Low-grade fibromyxoid sarcoma (LGFMS) is a rare slow-growing malignant tumour with a deceptively benign histologic appearance. Herein, we report two cases of LGFMS with variable clinical presentations. The first case was a 17-year-old female who referred to our department due to deaf ear on the right together with ipsilateral gag reflex impairment and globus sensation in the pharynx. The second case was a 35-year-old female with recurrent LGFMS, suffering from headaches, vertigo, and episodes of loss of consciousness. LGFMS of the temporal bone is a rare pathology, and to the best of our knowledge, no such cases have been reported before. Head and neck sarcomas account for only 1% of all head and neck malignancies and 5% of all sarcomas . Among tA 17-year-old girl presented with globus sensation in the pharynx and deaf ear on the right side. Clinical examination revealed gag reflex impairment on the right, indicating paralysis of the glossopharyngeal nerve. Otoscopy showed an expansive process of the right middle ear. The function of the facial nerve and other cranial nerves was intact. Otorhinolaryngological findings were otherwise normal. An MRI scan revealed a tumour measuring 25 × 20 mm at the right jugular foramen with extension into middle ear and mastoid, causing involvement of the mastoid segment of the facial nerve and erosion of the posterior semicircular canal . The iniA 33-year-old woman was referred to our department due to recurrent LGFMS of the lateral skull base. She was first diagnosed with LGFMS in 2001 when the patient underwent partial tumour resection using the transtemporal approach with blind-sac closure of the external auditory canal without rerouting of the facial nerve in another hospital. The definite diagnosis of LGFMS was established based on postoperative histopathology and immunohistochemistry Figures . For theLGFMS most commonly presents as an intramuscular soft tissue mass of the proximal extremities or the trunk . Other mThere are three main challenges in the management of skull base tumours. First, the preoperative biopsy and histological diagnosis are usually impossible for skull base tumours. Therefore, the diagnosis is established based on clinical findings and radiographic imaging. Neither clinical signs nor radiological findings of the skull base lesions are specific. The lesions can present with various neuropathies of affected cranial nerves (CN) V–XII, tinnitus, hearing loss (HL), and disequilibrium. The location of the tumour, its consistency, infiltrative nature, and its extensions seen on radiologic images are also not diagnosis-specific. The main preoperative diagnosis in our first case was tympanojugular paraganglioma class C2 based on CT and MRI findings. However, typical “salt and pepper” appearance on precontrast T1-weighted sequences, a characteristic for paragangliomas, was not encountered . TherefoThe second challenge in management of skull base tumours is that en bloc resection is impossible in most of the cases. The latter could lead to potentially life-threatening complications due to risk for damaging vital neurovascular structures . FortunaThe third challenge is that the histological sample of LGFMS does not usually look malignant, so a special attention should be drawn to recognize a certain histopathological pattern of the tumour. LGFMS is usually relatively hypocellular, composed of uniform, bland short spindle cells, although occasional cases can show variations in cytomorphology and cellularity . Other rAs the tumour is lacking of atypical features, it has to be distinguished from benign lesions, such as benign nerve sheath neoplasms, perineurioma, myxoma, and fasciitis . DiffereIn our first case, the immunophenotype tests were negative for S100, EMA, SMA, CD34, and Bcl-2 and positive for MUC4 in the cytoplasm. In our second case, the immunophenotype of the sample tissue was negative for S100 and EMA, with focal positivity for MUC4. As both of our patients were young adults and had characteristic histopathologic findings and immunoprofile, the decision was made to diagnose LGFMS.In both of our cases, the patients received an adjuvant postoperative radiotherapy course. The decision was made in order to control either contaminated resection margins or residual tumour. However, it has been demonstrated that radiotherapy and chemotherapy do not affect the course of the disease with regard to subsequent recurrences, metastases, dedifferentiation, and life expectancy. The reason for low chemo- or radiosensitivity of the LGFMS is connected to its low grade of malignancy and low mitotic rate . HoweverThe lateral skull base is an extremely rare localization of LGFMS. To our best knowledge, we report the first two cases of such an entity. In light of rarity of such LGFMS localizations, each case requires individual treatment approach using different lateral skull base surgery methods. It is potentially life-threatening to perform en bloc resection in lateral skull base surgery approaches, and therefore, the residual tumour is oftentimes preserved in attempt to save important neurovascular structures and their function. Adjuvant radiotherapy may compensate for the difficulty in removing a safety margin of normal tissue in case of LGFMS of intracranial origin. The long-term follow-up is essential in case of LGFMS, as the disease often displays high rate of metastasis.


