this could possibly explain the improvement of visual symptoms in some cases after these treatments targeting the malignancy . PlasmapIn summary, identifying BDUMP warrants further investigation of a primary malignancy. Our case provided evidence for the link between gastric adenocarcinoma and BDUMP.


Traumatic rupture of the diaphragm with herniation of abdominal viscera into the pericardium is a rare injury. We present a case of intrapericardial diaphragmatic hernia after one year of thoracoabdominal trauma. This case is the 106th in the literature and only the fifth treated by the laparoscopic approach including this case. After case presentation, we will discuss post-traumatic intrapericardial diaphragmatic hernias by reviewing the literature.A 48-year-old male presented to our outpatient clinic with a history of thoracoabdominal trauma due to a motor vehicle accident one year ago. The patient complained from abdominal pain and constipation over the last year. After a thorough history and physical examination, thoracoabdominal computed tomography (CT) was performed. The CT scan revealed herniation of the transverse colon and omentum through a large anterior diaphragmatic defect into the pericardial sac. Laparoscopic composite mesh repair was done. The patient had an uneventful postoperative course and was discharged home on the second postoperative day. The patient was asymptomatic at regular follow-up six months postoperatively.Late presentation of intrapericardial diaphragmatic hernia is rare. Chest computed tomography is the most useful diagnostic tool. Delayed diagnosis of Intrapericardial diaphragmatic hernia does not preclude laparoscopic repair by primary closure or with mesh according to the size of the defect. The diaphragm is originated between fourth to twelfth weeks of gestation from septum transversum, pleuroperitoneal membranes, mediastinal dorsal mesentery of the esophagus and the body wall muscles . The diaDiaphragmatic hernia caused by congenital defects or trauma (blunt or penetrating). The estimated prevalence of traumatic diaphragmatic hernia is 3–7% of all abdominal and thoracic traumas .22 gas was maintained at 12 cm H2O and with reverse Trendelenburg modified lithotomy position. A defect measured 12 × 7 cm was seen in the central area of the diaphragm. Greater omentum and part of transverse colon herniated into the pericardial sac  was performed. The CT scan revealed herniation of the transverse colon and omentum through a large anterior diaphragmatic defect into the pericardial sac . Laparosdial sac . Adhesiodial sac . No drai3Keith reported the first two cases of traumatic intrapericardial diaphragmatic hernia (TIPDH) as autopsy findings in 1910 . Reina eBy reviewing the literature, four additional cases of TIPDH were found before 2012 that were not mentioned by Kuy et al. Since 2012, five additional cases of TIPDH were reported in the literature. This review includes 10 cases of TIPDH including the presented case, so the total number of cases became 106 cases.Our patients was treated with laparoscopic approach and it is the fifth reported in literature and the second to be repaired with composite mesh. 4The traumatic diaphragmatic hernias mostly occur at anatomic weakness where the diaphragm attaches to the chest wall in the left posterolateral aspect. The blunt abdominal trauma may rarely cause central tendon rupture and give the access of the abdominal viscera to herniate into the pericardial sac. As the pericardial space has limited space, abdominal viscera herniation into it mandates surgical repair to prevent life-threatening cardiac tamponade .The surgical approach for TIPDH repair is not clear yet. Nevertheless, laparotomy is the preferred approach for acute presentation and thoracotomy for chronic delayed presentation owing to more feasible for adhesiolysis. Other authors reported that the laparoscopic, either primarily or with mesh, approach is a safe, feasible and effective for both acute and chronic TIPDH repair. Some reports showed that there were no significant adhesions between the heart itself and the abdominal viscera. Adhesions between the abdominal viscera and the pericardial lining can be easily divided using harmonic scalpel, and this is what we experienced in the this case ,11. Acut5Delayed intrapericardial diaphragmatic hernia is a rare presentation after trauma. Chest computed tomography is the most diagnostic tool of choice. Delayed diagnosis of Intrapericardial diaphragmatic hernia does not preclude laparoscopic repair by either primary closure or with mesh for large defects.No conflict of interest.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Ethical approval for this study was obtained from Mu’tah faculty of Medicine Ethics committee (Reference number. 202029).Based on our ethical considerations and guidelines, the committee has approved the project without amendments.Statement in the publication”Ethical approval was obtained from Mu’tah faculty of Medicine Ethics committee”.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Aborajooh A. Emad: study concept or design, data collection, data analysis or interpretation, writing the paper.Al-Hamid Zaid: study concept or design, data collection, data analysis or interpretation, writing the paper.1.Name of the registry: Traumatic Intrapericardial Diaphragmatic Hernia: Laparoscopic Composite Mesh Repair and Literature Review.2.Unique Identifying number or registration ID: researchregistry5384.3.https://www.researchregistry.com/browse-the-registry#home/.Hyperlink to your specific registration (must be publicly accessible and will be checked). Aborajooh A. Emad.Department of General Surgery, Mutah University, Al-Karak, 61710, Jordan.Editorially reviewed, not externally peer-reviewed.