We report a case of stage T1b gallbladder carcinoma with concurrent hepatic anastomosing hemangioma managed by operative resection. We review the work-up and surgical management of this patient. We also discuss the relevant literature of both gallbladder cancer and hepatic anastomosing hemangioma, a recently described and rare variant of capillary hemangioma. The history of gallbladder carcinoma has been one of dismal prognosis with a short expected median survival despite advances in chemotherapy and surgical intervention . This haAn 80-year-old female with a past medical history of hypertension and diabetes mellitus, on oral medication, presented to a community hospital with postprandial right upper quadrant pain and dark stools. She was found to have mild thrombocytopenia. Computed tomography (CT) scan of the abdomen revealed cholelithiasis and an eccentric thickening of the medial wall of the gallbladder concerning for malignancy. There were a few esophageal varices seen; evidence of liver cirrhosis was present. There were no intraabdominal ascites. The patient had no history of alcohol abuse. She was referred to us for further care. Her blood work demonstrated a hemoglobin of 13.9, platelet count of 117,000, total bilirubin 1.4, international normalized ratio (INR) 1.3, and creatinine of 1.1. Her complete hepatitis serology was negative. Upon repeat imaging, her contrast-enhanced CT scan of the abdomen and pelvis revealed a 2 cm liver nodule in the left lateral sector with intense early arterial phase-contrast enhancement, with no early washout or fill-in typical of hepatoma or hemangioma, respectively  and a model for end‐stage liver disease (MELD) score of twelve. Operative intervention was decided on with the patient. Laparoscopic examination showed no carcinomatosis; however, it showed a nodular liver, and intraabdominal venous varicosities. Laparoscopic liver ultrasonography showed no additional liver masses aside from the known left liver lobe mass. Ultrasonography of the gallbladder showed a hyperechoic intraluminal mass with a hypoechoic rim of interface between it and the medial gallbladder wall. No evidence of invasion into the liver was seen grossly and the gallbladder wall thickness was normal. Therefore, a laparoscopic non-anatomic wedge resection of a portion of left lateral lobe containing the mass and a laparoscopic cholecystectomy were performed. During cholecystectomy, dissection of the gallbladder off the cystic plate was unremarkable and no evidence of transmural extension of tumor into cystic plate was encountered. The left lateral liver resection specimen and gallbladder were submitted for frozen section analysis Figure . This reAs a result of the malignant gallbladder diagnosis with no exact T-staging available on frozen section, a decision was made to perform non-anatomic resection of segments IVb and V en bloc with the cystic plate and hepatoduodenal lymphadenectomy. Therefore, a limited right subcostal incision was made and a non-anatomic resection of portions of segment IVb and V, en bloc with the cystic plate, was performed as well as a hepatoduodenal lymphadenectomy. Cystic duct margin, on frozen section, was negative for malignancy. Final pathology revealed a 2.7 cm T1b well-differentiated grade one papillary carcinoma of the gallbladder; no lymph nodes were found in the specimens Figures -6.The liver mass was determined to be an anastomosing hemangioma with sharp demarcation from the surrounding liver parenchyma, associated with a branching vascular pattern with mild nuclear atypia and absence of mitoses Figures -10. Postoperative recovery was uneventful and the patient was discharged on postoperative day five. She was doing well 18 months postoperatively with no evidence of recurrence.In this patient, it was not clear whether there was gallbladder malignancy on laparoscopic intraoperative ultrasonography. Although an intraluminal gallbladder hyperechoic mass was visualized, there was no associated gallbladder wall thickening. In addition, on intraoperative ultrasonography, there was a hypoechoic rim/interface all around the hyperechoic mass suggesting lack of origin from or invasion into the gallbladder wall. This guided the decision to perform laparoscopic cholecystectomy first. In addition, the hyperechoic mass in the gallbladder might have been inspissated sludge. Given the fact that there was no clear evidence of invasion on ultrasonography, a laparoscopic cholecystectomy was performed with a plan to analyze the hyperechoic mass by frozen section. Caution was taken to ensure no spillage of gallbladder contents occurred. Once malignancy was confirmed on frozen section, radical resection of portions of segments IVb and V of liver en bloc with the cystic plate and hepatoduodenal lymphadenectomy were performed as above. This was because the exact T-staging of the malignancy was not known at the time of surgery on a frozen section to guide the extent of resection.Gallbladder carcinoma is a highly aggressive malignancy that carries a poor prognosis. In 1994 a study by Cubertafond et al. -3. This There is an ongoing debate as to whether simple cholecystectomy alone is adequate, or radical resection with adjacent liver resection and regional lymphadenectomy is superior in managing T1b tumors. Those in favor of radical resection cite evidence of residual cancer being left in the resected liver bed in 46% of T1b tumors treated with prior cholecystectomy . Given tAlthough it remains controversial, the supportive rationale for selecting extended cholecystectomy relies on the evidence that the incidence of lymph node metastasis and recurrence rate are both significantly higher in T1b gallbladder cancer than T1a . There iWithout definitive evidence suggesting one approach is superior, it is up to the surgeon’s judgment and the results of frozen section analysis at the time of the procedure to guide decision-making in the management of early-stage gallbladder carcinoma. However, like in this patient, the exact T-staging of the tumor is often not available at the time of frozen section to guide the extent of resection. So it is wiser to err on the side of extended resection than simple cholecystectomy when an intraoperative diagnosis of gallbladder cancer is made. If a postoperative diagnosis of gallbladder cancer is made, then T-staging can guide decision-making on the need for reoperation for extended resection.The left hepatic vascular tumor was confirmed to be an anastomosing hemangioma after analysis by the second senior co-author (MSW) with the additional expert review by Dr. Andrew Folpe of the Mayo Clinic. Anastomosing hemangioma is a rare vascular tumor first described by Montgomery and Epstein in 2009 , with moThis patient’s early stage gallbladder carcinoma completely resected with negative margins improves her chance of five-year survivability . The NatThe surgical approach for stage T1b gallbladder carcinomas is controversial, with no definitive evidence demonstrating the superiority of extended radical cholecystectomy over routine laparoscopic cholecystectomy. Anastomosis hemangioma, a subtype of cavenous hemangioma, can be confused with angiosarcoma. However, anastomosing hemangioma has well-defined margins and appears sharply demarcated with mild cytologic atypia and lack mitotic figures, in contrast to angiosarcoma which displays prominent cytologic alterations and a diffuse infiltrating border.


Fibrous tumor of the stomach is a rare mesenchymal tumor composed of hyalinized fibrous tissue with spindle shaped fibroblasts. This type of tumor usually occurs in the subcutaneous tissues and the deep soft tissues. Its occurrence in stomach is extremely a rare finding and only few cases have been reported worldwide, the exact etiology is still unclear.A 68-year-old female was complaining from epigastric and right hypochondrial pain for few months. It was associated with attacks of non-bilious vomiting and early satiety, with attacks of melena. During abdominal examination there was a palpable mass in the epigastric region which was oval in shape, soft, mobile in the vertical direction, and was non-pulsatile. The hemoglobin was 9 gm/liter. Ultrasound and CT-scan of the abdomen showed a well-defined non-enhancing, multi-loculated mass related to the body of the stomach. Gastroscopy showed soft tissue mass with intact mucosa in the anterior gastric wall, multiple biopsies were taken which revealed fatty tissue with no evidence of malignancy.Laparotomy was done and a soft, mobile mass was found in the anterior gastric wall in the pre-pyloric region, the serosa was intact, complete excision of the mass was performed.The histopathological result showed fibrous tumors of the stomach.The treatment of this tumor is mainly through surgical intervention. Which should be done at the time of diagnosis. The prognosis after successful resection is excellent, few cases are reported with local recurrence. This tumor usually occurs in the subcutaneous tissues, the deep soft tissues, the subserosal tissues, and the gastrointestinal tract. Its occurrence in the gastric wall is an extremely rare finding and only few cases have been reported worldwide, the exact etiology is still unclear ,2.The tumor may be completely asymptomatic or may cause a variety of symptoms depending on its anatomical location. Gastric involvement may asymptomatic, or may present with epigastric pain, nausea, vomiting which may be bloody due to mucosal erosion, melena, iron deficiency anemia, pyloric obstruction. The characteristic histopathological feature is the presence of spindle cells that express factor XIIIa .The CT-scan findings of these tumors typically show well delineated outlines with lobulated appearance, occasionally there may be scattered calcifications inside the tumor .The cut surface of the tumor is pale brown or pale yellow in color, with cystic changes, the tumor may also contain stony hard nodules due to focal areas of calcifications .The biological behavior of the tumors located in the abdominal viscera is not clearly studied due to rarity of the tumor, and many cases are discovered on autopsy samples .Surgery is the principal option of treatment which can be done endoscopically, or by surgical resection .The work of this case report has been reported in line with the SCARE 2018 criteria .2A 68-year-old female was complaining from epigastric and right hypochondrial pain for few months, the pain was dull aching in type and poorly localized, coming in attacks and associated with meals, there was no radiation of the pain. There patient had no nausea or vomiting. The patient visited the hospital and the initial investigations and ultrasound of the abdomen showed multiple gall stones with no signs of inflammation. The pain was attributed to gallstones. The patient had diabetes mellitus which was controlled with oral antidiabetic drugs and dietary modifications, the past surgical history was negative.Laparoscopic cholecystectomy was done for the patient and the patient was discharged home after 2 days. One year after cholecystectomy the patient presented with epigastric pain which was increasing in intensity, and was associated with attacks of non-bilious vomiting and early satiety. The patient had 2 attacks of melena. The patient had no history of weight loss.The patient had a negative history for chronic drugs administration other than the oral hypoglycemic drugs. There was no family history for similar problems or for genetic abnormalities. The psychosocial history was non-relevant.2.1During abdominal examination there was a palpable mass in the epigastric region which was oval in shape, soft, mobile in the vertical direction, and was non-pulsatile. The patient had normal pulse, blood pressure, and temperature.The hemoglobin level was 9 gm/liter and the white blood cells and the inflammatory markers were normal.2.2Abdominal ultrasound and CT-scan of the abdomen showed a well-defined non-enhancing, multi-loculated lesion measuring 9 × 4 cm related to the body of the stomach, the lesion contained mixed fatty and soft tissue components .Fig. 1CTGastroscopy showed a soft tissue mass with an intact mucosa in the anterior gastric wall, biopsies were taken which revealed fatty tissue with no evidence of malignancy .Fig. 2An2.3Laparotomy was done through an upper midline incision, during surgery there was a soft, mobile mass which was related to the anterior gastric wall in the pre-pyloric region, the serosa was intact and there were no other masses in other parts of the bowel, and other organs .Fig. 3AnExcision of the mass was performed with a rim of 2 cm apparently healthy tissue; the mass was causing mucosal ulceration. The gastric wall was closed in 2 layers using a slowly absorbable suture material .Fig. 4AnThe histopathological examination of the mass was containing hypo-cellular myxoid areas with small blood vessels, spindle cell and keloid like pattern of collagen, no atypia or mitosis were detected. The tumor was strongly positive for vimentin and CD34, and negative for desmin, H-caldesmon, SMA, S-100 protein. These features were consistent with fibrous tumors of the stomach arising from the subserosal tissue plane .Fig. 5A The operation was performed by a general surgeon who is specialized in the field of gastrointestinal surgery.There were no any specific post-intervention considerations, but the patient was advised to avoid heavy meals for 2 months.2.4The patient was admitted in the hospital for 5 days, and was discharged with good general clinical conditions with no postoperative complications.3Solitary fibrous tumors are spindle cell tumors that predominantly occur in the pleura or the peritoneum, their occurrence in the stomach is an extremely rare finding. When these tumors arise from the pleura there are easily recognized but when arise in other anatomical locations, the diagnosis is very challenging .The tumor can affect any age group and in a literature review for the reported cases, the mean size of the tumor was around 3 cm and there was male predominance, all tumors were unifocal, and occurring in old ages ,4.The etiology of such tumors is not well studied because they are uncommon and no adequate data are available, but when the tumors occur in the pediatric age group they are usually attributed to an embryonic insult, genetic derangements or vascular insults .The differential diagnoses of such tumors include GISTs, leiomyoma, leiomyosarcoma, schwannoma, inflammatory fibrous polyps, desmoid tumors, inflammatory myoblastic tumor, plexiform fibromyxoma, and lipoma. The most important point is the differentiation of these tumors from gastrointestinal stromal tumors (GISTs), this can be done according to the differences in the attenuation during imaging, GISTs usually have a central lower attenuation due to central necrosis or hemorrhage, are relatively larger in size, have homogenous appearance, and have an exophytic growth when compared to fibrous tumors. The immunohistochemical studies and histopathological examination are the definitive tests for the differentiation ,4,7.The treatment of these tumors is mainly through surgical intervention. Surgical excision should be done at the time of diagnosis, surgical resection can be done adopting the open or the laparoscopic techniques, small size tumors are feasible for endoscopic resection. The prognosis of these tumor after successful resection is excellent in about 90% of the patients and long term follow up is not recommended in the majority of patients .The learning message in this case report is that although gall stones are commonly diagnosed, but many patients are non-symptomatic, the patient’s symptoms should be evaluated carefully and the character of the pain analyzed before attributing them to gall stones. Patients may present with more than one pathology at the same time.Patient’s perspective: I had the same attacks of pain before cholecystectomy and after it, I knew that there something going wrong in my abdomen until finally the doctors discovered that the problem is in my stomach.The author has no conflicts of interest to declare.None.Ethical approval has been exempted by my institution for reporting this case.Written informed consent was obtained from the patient for publication of this case report and accompanying images.The concept of reporting the case, data recording, and drafting the work done by Dr Wan Aldohuky and Dr Ayad Ahmad Mohammed.Dr Wan Aldohuky took the consent from the patient for publishing the case.Final approval of the work to be published was done by Dr Ayad Ahmad Mohammed.This work is case report and there is no need of registration.Dr Ayad Ahmad Mohammed is guarantor for the work.Not commissioned, externally peer-reviewed.