Erdheim-Chester disease (ECD) is a rare type of blood cancer characterized by infiltration of the body tissues by pathologic histiocytes, leading to widespread inflammation and damage. Clinical presentations range widely, from asymptomatic infiltration of bone to multiple organ system damage and resultant dysfunction. This report describes a case of a patient with several unusual imaging findings that led to a differential diagnosis of ECD; however, a biopsy of a mediastinal mass suspected to be due to histiocyte infiltration instead revealed primary lung cancer. Ultimately, ECD could not be ruled out, and the patient was referred to dermatology for a superficial facial xanthelasma biopsy, results of which were consistent with ECD. Concurrent ECD and adenocarcinoma is highly unusual; this case demonstrates the importance of a thorough investigation and the consideration that not all findings may be attributable to a single disease process, even when the alternative is very unlikely. This case also highlights the diversity of ECD in terms of its clinical presentation and affected organ systems, and discusses the utility of multiple imaging modalities in contributing to making the diagnosis, emphasizing the importance of a thorough radiological workup and high level of clinical suspicion.A 56-year-old Trinidadian female presented to the emergency department with intermittent left-sided chest pain for 3 days, which was exacerbated by exertion and associated with shortness of breath. Additional symptoms included headache, fatigue, and 15- to 20-pound weight loss with bilateral lower extremity pain over the preceding year. Physical examination revealed numerous facial xanthelasmas. Chest X-ray revealed an enlarged cardiac silhouette, suspected pulmonary vascular congestion, and subcentimeter calcifications due to old granulomatous disease. Computed tomography (CT) of the chest revealed small pericardial and left pleural effusions , and a lCT-guided biopsy of the superior mediastinal lymph nodes revealed poorly differentiated adenocarcinoma with necrosis positive for cytokeratins AE1/AE3 and TTF1, compatible with lung primary. Findings and suspicions were discussed with the patient, and she was referred to dermatology for biopsy of right cheek xanthelasma. This biopsy revealed a dermal nodular infiltrate of histiocytes with foamy cytoplasm mixed with lymphocytes and rare Touton-type giant cells, consistent with ECD.2 Because ECD affects multiple organs and organ systems, however, presentation at diagnosis and associated imaging findings can be highly variable. In this case, demonstration of perinephric soft tissue encapsulating the kidneys on CT and giving them a “hairy” appearance was the first sign alerting clinicians to the potential presence of ECD. CT findings of pericardial and pleural effusions,2 combined with findings of increased uptake in the lower extremities on bone scan,1 further supported this diagnosis. As central nervous system involvement, often characterized by multiple lesions, has been reported in >50% of patients.5 The 2 brain lesions were also considered consistent with the working diagnosis. It was therefore somewhat surprising when the mediastinal lesion was biopsy proven as adenocarcinoma, with ECD proven later on via xanthelasma biopsy.Although definitive diagnosis of ECD relies on identification of CD68(+), CD1a(−) histiocytes on biopsy, radiological findings can suggest the diagnosis.6 Given confirmation of the presence of both types of cancer, it is unclear if the central nervous system lesions are due to ECD or to metastatic spread of adenocarcinoma.Concurrent diagnosis of ECD with adenocarcinoma is rarely seen: a PubMed search using the terms “Erdheim Chester cancer,” “Erdheim Chester carcinoma,” and “Erdheim Chester adenocarcinoma” revealed no other reported cases of ECD co-occurring with adenocarcinoma, and only one case of Erdheim-Chester co-occurring with papillary thyroid carcinoma.7; a recent study by Chasset et al identified xanthelasma-like lesions as the most common cutaneous manifestations (25% of cases).8 As such, it was unclear if the biopsy would indeed yield positive results or if the patient’s facial xanthelasma would be due to other causes. Given the multitude and diversity of what were highly suspected to be ECD manifestations in this patient, suspicions that the facial lesions would demonstrate the same etiology were also high; however, with limited information available on how frequently xanthelasmas in ECD patients are in fact due to the disease, this potential downfall of the biopsy was emphasized when the option was presented to the patient. Finding a positive result was therefore encouraging; it raises the question of how specific this sign may be for the disease, since a facial xanthelasma biopsy, if possible, would be a less invasive and low-risk diagnostic option for patients with xanthelasma suspected to have ECD.It is estimated that only a quarter to half of patients with ECD present with cutaneous manifestations of the diseaseErdheim-Chester disease should be considered as a differential diagnosis in patients with signs of malignancy accompanied by diffuse tissue thickening and fibrosis. Simultaneous occurrence of ECD and adenocarcinoma is highly unusual; in this case, the presence of adenocarcinoma was biopsy confirmed, but could not explain the majority of imaging findings. Xanthelasma biopsy may be a viable option to diagnose ECD, but it may be beneficial to further investigate the specificity of this sign in this patient population. Ultimately, this case demonstrates the importance of a thorough workup: in a disease like ECD with such diverse features, it is important to consider that not all findings may be attributable to a single disease process.


Main teaching point: Solitary necrotic nodules of the liver can be multiple and usually appear as hypovascular nodules mimicking hepatic metastases, but they are relatively small and located in the subcapsular areas of the liver.We report a very rare case of multiple solitary necrotic nodules of the liver mimicking hepatic metastases in a patient with previous history of lung cancer. The lesions appeared as low-attenuated or low-signal intensity nodules with thin rim enhancement on both contrast-enhanced computed tomography (CT) and magnetic resonance (MR) imaging, making them difficult to differentiate from hepatic metastases. This rare benign entity should be kept in mind, especially when lesions are small and located in the subcapsular areas of the liver. Solitary necrotic nodule of the liver is an extremely rare lesion that is benign but commonly mistaken as malignancy, such as hepatic metastases, due to its nonspecific imaging findings. First reported by Shepherd et al. in 1983 3. A soliA 64-year-old woman was referred to our hospital for incidentally discovered hypoechoic hepatic nodules on abdominal ultrasound (not shown). Her past medical history was significant for surgery and chemotherapy for adenocarcinoma of the lung 12 years prior. The patient had no clinical symptoms at presentation. Laboratory tests showed elevated serum carbohydrate antigen 19–9 (CA 19–9) of 42.2 U/mL . All other laboratory tests, including complete blood count (CBC), electrolytes, aspartate aminotransferase (AST), and alanine aminotransferase (ALT), were within normal limits. The hepatitis virus serological tests were also normal.2, the lesions showed high signal intensity  positron-emission tomography (PET)/CT revealed mild hypermetabolism of the lesions  and magnetic resonance (MR) imaging were performed. Contrast-enhanced CT revealed two, approximately 1.2-cm-sized, low-attenuated nodules showing rim-enhancement in liver segments 2 and 8 Figure . Both noy Figure  and 2-[1s Figure . TherefoThe patient underwent laparoscopic segmentectomy for segments 2 and 8. Pathologic examination revealed two necrotic nodules located in the subcapsular areas of the liver. The lesions were slightly lobulated, and a small cystic change was observed in the nodule of segment 2, correlating with the MR imaging findings Figure . MicroscWe herein report a case of two incidentally found solitary necrotic nodules of the liver that mimicked hepatic metastases on imaging. Even with remarkable improvements in diagnostic imaging of the liver with CT and MR, some hepatic lesions are challenging to diagnose preoperatively. Solitary necrotic nodules are benign lesions, with no reported cases of serious complications, such as malignant transformation. Thus, to avoid unnecessary surgical exploration, radiologists should be aware of the imaging findings associated with solitary necrotic nodules of the liver.Solitary necrotic nodules of the liver are mostly small, well-defined lesions measuring less than 3.0 cm with a round, oval, or lobulated shape 5. They a4On contrast-enhanced CT, solitary necrotic nodules usually present as well-defined low- or iso-attenuated nodules. Usually, the lesions show no contrast-enhancement, but thin rim-enhancement may be seen . The les8Geng et al.  reportedPET/CT is a clinically useful imaging modality not only for staging or follow-up of malignancies, but also for further evaluation of suspicious findings on CT or MR imaging . HoweverIn conclusion, solitary necrotic nodule of the liver is a benign entity that usually shows low attenuation on contrast-enhanced CT and variable signal intensity on T2WI of MR imaging. Absence of contrast enhancement on CT or MR imaging may be helpful in differentiation of solitary necrotic nodules from hepatic metastases. However, thin rim-enhancement may also be seen due to the fibrous capsule. Radiologists should keep solitary necrotic nodules in mind as a possible differential diagnosis when hypovascular hepatic nodules are relatively small, solitary, or located in the subcapsular areas of the liver.