Xanthogranulomatous inflammation is a rare form of chronic inflammatory response consisting of macrophages, lymphocytes, plasma cells and neutrophils too. Due to its locally destructive nature and mass forming capacity as a result of adhesions, this type of inflammation may mimic malignancy or tuberculosis both clinically and radiologically. We present a report of two such cases, one mimicking tuberculosis and the other mimicking malignancy clinically. Awareness of this condition and a higher index of suspicion among clinicians, radiologists and pathologists can help in early diagnosis and more appropriate treatment of this potentially destructive disorder. Xanthogranulomatous inflammation is a well known histopathological entity. More commonly described in the gall bladder and kidney, this form of inflammation is uncommon in the fallopian tubes and ovaries. It is a relatively rare form of chronic inflammatory response that causes destruction of the affected organs. Dominated by the presence of lipid containing foamy histiocytes, there are sheets of mixed inflammatory infiltrates consisting of lymphocytes, plasma cells, neutrophils and multinucleated giant cells in the tissue parenchyma causing destruction . HoweverA 21 year old female presented to gynecology out patient department with complaints of inability to concieve for the last 5 years. Her menstrual history was within normal limits except for the last 5 months whe she developed irregular menstrual bleeding and dysmennorhea. There was no history of any chronic illness like tuberculosis in the past. She had undergone appendicectomy 11 years back for acute appendicitis. On general physical examination she had pallor and mild pedal edema. On per abdomen examination there was a lump in abdomen measuring approximately 8 X 8 centimeters in the left iliac fossa. Her per vaginum examination revealed bilateral tender fornices and a bulky uterus while per speculum examination showed healthy vagina and cervix. Patient was further investigated and on Ultrasonography was found to have left sided ovarian cyst measuring 5 X 4 cm, diagnosed as a complex cyst with septations. Uterus was found to be anteverted, bulky and with multiple fibroids both on anterior and posterior walls. Kidney also showed hydronephrotic changes. On Hysterosalpingography, bilateral tubes were blocked. Laparotomy was perfomed which revealed a large tubo-ovarian mass on left side measuring 6 X 7 cm. Omentum and bowel were found to be adherent to this mass. Along with a large 5 X 2 cm fibroid found in anterior wall of  uterus. Right sided falllopian tube was tortuous and edematous while right ovary was apparently normal. So a Left salpingo ophrectomy was performed and sent for histopathological examination. Pus drained from omentum was sent for culture and sensitivity. However it did not reveal any growth (including Mycobacterium tuberculosis) even after 4 weeks .Other investigation of the patient revealed CA 125 levels to be 246 U/ml and serum LH levels to be 7.58mIU/ml. Thyroid profile was normal.Histopathological examination: We received a mutilated specimen in three pieces. The largest piece measured 5×3×2 cm. Outer surface was exudate covered and congested. Cut surface revealed red brown areas with a luminal structure likely to be tube. Second and third pieces were measuring 4×3×1 cm and 1×1×0.5 cm. Sections from the ovary showed sheets of foamy histiocytes along with the presence of inflammatory infiltrates in the form of lymphocytes, plasma cells and some neutrophils and eosinophils. There were fair number of foamy histiocytes with abundant lipid laden vacoules in the cytoplasm and hypochromatic nuclei. There was fibrosis along with some vascular proliferation in the ovarian parenchyma. Sections from fallopian tubes also showed the presence of xanthogranulomatous inflammation in the lamina propria and the serosa of the tube walls . Inf. Inf3). The average age for this lesion is 38.5 years (range 23-72) and the youngest case reported was of 18 years . One of the most important differentials resembling xanthogranulomatous inflammation microscopically is malakoplakia where concentric calcific bodies (Michaelis- Gutmann bodies) are found in the cytoplasm, which were not seen in either of our cases. Sometimes neoplastic conditions like lymphoma or leukemia, malignant small cell tumor and sclerosing stromal tumor may also be considered in the list of differential diagnosis. However since we had a polymorphous population of inflammatory cells confirmed on immunohistochemistry by CD 68, CD3 and CD 20, we ruled out these possibilities .Thus cliAs done in our cases, surgery is the treatment of choice. But an Awareness of this inflammatory lesion among the clinicians, radiologists and pathologists may not only prevent overdiagnosis and extensive surgeries for the patients but may also reduce morbidity giving a better prognosis to these patients .Although the exact etiology of this locally desructive inflammatory lesion is unknown, we suggest that patients with PID, endometriosis, and IUCD etc. should be kept under follow up to prevent the occurrence of this disease. Awareness of this condition and a higher index of suspicion among clinicians, radiologists and pathologists can further help in early diagnosis and more appropriate treatment of this potentially destructive disorder.