A previously well female presented with a history of progressive functional decline. She had a known history of generalized seropositive myasthenia gravis on long-term mycophenolate mofetil and pyridostigmine. MRI of her brain reported multiple cerebral abscesses based on radiological features. The patient was commenced on intravenous antibiotics, but there was no clinical or radiological response to treatment. Decision was made for a stereotactic biopsy. Intraoperative tissue cultures were negative for infection. However, histology reported B-cell lymphoma with morphological features changes typically seen in corticosteroid-treated lymphoma - an unexpected finding as no steroids were administered as part of her treatment. Owing to the unusual diagnosis, the case is presented in corroboration with current literature. Primary central nervous system lymphoma (PCNSL) is a rare, extranodal non-Hodgkin lymphoma accounting for up to 6% of all primary brain tumors ,2. WhileAn 89-year-old female presented with progressive functional decline of two weeks’ duration. Prior to this, she was fully independent in her activities of daily living and ambulated without walking aid. There was no family history of malignancy, recent travel, or contact history. During this period of decline, her family members noted global memory impairment, dysphasia, and increasing gait and swallowing difficulties. The patient had a background of non-thymomatous generalized seropositive MG and was given a course of prednisolone and azathioprine. Both medications were tapered off after a period of sustained remission. After a subsequent myasthenic crisis, she was started on mycophenolate mofetil (MMF) and pyridostigmine. She recovered well and was in remission since. At the time of this report, the patient had been on long-term MMF and pyridostigmine for more than 20 years Table .Clinical examination did not demonstrate any obvious visceral or neurological abnormality. During physical examination, the patient was alert, non-toxic looking, and oriented to place and person, although she had a short attention span and slowed processing. There were no focal neurological deficits, meningism, or signs of raised intracranial pressure. Her laboratory results were largely unremarkable for any significant infective, metabolic, or systemic abnormalities Table .Magnetic resonance imaging (MRI) of her brain reported multiple, variably sized, ring-enhancing parenchymal lesions in the periventricular and lobar regions. There was associated perilesional vasogenic edema and local mass effect and gyral swelling. Some of the lesions displayed contents with restricted diffusion on diffusion-weighted imaging (DWI) Figure . A folloThe imaging findings, in concert with the clinical presentation, were concerning for several etiologies given her history of long-term immunosuppressant therapy and radiological findings of multiple ring-enhancing lesions, the working diagnosis was that of an infective cause such as multiple cerebral abscesses versus cerebral toxoplasmosis. Another possibility based on her age, multifocal nature of lesion, and degree of vasogenic edema was that of a neoplastic process. Nonetheless, comprehensive CT imaging of her thorax, abdomen, and pelvis was performed as part of the workup. The results were negative for infective lesion or tumor growth. Extensive microbiological investigations including microbiological cultures and serological studies, including a transthoracic echocardiography, were unremarkable Table . A lumbaAs multifocal cerebral abscesses were the top differential, the patient was started on meningitic doses of ceftriaxone and metronidazole. A repeat contrast-enhanced MRI of the brain was performed after a week of antimicrobial treatment, which revealed grossly stable size and appearance of the multiple ring-enhancing lesions with perilesional edema, and no new lesions were seen. In view of the lack of clinical and radiological improvement following a trial of antibiotics, stereotactic biopsy was performed to obtain definitive histopathological diagnosis. The right caudate lesion lateral to the frontal ventricular horn was selected for the procedure. A key consideration was the benefit of preventing its rupture (in view that its contents were possibly infective) into the CSF-filled ventricles. Intraoperative sampling from the lesion and its cystic component yielded firm necrotic tissue and small amounts of turbid fluid. These samples were sent for histological and microbiological examination. The intraoperative fluid and tissue aerobic and anaerobic cultures, fungal stains and cultures, TB (tuberculosis) stains, and TB PCR (polymerase chain reaction) tests were all negative. On histopathology, there was abundant necrotic tissue and inflamed granulation tissue. Within the necrotic tissue, atypical cells were seen around blood vessels. On immunohistochemistry, there were accompanying concentric networks of reticulin fibers around the blood vessels, and strong staining for CD20, a B-cell marker, in the atypical cells and necrotic cells  scans and bone marrow aspiration were not performed. Instead, decision was made for palliative whole-brain radiation therapy (WBRT). Antibiotics were discontinued, and the patient was maintained on low dose-dexamethasone during the duration of her WBRT (20 Gy in five fractions). She was discharged well with home hospice support.PCNSL is an uncommon and malignant variant of extranodal non-Hodgkin lymphoma that involves the brain, leptomeninges, eyes, and spinal cord without evidence of systemic disease . The locAlthough MRI is the radiological modality of choice, differentiating between PCNSL and cerebral abscess can be challenging. This is because ring-enhancing lesions on conventional MRI sequences have a list of differentials, ranging from infective processes to high-grade tumors . FurtherAt this point in time, the association between iatrogenic immunosuppression and the development of PCNSL is unclear. We are already aware of how corticosteroids have a cytolytic effect on lymphoma cells ,13. ThisPutting it all together, any subacute presentation of neurological deterioration, progressive lateralizing deficits, or signs and symptoms of raised intracranial pressure or meningism should prompt early neuroimaging, especially in high-risk patients. In immunocompromised patients, other important differential diagnoses such as CNS infections and other CNS malignancies should be considered. Neuroimaging findings may mimic other diagnoses such as cerebral abscesses. The use of advanced metabolic imaging can be helpful. Presently, histological diagnosis remains to be the gold standard and is paramount in guiding clinical treatment and management.In summary, the diagnosis of PCNSL requires a high index of suspicion, especially in patients on chronic immunosuppressants. The case in this report highlights the need for clinicians to be mindful of this rare entity as a differential diagnosis in a selected group of patients, as early intervention is paramount. We emphasize the need for international collaborations to work closely together for better, in-depth mechanistic understanding of this devastating disease.


Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited syndrome that concurrently involves various endocrine glands. We report a rare case of MEN1 in a 43-year-old man whose first manifestation was an asymptomatic mediastinal mass.MEN1 gene mutation was observed in the patient and his 7-year-old son.A 13-cm-sized mediastinal mass was diagnosed as an atypical thymic carcinoid by computed tomography and percutaneous needle biopsy. In addition, hypercalcemia from a left inferior parathyroid hyperplasia, and a non-functioning gastric neuroendocrine tumor seen on esophagogastroduodenoscopy were found. Therefore, the patient was clinically diagnosed with MEN1 syndrome, and underwent surgical resection of thymic carcinoid tumor after pre-operative concurrent chemoradiation therapy to decrease tumor size and volume. Parathyroid lesion and gastric neuroendocrine tumor were also removed. Finally, a Despite its rare occurrence, thymic carcinoid tumor should be considered as a MEN1-associated tumor and necessitates screening of other endocrine glands. Thymic carcinoid tumor carries a poor prognosis in patients with MEN1, and thus it needs to be carefully monitored even after radical excision.The online version contains supplementary material available at 10.1186/s12902-021-00695-9. Multiple endocrine neoplasia type 1 (MEN 1) is a rare hereditary syndrome characterized by a predisposition for neoplasms involving two or more endocrine glands, primarily of parathyroid, anterior pituitary, and enteropancreatic origin . Among tA 43-year-old man was admitted to the hospital due to an enlarged mediastinum incidentally observed on chest x-ray during a routine health check-up Fig. a. The paEnhanced chest computed tomography (CT) showed an anterior mediastinal mass invading the superior vena cava and pericardium that measured approximately 13.0 × 8.0 × 6.0 cm in size , fasting C-peptide was 4.85 ng/mL , and glucagon was increased to 431.7 pg/mL . Serum calcium level was slightly elevated at 10.9 mg/dL  and albumin-corrected calcium was 10.8 mg/dL with a normal phosphorus level of 3.7 mg/dL . In the work-up for hypercalcemia, serum intact parathyroid hormone (PTH) level was elevated at 78.7 pg/mL, and recheck level was more increased up to 128 pg/mL . A 25-hydroxy vitamin D was low at 8.0 ng/mL, and the 24-h urine calcium amount was 300 mg/day. A bone density scan showed anterior-posterior spine L1–4 bone mineral density (BMD) of 0.806 g/cmAbdominal CT Fig. a found mEvaluations of the pituitary gland revealed a slightly elevated serum prolactin level of 21.9 ng/mL , normal thyroid hormones, normal IGF-1, normal FSH, normal testosterone, but a low LH level < 0.5 mIU/mL . Morning cortisol level was normal (10.2 μg/dL), and serum cortisol was suppressed by a 1 mg overnight dexamethasone. Basal ACTH level was 64.8 pg/mL . Sella MRI showed a 0.3 cm, delayed enhancing lesion, which was hard to differentiate between pituitary microadenoma and normal variant of pituitary gland , which was reported in two prior MEN1 cases in Korea [It is noteworthy that early diagnosis of MEN1 is mandatory for identification and subsequent treatment at a very early stage for preventing malignant progression and metastases as well as clinical consequences of hormone overproduction-related syndromes, which are the main causes of mortality and morbidity of patients with MEN1 . In thisial MEN1 . The patin Korea , 14. DNAin Korea , so we hMEN1 mutation.In conclusion, the present study reported an asymptomatic MEN1-associated thymic carcinoid as the first manifestation of MEN1. All MEN1-associated endocrine tumors were successfully removed, and there was no evidence of tumor recurrence at postoperative 1 year. Systematic evaluations and close follow-up of other endocrine organs are needed in patients with thymic carcinoid tumor and Additional file 1: Supplementary Fig. 1. (A) Pedigree chart of the patient. The index patient and his son carry the same MEN1 gene mutation. (B) DNA sequencing shows a heterozygous missense mutation in exon 3 .Additional file 2: Table S1. Diagnostic criteria of MEN1 *[


Definitive treatment of primary middle ear lymphoepithelial carcinoma (LEC) is not well established owing to the rarity of this disease entity. We report a case of locally advanced primary middle ear LEC treated with concurrent chemoradiation, with good oncologic outcomes.A 46 year-old female of Cantonese (Southern Chinese) descent presented with a four-month history of left sided hearing loss and non-pulsatile tinnitus, associated with progressive ipsilateral facial weakness. She had a left facial palsy (House-Brackmann 2) which then deteriorated to complete palsy over 2 weeks. Otoscopic examination of the left ear revealed a red-hued mass replacing the tympanic membrane. There was no cervical lymphadenopathy. Fibreoptic nasoendoscopy was unremarkable. Pure tone audiometry revealed profound mixed left hearing loss with type B impedance.Computed tomography of the temporal bone showed an ill-defined left middle ear mass with erosion of the malleus, tegmen tympani and facial canal. Magnetic Resonance Imaging showed an avidly enhancing lesion involving the dura of the left middle cranial fossa, tympanic and labyrinthine portions of the facial nerve. This mass extended into the apex of the left internal acoustic canal and basal turn of the cochlea. Histopathology confirmed EBV-positive primary middle ear LEC. Concurrent chemoradiation comprising 70Gy of intensity-modulated radiation therapy and 3 cycles of concurrent Cisplatin based chemotherapy over a 6 week period was administered. The patient achieved near-complete disease resolution on 3 month post-treatment imaging. Serum EBV DNA titres declined to undetectable levels and the patient is disease-free at 18 months post-diagnosis.Concurrent chemoradiation with curative intent may be a viable treatment option for locally advanced middle ear LEC not amenable to surgical resection due to expected surgical morbidity. It confers good oncologic outcomes that mimic the response in other head and neck EBV-related lymphoepithelial carcinomas.The online version contains supplementary material available at 10.1186/s40463-020-00489-4. Lymphoepithelial carcinoma (LEC) of the middle ear is an extremely rare disease entity , 2. LECsTreatment modalities reported in literature include RT alone and surgery with adjuvant radiation. However, surgery in locally advanced tumors in this region can result in significant morbidity .Presently, there are no reports of employing definitive concurrent chemoradiation (CCRT) for middle ear LEC. We report a case of locally advanced, EBV-related primary middle ear LEC that was treated with CCRT with good results.A 46 year-old female of Cantonese (Southern Chinese) descent presented with a four-month history of left sided hearing loss and non-pulsatile tinnitus without vertigo, associated with progressive ipsilateral facial weakness. Left ear otoscopy revealed a red-hued mass replacing the tympanic membrane Fig. a. She haComputed tomography (CT) of the temporal bone showed a left middle ear mass with partial erosion of the malleus, incus, tegmen tympani and facial canal Fig. b. The inThe patient underwent a transcanal biopsy and histopathology is shown in Fig. Correlating imaging features illustrating middle ear tumor epicentre, a diagnosis of primary middle ear LEC was made. Multidisciplinary head and neck tumor board review recommended for CCRT in view of anticipated radio-sensitivity and locally advanced disease with intracranial involvement. This is in line with treatment guidelines for nasopharyngeal carcinoma, where locally advanced disease is treated with a combination of chemotherapy and radiation . Our patPrimary tumors of the temporal bone are rare. Differentials for a vascular-appearing middle ear mass seen on otoscopy include glomus tumors, facial nerve schwanomma, middle ear adenoma/adenocarcinoma, meningioma and metastases from kidney, breast or lung tumors. Facial nerve schwannoma would be supported by the clinical presentation, but imaging features of dural involvement and permeative changes in the eroded tegmen make this differential less likely. Meningiomas are commonly epicentered in the cranial fossa, with middle ear cavity involvement usually due to direct spread rather than being the epicenter. CT features of tegmen permeative changes suggest an aggressive middle ear lesion, which excludes middle ear adenoma. Glomus tumors may result in similar bony erosion but glomus tympanicum with extensive dural involvement is unusual. Distinguishing middle ear LEC from other middle ear malignancies such as adenocarcinoma would require a biopsy and immunohistochemistry, as in this case, to confirm the diagnosis.Lymphoepithelial carcinomas have been reported in various sites in the head and neck, such as the major salivary glands, oral cavity, oropharynx and lacrimal gland. Primary middle ear LEC is rare, with little known about the optimal treatment. However, one would postulate that it is a radiosensitive tumor based on its morphologic similarity to EBV-related nasopharyngeal carcinoma. Furthermore, it has been suggested that the significant lymphocytic infiltration in LECs elicit a strong immunologic response, thus conferring a better prognosis than poorly-differentiated carcinomas of the same site . TreatmeIn a review of Surveillance, Epidemiology, and End Results (SEER) cancer epidemiological and survival data on non-nasopharyngeal LEC of the head and neck, Chan reported that treatment without RT was independently associated with poorer survival . Their sThere are several differentials for a primary middle ear lesion; CT and MRI imaging can assist in establishing a diagnosis if typical imaging features are present, otherwise a biopsy is indicated for definitive diagnosis. While rare, EBV-related middle ear LEC is a possibility and CCRT appears to be an effective treatment for locally-advanced disease achieving oncologic control while avoiding the morbidity of surgical resection.Additional file 1.Additional file 2.