Schwannomas of gastrointestinal tract are rare, mostly benign and notably different neoplasms from conventional schwannomas that arise in soft tissue or the central nervous system. These tumors are of clinical importance since they should always be considered in the differential diagnosis of submucosal lesions of gastrointestinal tract.Seven patients with a pathologically proven gastrointestinal schwannoma were identified in our series of mesenchymal tumors and reviewed retrospectively. Clinicopathological and immunohistochemical parameters along with the follow-up results were analysed.n = 5), the tumor was an incidental finding during other medical, imaging or surgical procedures. The tumors were located in the stomach (n = 4) and in the small intestine (n = 3) with an average size of 29 mm . A preoperative diagnosis was achieved only in one case with a CT-guided core biopsy. Otherwise the clinical, intraoperative, endoscopic or radiological findings were unspecific. Patients with gastric tumor underwent either laparoscopic (n = 2) or open (n = 2) gastric wedge resection of the tumor; in the cases of intestinal tumor (n = 3) a segmentectomy was performed. Pathological examination revealed solid homogenous tumors, which were highly cellular and composed of spindle cells with positive staining for S100 protein, and confirmed the diagnosis of schwannoma. All tumors were negative for c-Kit, smooth muscle actin, desmin and DOG-1 and showed very low proliferation index. There were negative resection margins and no malignant variants were recognized. At an average follow-up of 60 months  all patients were free of disease with no signs of recurrence or metastases and acceptable gastrointestinal function.The series included two males and five females, with a mean age 69 years . Most patients were asymptomatic on presentation, except for two patients with abdominal pain. In the other cases (Schwannomas are rare, slow-growing and mostly asymptomatic gastrointestinal mesenchymal tumors. They are difficult to be diagnosed preoperatively as endoscopic and radiological findings are nonspecific but histological and immunohistochemical features are of paramount importance to differentiate between benign and malignant schwannomas, or other spindle cell sarcomas. The treatment of choice is complete surgical excision without a conclusive preoperative diagnosis, and the long-term outcome is excellent as these lesions are mostly benign. Schwannomas are slow-growing, homogeneous, mostly benign tumors arising from the schwann cells of the nerve sheath , 2. TheySchwannomas of the GI tract, firstly presented by Daimaru et al. in 1988 , are usuThe purpose of our study was the description of clinical, histopathological and immunohistochemical features of GI schwannomas, providing long-term follow-up and confirming the benign nature of these tumors.Seven patients with histologically identified GI schwannoma during a 10 year period, were involved in this retrospective study. Clinical, histopathological, immunohistochemical and surgical data were reviewed. A complete surgical excision of the tumors was performed in all patients. Specimens were obtained from all patients. The diagnosis of GI schwannomas was based on histopathological analysis accompanied by immunohistochemical staining performed for S-100 protein, glial fibrillary acidic protein (GFAP), c-kit (CD117), CD34, discovered on GIST-1 (DOG-1), smooth muscle actin (SMA) and desmin. The patients were regularly followed up on an outpatient basis. Follow-up included physical examination, blood counts, serum chemistries, endoscopy and computed tomography (CT)-scan. Median follow-up was 60 months .n = 4) and in the small intestine in three patients (n = 3), with a median tumor size of 29 mm . Out of the four gastric schwannomas, two were localized in the gastric body and two in the antrum. Symptoms were reported in only one patient with gastric schwannoma who complained of persistent epigastric pain and nausea. In six cases the tumor was an incidental finding during imaging, endoscopic or surgical procedures for other reason. Particularly the tumors were incidentally discovered during surgery for colon carcinoma (case Nr. 1) and diverticulitis of sigmoid , during an abdominal ultrasound as preventative measure (case Nr. 2) and during a upper GI gastroscopy performed as preventative measure as well (case Nr. 5). Furthermore, in the seventh patient, the tumor was an incidental finding on a thoracic CT scan performed for evaluation of hemoptysis. CT scan revealed a round homogeneous mass with contrast enhancement and a predominantly exophytic pattern arising from the lesser curvature of stomach  underwent a gastric wedge resection by using either an open (n = 2) or a laparoscopic approach (n = 2)  received a small bowel segmentectomy . The median tumor size was 26.5 mm . Neither morbidity nor mortality was observed in our series.All patients underwent a tumor resection with negative resection margins (R0). Patients with gastric schwannomas ( 2) Fig. . In bothThe histopathologic diagnosis of schwannoma was confirmed in all patients. Pathological examination demonstrated the GI schwannomas to be solid homogenous tumors, which were highly cellular and composed of spindle cells. No areas of cystic change of gross necrosis were reported in any of the cases. Mitotic activity was very low (< 5/50 high power fields). All specimens demonstrated strong positive immunostaining for S-100 and GFAP proteins, whereas the tumor cells were not immunoreactive to DOG1, c-Kit (CD117), CD34, SMA and desmin Fig. .Fig. 2ReMedian follow-up was 60 months . During the follow-up period no recurrence was detected. Furthermore no excessive body weight loss and no gastrointestinal symptoms such as dyspepsia and bloating were observed.Gastrointestinal schwannomas are rare mesenchymal tumors –7, firstIn terms of pathologic evaluation, GI schwannomas are classified as non-epithelial tumours, and represent a separate, homogenous entity, distinct from leiomyomas, leiomyosarcomas, gastrointestinal autonomic nerve tumors (GANTs) and GISTs , 14, 15.Our series shows a remarkable regularity regarding to the characteristics of GI-schwannomas. They occur more frequently in the sixth decade of life and there is a predominance of females , 17–21. A preoperative diagnosis of GI schwannomas is generally difficult and challenging, if not impossible, since the tumor have no specific clinical symptoms and no diagnostic modality can show any pathognomonic features unique to this tumor . On CT eGI-schwannomas are supposed to arise from the myenteric plexus of the GI wall because of their immunophenotypic similarities; both schwannomas and myenteric plexus cells express S-100 protein and GFAP , 11. PatThe pathologic findings of the GI schwannomas in the present analysis were consistent with the previously described findings. On immunohistochemistry, vimentin, GFAP and S100 protein are diffusely and strongly expressed by the cells of GI schwannomas ; the S10The main differential diagnosis for an exophytic lesion arising in the wall of the GI tract is a GIST, as it is the most common mesenchymal tumor located in GI tract , 31, 32.Schwannomas of the GI tract are generally benign since previous follow-up studies have not identified any malignant variants , 19–21. Schwannomas are rare, slow-growing and mostly asymptomatic gastrointestinal mesenchymal tumors, notably different from conventional soft-tissue and central nervous system schwannomas. They present a challenge in GI tract surgical oncology due to difficulty in the preoperative diagnosis, as the clinical, endoscopic and imaging findings are nonspecific. The definitive diagnosis can only be established through the histopathological and immunohistochemical examination, which is confirmatory. Complete surgical excision is the gold standard in the treatment of schwannomas and the prognosis of patients is excellent since these lesions are mostly benign.