Lipoid proteinosis (LP) is a rare multisystem inherited disease. We report here in three LP cases with beaded papules of the eyelid. Their clinical presentations, histological characteristics, and genetic findings are described and discussed.A 12-year-old boy reported to our hospital with a complaint of ocular irritation, redness, and tearing for two years. He had a history of hoarseness since childhood. His younger brother (5 years old) also complained of hoarseness. Another patient, a 26-year-old woman, described many beaded papules on the edge of her eyelids since age 11 years. She additionally reported hoarseness since 4 years of age. Careful slit-lamp examination in these cases revealed waxy beaded papules on the margins of both eyelids and mild conjunctival congestion. Physical examination showed irregular, rugged scars on their facial skin. Genetic analysis showed the mutation located in exon 6 of the ECM1 gene.Three LP cases first diagnosed by ophthalmologists are presented. The presence of eyelid papules should prompt the ophthalmologist to pay close attention to the patient’s voice. If there is a definite history of hoarseness, these patients should undergo gene sequence analysis. If necessary, otorhinolaryngology and dermatology consults may help confirm the diagnosis. Treatment is primarily symptomatic to improve patients’ quality of life. Lipoid proteinosis (LP), first described by Urbach and Wiethe in 1929, is an autosomal recessive genodermatosis caused by mutations in the extracellular matrix gene 1 (ECM1) on chromosome 1q21 [ A 12-year-old boy (case 1) was referred to our department with complaints of ocular irritation, redness, and tearing for 2 years. He had a history of hoarseness since childhood and complained of recurrent large ulcerations on his tongue, as well as fragile skin with bullous lesions that appeared after minimal trauma. His younger brother  had no complaints other than hoarseness. They all denied a previous history of surgery or medication. Their parents and close relatives had no similar history.Slit-lamp examination revealed waxy beaded papules on the margins of both eyelids and mild conjunctival congestion in both cases Fig. a, b. PhyA biopsy of an eyelid papule was performed in case 1. Periodic acid-Schiff staining showed infiltration of an eosinophilic hyaluronic substance into many areas of connective tissue. Similar deposits were also found around sweat glands and capillaries, associated with epithelial thinning  in exon 6 of the ECM1 gene NM_004425.4)  in exon 6 of the ECM1 gene NM_004425.4) . ECM1 protein is expressed in the skin, mucosa, and several other tissues, including the placenta, heart, liver, small intestine, lungs, kidneys, and endothelial cells. ECM1 can stimulate blood vessel endothelial cell proliferation and influence the differentiation of keratinocytes. ECM1 promotes binding of collagen IV and laminin and acts as a biological glue by binding glycosaminoglycans to fibrillar protein growth factors [ECM1 function results in reduced production of normal collagen, with abnormal interactions with perlecan, MMP-9, fibulin, and laminin [ECM1 protein may play a role in wound healing, scarring, and aging.The molecular basis of LP has recently been elucidated, and it appears to result from mutations in the gene encoding extracellular matrix protein 1 (Ecm1,  factors . Abnorma laminin . ECM1 prECM1 gene have been reported in more than 50 unrelated patients with LP [To date, at least 47 different mutations in the  with LP . FrameshLP lacks effective treatment. Fortunately, with its slow progression and stable symptoms in adulthood, LP does not generally affect lifespan. It has been reported that oral dimethyl sulfoxide , D-penicTo conclude, when examining patients with eyelid papules, any voice or skin changes should be carefully noted. With a definite history of hoarseness, these patients should be sent for gene sequence analysis. If necessary, otorhinolaryngology and dermatology consults may help confirm the diagnosis. For LP, treatment is primarily symptomatic to improve patients’ quality of life.


Omental cystic lymphangioma is a rare benign intraabdominal anomaly with uncertain etiology, predominantly occurring in children. Most cases of abdominal lymphangioma are asymptomatic. However, patients may occasionally present with acute abdomen because of an intestinal obstruction or peritonitis caused by infected cysts, hemorrhaging, and/or torsion. This report describes a case of omental cystic lymphangioma with acute intracystic haemorrhage. Ultrasonography and computed tomography (CT) scan confirmed the diagnosis. Complete excision of the cyst along without omentectomy done with no clinical or radiological evidence of recurrence till 17 months. A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. Lamphangioma is located preferentially in head, neck, and axilla in children. However, lymphangiomas in abdomen are extremely rare. They account for 3%  to 9.2% A 3-year-old boy presented with huge abdominal swelling, he had experienced 2 months of progressive abdominal distension and had to walk bent over for a few days prior to admission. He had experienced abdominal pain or vomiting and although eating required a long time, it was nevertheless still possible. On physical examination, tachycardia, abdominal distension, and scarce bowel sounds were found. No mass could be palpated. The laboratory data presented anemia (6g/dl).The abdominal x-ray was unremarkable. The ultrasonographic examination demonstrated a giant cystic lesion with internal echogenic particles and septations that extended from epigastrium to pelvis, displacing all retroperitoneal and intraperitoneal organs posteriorly with clear margins between visceral organs . No appaAt surgery, a huge, soft cystic mass lesion with intracystic haemorrhage and smooth surface was found in the greater omentum, totally filling the abdominal cavity and displacing all intraabdominal viscera posteriorly, that was not adherent to the neighboring structures. The cystic lesion was resected totally; no bowel or other organ resection was done . The patLymphangioma is a benign lesion, with more than 80% of cases occurring during childhood , 10. It Plain radiographs may show a non-calcified soft-tissue mass, displacement of intestinal loops, or features of small bowel obstruction . A well-Abdominal lymphangiomas should be differentiated from other abdominal cystic masses such as cystic teratomas, mucinous cystadenomas, bronchogenic cysts, ovarian cysts, nonpancreatic pseudocysts, and complicated ascites , 16. TheIn a case of large cystic mass with thin walls, internal echogenicities and septations, the diagnosis of omental lymphangioma should be suspected, but histological diagnosis is necessary for definitive diagnosis. This rare case is presented because of its unusual presentation with huge size and acute intracystic haemorrhage.


Muir-Torre syndrome is a rare, autosomal dominant genodermatosis, characterized by sebaceous neoplasms and visceral carcinomas. The authors describe the case of a patient who, 16 years after the diagnosis of colon carcinoma, presented a verrucous plaque on the pubic region, histopathologically compatible with sebaceous adenoma. The need to investigate this syndrome is emphasized, especially in cases of sebaceous neoplasms located outside the head, face, and neck. Screening for neoplasms in these patients and their families is mandatory. The authors describe the case of a 79-year-old woman, presenting a warty, erythematous, and painless plaque for two years on the pubic region . There wa)Amelanotic melanomab)Condyloma acuminatumc)Squamous cell carcinomad)Sebaceous adenomaBased on clinical and histopathological findings, the authors reached the diagnosis of sebaceous adenoma in a patient with Muir-Torre syndrome (MTS). Sebaceous tumors are rare, most commonly located on the face and neck of the elderly.The diagnosis of sebaceous neoplasia makes MTS screening imperative. This is a rare variant of Lynch syndrome, autosomal dominant, characterized by the occurrence of sebaceous gland tumors  associated with visceral neoplasia. Among the sebaceous tumors related to MTS, adenoma is the most common, histologically characterized by lobular proliferation with predominance of mature sebocytes in the center and a minority of basaloid germ cells at the periphery. Colon adenocarcinoma is the most common associated visceral neoplasia, especially when proximal to the splenic flexure (unlike sporadic cases), followed by tumors of the genitourinary tract.The diagnosis of MTS is clinical, by finding sebaceous neoplasia and at least one visceral tumor without other contributing factors, such as radiotherapy or immunosuppression. Immunohistochemistry and molecular analysis are indicated in cases where there is no family or personal history of visceral neoplasms.The authors report a case of sebaceous adenoma with unusual location in the pubic region in a patient with colon and bladder cancer. The importance of investigating systemic neoplasms associated with SMT and the monitoring of these patients and their families is emphasized.None declared.Diego Henrique Morais Silva: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; critical review of the literature; critical review of the manuscript.Anna Karoline Gouveia de Oliveira: Design and planning of the study; collection, analysis, and interpretation of data.Neusa Yuriko Sakai Valente: Approval of the final version of the manuscript, effective participation in research orientation, intellectual participation in propaedeutic and/or therapeutic conduct of studied cases, critical review of the manuscript.Thais do Amaral Carneiro Cunha: Approval of the final version of the manuscript, intellectual participation in propaedeutic and/or therapeutic conduct of studied cases.None declared.


Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome is a rare obstructive uterovaginal anomaly involving both mesonephric and paramesonephric ducts. It usually presents after menarche with non-specific symptoms like pelvic pain, dysmenorrhea, or paravaginal mass and examination findings of paravaginal or pelvic mass. Because of non-specific symptoms and signs, the diagnosis is usually overlooked, which leads to complications like endometriosis, tubo-ovarian abscess compromising patient's fertility, and quality of life. Therefore, in presence of these nonspecific clinical features along with imaging findings of uterine didelphys and unilateral renal agenesis, this syndrome should be considered in the diagnosis. We present a case of a 17-year-old lady with Obstructed Hemivagina and Ipsilateral Renal Anomaly syndrome, diagnosed by finding of paravaginal mass on examination and uterine didelphys with ipsilateral renal agenesis in USG and managed successfully by resection of vaginal septum. However, it can also present with an acute abdomen and tubo-ovarian mass.4 The nonspecific presenting complaints and heterogeneity of presentation can make the diagnosis a challenge.Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich (HWW) syndrome is a rare combined paramesonephric and mesonephric duct anomaly.0A1 presented to the OBGYN outpatient department with complaints of mass associated with pain at vaginal introitus for the last one year. She also complained of difficulty in passing urine with a small urinary stream, intermittent flow, and a sense of incomplete evacuation. Her menstrual cycles were irregularly occurring every 30-45 days since her menarche at 14 years of age but did not have menorrhagia or dysmenorrhea. She did not have dyspareunia but complained of some discomfort during coitus because of the mass. She was married for one year and had an induced medical abortion at six weeks of gestation for unwanted pregnancy 8 months back. She had then been regularly taking low dose combined oral contraceptive pills. On examination, a cystic, nonreducible bulge of around 5X7 cm was seen on the right lateral vaginal wall extending up to the right fornix which was non-tender on palpation. On per speculum examination, the cervix was visualized and on bimanual examination,the uterus was deviated to the right side and was 10 weeks size. A provisional diagnosis of right lateral vaginal wall cyst was made and she was advised ultrasonography to rule out other pelvic pathologies  with mild fluid collection in the right endometrial cavity and vagina on the right side  . There wShe and her husband were counseled regarding the condition and management plan. After all preoperative investigations and evaluation, drainage of hematometrocolpos, and resection of the vaginal septum was performed as a single-step procedure. Preoperative antibiotic prophylaxis was given. The vaginal pouch was opened and around 150 ml of inspissated blood was drained . CompletHer postoperative period was uneventful. She was discharged on the third postoperative day. She followed up after 15 days in the OPD when she reported no symptoms, her per speculum examination revealed two cervices. She was counseled regarding the possibility of vaginal stenosis. She followed up again after her next menstrual period. On per speculum examination, the vagina looked normal and both cervices were visualized. She was reassured of the normal findings and asked to follow up if she had any problems.3 Other studies have reported its proportion to be between 0.16 to 10% of all Mullerian anomalies.5 The condition was first reported by Purslow in 1922.6 For the first 60 years after the first reported case, only 115 cases were reported.4 Poor awareness and incorrect diagnosis could have been responsible for the under-reporting. However, in recent years multiple large case series have been published.7 This may be because of the increasing awareness about the condition as well as improvement in diagnostic modalities.The incidence of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is reported to be 2-3% of all Mullerian anomalies in various case series.9 Though the commonly reported uterine anomaly is uterine didelphys, other anomalies like septate and bicornuate uterus also have been reported. Meanwhile, the distal part of the vagina which arises from the urogenital sinus is not affected and develops normally. Similarly, commonly reported unilateral genital obstruction is vaginal, but cervical obstruction or atresia has also been reported. In the case of renal malformations, the commonest anomaly is ipsilateral renal agenesis; however, duplicated and dysplastic kidneys have also occasionally been reported.6The frequent association of genital anomalies with urinary system anomalies is because the development of the genital and urinary system is closely related. The mesonephric ducts, not only give origin to the ipsilateral renal system but also act as inductor elements for the adequate fusion of Mullerian ducts. The ureteric bud arises from the opening of the mesonephric duct in the urogenital sinus which is responsible for the formation of the kidney on that side. In the absence of a mesonephric duct on one side, the ipsilateral kidney and ureter fail to develop. The ipsilateral Mullerian duct is displaced laterally which then fails to fuse adequately with the urogenital sinus, leading to the formation of a blind sac, or obstructed hemi-vagina. The latter also fails to fuse with the contralateral mOllerian duct which results in uterine didelphys.10 Our patient also had obstructed right hemivagina and right renal agenesis with uterine didelphys.In literature, both obstructed hemivagina and renal agenesis have been reported slightly more often on the right side (around 60%) in OHVIRA.11 In one review from China in 2013, the reported median duration from menarche to diagnosis was one year (range: 0-25 years).1 Thus, cases may present quite late in reproductive life like our patient who presented at the age of 17 years, three years after her menarche. A case series from the USA in 2007 found the median age of presentation as 14 years with a range of 10-29 years4 while another series had mean age 20.7 years with a range of 11-42 years.6 Age of presentation also varied according to whether the vaginal obstruction was complete or incomplete i.e. 12.8±1.84 years in complete obstruction and 20.68±7.43 years in incomplete obstruction.1Patients with OHVIRA syndrome are usually asymptomatic till puberty. Diagnosis is usually made after menarche with most patients diagnosed within 2 years after menarche due to the development of hematocolpos or hematometra. Some may even present in early infancy with mucocolpos or hydrocolpos due to secretions in obstructed vagina under the influence of maternal hormones.4 Common presenting symptoms are pelvic pain, dysmenorrhea, foul-smelling discharge, and pelvic mass or paravaginal mass.12 Our patient did not have symptoms like dysmenorrhea or cyclical