Alveolar soft part sarcoma (ASPS) is an extremely rare neoplasm that tends to occur in the lower limbs of children and adolescents. Metastatic breast tumors constitute 0.5–2.0% of all malignant mammary neoplasms, and cases of ASPS with mammary metastases are very rare.Three years ago, an 11-year-old girl presented to the hospital with pain in the right jaw after becoming aware of a mass in the right cheek. After detailed examination, the patient was diagnosed with ASPS with the primary tumor in the right cheek and multiple lung metastases, and chemotherapeutic treatment was initiated. One year later, accumulation of fluorodeoxyglucose (FDG) was observed in the right front of the skull -max 2.8) and left breast (SUV-max 2.4) using FDG-positron emission tomography (PET) / computed tomography (CT). Ultrasonography revealed the mammary tumor as a hypoechoic, internally heterogeneous mass measuring 22.4 × 16.2 × 21.1 mm with a rich blood supply. Using pathological findings of core-needle biopsy, we diagnosed it as ASPS. Based on the above information, we made a diagnosis of ASPS with left mammary and cranial metastases. Due to chemoresistance, surgical excision was selected as the mode of treatment; resection of the metastatic cranial bone was performed first, and partial mastectomy of the left breast was performed in two stages. Postoperative conditions were good, and we are currently performing regular follow-ups .We have reported an extremely rare case of ASPS with mammary metastasis with some reference-based discussion. In our case, disease control was obtained by a combination of drug therapy and surgical treatment. Alveolar soft part sarcoma (ASPS) is an extremely rare neoplasm that tends to occur in the lower limbs of children and adolescents and was first described by Christopherson et al. in 1952 , 4, and 2]), the patient developed grade 4 neutropenia. After this treatment, the patient received 1 cycle of the treatment regimen prescribed for rhabdomyosarcoma  and 1 cycle of ifosfamide (1800 mg/m2), etoposide (100 mg/m2), actinomycin D (0.045 mg/kg), and vincristine (2 mg); however, the development of severe neutropenia made it difficult to continue administration of these drugs. The patient was then treated with oral administration of 800 mg/day of pazopanib for 1 year, and clinical benefit was achieved. Upon stabilization of the disease, oral administration of pazopanib was discontinued; however, 1 year later, fluorodeoxyglucose accumulation was observed in the right front of the skull  (Fig. Three years ago, an 11-year-old girl presented to the hospital with pain in the right jaw after becoming aware of a mass in her right cheek. After detailed examination, the patient was diagnosed with ASPS with primary tumor in the right cheek and multiple lung metastases, and chemotherapeutic treatment was initiated. After receiving 1 cycle of VAC therapy (vincristine [2 mg], actinomycin D [0.045 mg/kg], and cyclophosphamide [1.2 g/mAn elastic, soft tumor, approximately 3 cm in size, was palpated in the lower lateral region of the left breast. Ultrasonography revealed a hypoechoic, internally heterogeneous mass measuring 22.4 × 16.2 × 21.1 mm with a rich blood supply Fig. a, b. HisMetastatic tumors of the breast are rare and account for approximately 0.5–2.0% of all malignant mammary tumors ; howeverConversely, ASPS is rare and accounts for 0.5–1.0% of soft-tissue sarcomas; it commonly occurs in young women. In adults, it is often found in the lower limbs, while it commonly occurs in the head and neck in children , 10. At Surgical excision is most commonly used for treatment because of chemotherapeutic resistance , 15. In We reported an extremely rare case of ASPS with mammary metastasis with some reference-based discussion. In our case, disease control was obtained by a combination of drug therapy and surgical treatment.


Collagenous colitis (CC) is a clinicopathologic syndrome characterized by chronic watery diarrhea and distinctive histopathologic features. Spontaneous perforation of CC is extremely rare, because CC is usually managed medically, and the need for surgical intervention is rare. We report a surgical case of spontaneous colonic perforation of CC with acute abdomen disease.A 77-year-old man was admitted to our hospital for abdominal pain and watery diarrhea. Computed tomography (CT) showed a thickened bowel wall with edema involving free air around the splenic flexure of the colon. Therefore, we performed emergency surgery with a diagnosis of colonic perforation. Intraoperative findings revealed colonic necrosis at the splenic flexure, so we performed a left hemicolectomy. Histopathological examination revealed typical findings of CC, a thick subepithelial collagenous band and deep ulcers with perforation. The postoperative course was uneventful, and the patient was discharged on the 28th postoperative day. After changing the proton pump inhibitor (PPI) from lansoprazole (LPZ) to rabeprazole (RPZ), he has not complained of diarrhea symptoms.Although spontaneous perforation is a rare complication of CC, it is possible to be diagnosed by symptom of acute abdomen disease. This is the seventh case of spontaneous colonic perforation of CC worldwide. Collagenous colitis (CC) is characterized by chronic diarrhea and usually occurs in middle-aged women. Endoscopic examinations are generally unremarkable , laboratEndoscopic evaluation of the colon is often normal, but erythema, edema, changes in blood vessels, and mucosal clefts may be observed . DefinitCC is a heterogeneous disorder and may have multiple etiologies making pathogenesis unclear. Several medications have been implicated as contributing factors, notably aspirin and other non-steroidal anti-inflammatory drugs (NSAIDs), histamine 2 receptor blockers, and proton pump inhibitors, such as lansoprazole and certain selective serotonin reuptake inhibitors . Autoimmbody temperature was within the normal range. Physical examination revealed acute left abdominal pain and muscular defense. Laboratory results revealed a white blood cell count of 2100/μl , and C-reactive protein (CRP) level was 0.19 mg/dl . Computed tomography (CT) showed a thickened bowel wall with edema involving free air around the colonic splenic flexure, and ascites was found on the liver surface  Hospital complaining of abdominal pain and frequent episodes of non-bloody watery diarrhea, lasting for 2 months. His past medical history included a gastric ulcer 40 years earlier, hypertension, and chemotherapy for multiple myeloma. His current medications were aspirin, prednisolone, melphalan, and lansoprazole (LPZ). His Postoperative course was uneventful, and the patient was discharged on the 28th postoperative day. PPI-induced CC was suspected due to his past history; therefore, the PPI was subsequently changed from LPZ to RPZ. Following this change, he noted an improvement in diarrhea symptoms.CC is a relatively uncommon, but increasingly diagnosed form of microscopic colitis. CC was described in 2 independent reports in 1976 from Canada and Sweden . PatientOnly 6 patients have been reported to have a spontaneous perforation in CC Table  9, 10, –15. In aAlthough the precise mechanism is unknown for the cause of CC, previous articles report that CC is caused by genetic factors , 24, intProton pumps exist in colonic epithelial cells. Therefore, a proton pump inhibitor is presumed to change the composition and pH values of secretions from colonic mucosa and affect the immune response, which is a possible mechanism in developing CC . LPZ is In summary, we report a case of CC triggered by a PPI. It is important to note that spontaneous perforation of CC is a possible complication of diagnosed acute abdomen disease.


Intrapericardial teratomas are rare primary cardiac tumors. These tumors, albeit benign in essence, can be fatal if they exert pressure on the cardiovascular and/or respiratory system. We describe a 34-day-old infant, who needed emergent surgery due to cardiovascular structure compromise. Proper anesthetic and surgical techniques conferred an uneventful postoperative course. Histologic examination confirmed the tumor as an intrapericardial teratoma. At 8 months’ postoperative follow-up, the child had a good developmental status and a normal echocardiogram.  The ability to detect cardiac tumors noninvasively has led to a clear increase in the diagnosis.5, 6 With an embryonic origin, teratomas are composed of elements derived from the 3 germinal layers in varying degrees. If at least 50% of the tumor is comprised of well-differentiated elements, the tumor is referred to as a mature teratoma.3 These tumors are usually benign, and their clinical signs and symptoms depend on the tumor size and location.7 They may be diagnosed during fetal life (often in the anterior mediastinum), and they are usually associated with pericardial effusion and hydrops fetalis, or soon after birth with severe cardiorespiratory system compromise.6, 8Primary tumors of the heart in infants and children are rare, with an incidence rate of 0.0017% to 0.28%.A 34-day-old female infant, weighing 2.3 kg, was admitted to our institute with severe respiratory distress. Physical examination demonstrated severe tachypnea (respiratory rate = 35-40/min), sweating during breastfeeding, peripheral cyanosis, and indrawing of the chest cavity with inspiration. Chest X-ray revealed only cardiomegaly . Transth2) before induction was 88% and after the administration of 100% oxygen with a mask, SpO2 rose to 94%. Considering the infant’s age, severe respiratory distress, and cardiovascular instability, we performed awake-sedate intubation after the administration of 5 μg of fentanyl and topicalization of the tongue, larynx, and vocal cords with 15 mg of 1.0% lidocaine and a 3.0-mm Portex tracheal tube. Subsequently, the general anesthesia was maintained with a mixture of oxygen/air, isoflurane, 1-mg increments of atracurium, and 5 μg of fentanyl intermittently. Thereafter, an arterial line and a central venous line were established. Other monitoring modalities such as the bispectral index (BIS), capnograph, and pulse oximetry were utilized as well. Extracorporeal circulation was on standby. Via a median sternotomy with the preservation of the thymus gland, the pericardium was opened and a large quantity of cloudy yellow fluid was aspirated. A multicystic lesion, around 50 × 45 × 30 mm in size, was revealed. It covered most of the right ventricle and pressed on the right atrium  is relatively common. In our patient, although the teratoma was anchored to the ascending aorta and thus caused pressure on the right atrium, we achieved its complete removal. The postoperative period was uneventful, with the patient being extubated on the first postoperative day in the intensive care unit and then discharged home on the fifth postoperative day. Microscopic examination of the specimen yielded a definitive diagnosis. The surgical removal of such tumors is not only curative but also lifesaving because failure to achieve a prompt removal of these lesions can prove fatal. Our patient’s prognosis was good insofar as at 8 months’ postoperative follow-up, she had a good developmental status and a normal echocardiogram.Intrapericardial teratomas are rare; they should, however, be included in the differential diagnosis of a newborn or infant presenting with respiratory distress. Intrapericardial teratomas should be removed as soon as they are detected because, despite their inherent benignity, they can be potentially fatal.


The diagnosis of deep intestinal endometriosis is mandatory to plan treatment and for follow-up; however, there is no consensus worldwide in the use of rectal/ vaginal opacification and anti-peristaltic agents for magnetic resonance imaging (MRI) scanning, being defined as an option for the examination. The transvaginal ultrasound images of previous MRI with the standard protocol, and recent MRI in our institution with rectal/vaginal opacification with water and the anti-peristaltic agent are presented in four cases for comparison, respectively. The technique in our institution seems to be more effective than routine pelvic MRI scans in the intestinal endometriosis. Transvaginal ultrasound (TVUS) is the first-line method and magnetic resonance imaging (MRI) should be considered as a second-line technique after TVUS. However, the MRI diagnosis of DIE is a dilemma in radiology departments because most MRI scans with suspicion of DIE are negative in spite of either an intestinal endometriotic nodule in TVUS or the clinical history being in favor of DIE. There is no universally accepted protocol for MRI in endometriosis for the use of vaginal and/or rectal opacification (RVO). In the presented cases, which were initially diagnosed using TVUS as intestinal endometriosis, the findings of MRI scans with and without RVO with water and anti-peristaltic agent use are presented.Deep infiltrating endometriosis (DIE) is involvement of the retrocervical septum, rectovaginal septum, uterosacral ligaments, vaginal fornix, and bladder restriction of uterus mobility or pain with probe compression;  kissing ovaries;  unilateral or bilateral ovarian endometrioma;  intestinal wall thickening; and  intestinal endometriotic nodule either as the mushroom cap sign or the Indian headdress sign.The 53 patients with TVUS findings in favor of deep pelvic endometriosis were collected between 2016 and 2018. The criterion for patient selection was the presence of at least three of the following TVUS findings: (3). Written informed consent was obtained from all subjects, according to the World Medical Association Declaration of Helsinki, revised in 2000, Edinburgh. The histories and the clinical information of the 4 patients are obtained as suggested by the IDEA group and these were noted for each patient respectively  was used for the MRI re-scans anda Toshiba Applio 500 ultrasound system was used for transvaginal examinations . The patients were informed to use a rectal enema 12 and 2 hours before the MRI exams for distal bowel cleansing. If the urinary bladder filling was inadequate in the survey image, 200 mL of saline infusion was administered through a Foley catheter. Isotonic saline solution was used for vaginal and RVO through the Foley or Nelatone urinary catheter. The vaginal filling with saline infusion was stopped when the patient started to feel overflow. The total amount of fluid used for rectal filling was between 500 mL and 1000 mL. After the intravenous administration of the single-dose (20 mg) American Psychological Association (APA) , 100-200 mL of additional fluid for bowel filling was given. The urinary bladder (if needed), vaginal and rectal saline infusions were easily performed