abdominal pain but presented with paravaginal mass and difficulty in micturition. Periodicity of menstrual cycles is usually unaffected in this syndrome, as in our case. However, irregular vaginal bleeding has been reported in 20-25% of patients. Some other cases presenting with complications of obstructed hemivagina like endometriosis and tubo-ovarian abscess have also been reported .4The common clinical presentation of the condition has remained the same over the years since the first reported case. However, increasing experience has broadened the understanding of the heterogeneity of the anatomic disorders among clinicians. An adolescent girl with nonspecific symptoms of pelvic pain or dysmenorrhea is described as a typical presentation of this condition. On examination, the finding of a visible vaginal sidewall bulge leads to the diagnosis of an obstructed hemivagina, and also the finding of a second cervix.13 Proper history-taking, detailed physical examination, and appropriate imaging modalities help reach the correct diagnosis. In our case, just from history and examination, diagnosis of OHVIRA syndrome was not thought of. Ultrasonography was advised to see the extent of paravaginal mass and also to rule out other pelvic pathology. However, it led us to the diagnosis.Early detection and management are necessary to prevent associated complications like infertility, endometriosis, pelvic adhesions, and pyosalpinx or pyocolpos. Because of non-specific symptoms and lack of menstrual irregularity, the possibility of Mullerian anomaly in these patients is easily overlooked. Since the vagina is quite distensible and can accommodate a large amount of accumulated blood on the obstructed side, these patients can go unrecognized for years. Also, there is sufficient absorption of menstrual blood in between periods.14 The role of computed tomography (CT) is limited due to radiation exposure and limited soft-tissue resolution. It does not usually add extra information on sonography findings.8 Our patient underwent a CT scan of the abdomen and pelvis which confirmed the USG findings but did not add any further information. The main objective of the surgical management of this condition is to relieve obstruction and prevent associated complications. Treatment includes full resection of the vaginal septum. Resection of as much of septal tissue as possible with special care to avoid both cervices bladder and rectum should be made. Many times obstructed cervix is dilated and pulled towards the septum medially.15 Because of the anatomic distortion from hematocolpos and chronic inflammation as well as the narrow vagina in adolescent girls, the procedure becomes difficult. Therefore, some prefer a two-stage procedure with a reduction of hematocolpos in the first stage and resection of the septum in the second stage.4 However, in our case we performed a single-stage procedure. The use of laparoscopy to assess exact uterine anatomy at the time of resection of the vaginal septum has also been advocated; however, its effect on prognosis is not yet known.16 Vaginal stenosis may occur post-operatively. We followed up with our patient after she had her menstrual cycle two months later. On examination, two cervices were seen and there was no stricture.Both Ultrasonography and Magnetic resonance imaging (MRI) is helpful in diagnosis as well as in planning management of the case. While ultrasonography can provide information about uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney, MRI pelvis provides more detailed information regarding uterine morphology, its continuity with each vaginal channel, and the nature of the uterovaginal contents.OHVIRA syndrome is a rare condition and many treating doctors being unfamiliar with the condition, it does not come as a common diagnosis in clinical practice. And also this condition does not always present with specific symptoms. Therefore, a high degree of suspicion, proper knowledge of the condition, and use of appropriate imaging techniques is a must for the diagnosis. Early diagnosis and appropriate treatment area must for relieving symptoms, preventing complications related to obstruction, and preserving reproductive function.JNMA Case Report Consent Form was signed by the patient and the original article is attached with the patient's chart.None.


Enteric duplication cysts are rare malformations mostly diagnosed before the age of two, with varied clinical presentations. Ectopic gastrointestinal epithelium can be present, and management involves surgical resection. A three-month-old girl presented with rectal bleeding due to an ileocolic intussusception. Abdominal ultrasound revealed a target sign in the right upper part of the abdomen. At hydrostatic contrast enema, an incomplete reduction of the intussusception was obtained: only a trickle of contrast material entered the terminal ileum. An exploratory laparotomy ensued with manual reduction of the intussusception. At the end of the maneuver, a soft intraluminal mass was palpated within the ileocecal valve. Thus, an ileocecal resection was performed. At histology, an intraluminal enteric duplication cyst was documented, containing ectopic gastric mucosa. Secondary intussusception should be suspected even in infants in case of abnormal findings at hydrostatic contrast enema. Intraluminal enteric duplication cysts may be a rare cause of intussusception. Enteric duplication cysts are a rare congenital anomaly with varied clinical presentations that require surgical resection for definitive treatment ; the rep12/L normal 4.50–5.50 × 1012/L), and leukocytes . At hydrostatic contrast enema, the cecum was visualized but flooding of the terminal ileum with contrast material was not obtained (A full-term three-month-old girl presented at the emergency room for rectal bleeding. Her perinatal history was unremarkable, and the clinical evaluation was normal. Abdominal ultrasound revealed a target sign in the right upper quadrant with a hypoechoic formation 1.6 cm in diameter . Laboratobtained . After pobtained . Ileocecobtained . At 3-moDuplications of the small bowel are most commonly encountered. They are generally located on the mesenteric side of the intestine as opposed to Meckel's diverticula which occur on the antimesenteric side.The majority of duplication cysts is diagnosed in the first 2 years of life. They present in a variety of ways depending on their size, location, adjacencies, and whether they contain heterotopic gastric mucosa. In some cases, intestinal or respiratory mucosa, but also annular or an ectopic pancreas, was associated . Due to Signs and symptoms may vary though they generally present with abdominal pain and/or the finding of a mass. Small cystic duplications can result in localized volvulus or they can act as a lead point for small bowel intussusception. In 1989, Adamsbaum et al. first reported a 17-month-old boy who presented with a history of abdominal pain and vomiting due to an ileocecal straddling duplication cyst . In 2016