with a simple and cheap system .2D T2W sagittal/axial/coronal, 2D T1W sagittal/axial with and without fat saturation, T1W Dixon sagittal, and diffusion-weighted sequences were performed. Gadolinium is not used for intravenous or in saline solution for contrast opacification. If there was an interval more than 3 months between the previous and the present MRI scan, initial T2W sagittal images were obtained just before applying the RVO with water and the APA in order to exclude recent nodule growth. The findings in TVUS, the previous MRI scans, and the re-scanned MRI examinations are presented as images, respectively. The endometriotic nodules in the bowel wall are demonstrated clearly in all patient re-scans, which had been reported as normal previously , 5, 6.. Mostly the lesions are in the chronic phase, which is why the bright intestinal lumen is mandatory in order to visualize the fibrotic nodules, especially for inexperienced examiners. However, there is no consensus for RVO with water and APA use, which is published to be an option in the medical literature,7. In daily practice, MRI scans are performed without these options. Here, it is clearly seen that MRI with RVO with water and APA use is more appropriate than routine MRI scans for intestinal DIE. Administration of the optional procedures requires an extra 10 to 15 minutes, so it might be not preferred in some radiology departments. In Turkey, public hospitals are autonomized to allow them to out-source some medical services such as diagnostic imaging, and the less time required for MRI scanning means more income for the out-source services. Therefore, the use of the optional technique should be restricted by choosing patients with DIE before the MRI scans using TVUS. In our experience, the diagnosis of intestinal involvement in endometriosis using MRI needs RVO with water and APA use; therefore, it should not be an option in MRI scans as in the guidelines, but an obligation in patients with endometriosis with intestinal involvement in order to increase the detectability.The MRI examinations are crucial for the proper diagnosis and for the demonstration of the extent of the lesions preoperatively, for postoperative follow-up or for the efficacy of medical treatment. The MRI findings of endometriotic nodules in the intestinal wall vary depending on the progression of the infiltration. On T1W sequences, the lesions are hyperintense in early phases due to bleeding and hypointense in the chronic phases due to fibrosis


Isolated enteric duplication cyst is an intestinal duplication cyst found in a distant location from the intestinal tract and it is said to have its own blood supply. Meckel’s diverticulm is considered as an antimesenteric structure and has its own blood supply. However, there are some reported cases of Meckele’s diverticum in the mesenteric side. Ectopic pancreas may be found in both entities.A 5-year-old girl presented with increasing abdominal pain around the umbilicus. On laboratory investigation serum pancreatic enzymes and C-reactive protein were elevated. Abdominal computed tomography (CT) revealed a normal pancreas but a cystic lesion in the mesentery of the ileum. A nodule with a marked enhancement was observed in the wall of the lesion. During the laparoscopy, the lesion was found at the root of the mesentery and was distant from the ileum. The lesion was resected suspecting an abscess. Pathologically, the wall of the lesion consisted of small bowel like tissue, and pancreatic tissue was seen beneath the mucosa. There were some post inflammatory changes in the pancreatic tissue. Retrospectively on thin slice enhanced CT, an independent blood supply was noted. Based on these findings, a diagnosis of ectopic pancreatitis in an iliac intestinal duplication cyst was made.Isolated enteric duplication cyst in the root of ileal mesentery and mesenteric Meckel’s diverticulum have similarities. In the present case, the diagnosis of isolated enteric duplication cyst was made since it was found distant from the ileum. It is important to consider the possibility of ectopic pancreatitis when serum pancreatic enzymes are elevated even when the pancreas appears normal. Enteric duplication is a rare anomaly, which is found along the gastrointestinal tract and generally shares the same blood supply as the nearby intestine . It has A 5-year-old girl with no past medical or surgical history had complained of waxing and waning pain around the umbilicus for a few years. On several occasions, she had visited her general practitioner due to abdominal pain, but no abnormalities had been noted. The pain recurred with greater severity, and abdominal ultrasound performed by her general practitioner revealed a cystic lesion in the right lower abdominal cavity. An abdominal abscess was suspected thus she was referred to a tertiary hospital.On admission, the vital signs were as follows: blood pressure, 125/81 mmHg; pulse, 166 beats per minute; and temperature; 36.5 °C. On palpation, rebound tenderness was noted on the right iliac fossa. On laboratory investigation, the white blood cell count was 17,400/μL and the C-reactive protein level was 1.54 mg/dl. There was an elevation in the serum pancreatic amylase and lipase level . Contrast enhanced abdominopelvic computed tomography (CT) (Fig. An abscess in the mesentery was suspected, and the patient was put on nil by mouth and treated with antibiotics. There were significant improvements in the symptoms and the laboratory data on the second week and she was discharged on the fifth week. Follow-up enhanced CT at 3 months showed a significant decrease in size of the cystic mass (Fig. Pathologically, the wall of the lesion assimilated the small bowel wall with villi in the mucosa and under the mucosa there was pancreatic tissue consisted of acini, ducts and islets of Langerhans Fig. . The panEnteric duplication is a rare gastrointestinal anomaly with an incidence of 2 in 9000 among postmortem newborn infants and fetuses . It can Some cases of enteric duplication are not anatomically associated with the gastrointestinal tract and are designated as isolated enteric duplication cysts . In contIn the present case, the wall of the cystic lesion consisted of small bowel wall without sharing the smooth muscle with the nearby ileum and it had its own blood supply. Nonetheless its location was at a feasible distance from the ilioceolovalve for Meckel’s diverticulum and pancreatic tissue is observed more frequently in Meckel’s diverticulum, it was distant from the small intestine siting at the root of the mesentery almost in the retroperitoneum. These findings support the diagnosis of an isolated enteric duplication cyst.We have experienced a case of ectopic pancreatitis in an isolated intestinal duplication. It had a distinguished blood supply and Mesenteric Meckel’s diverticulum was considered for the differential diagnosis. It was located at the root of the mesentery where the lesion was distant from the border of the ileum, thus the diagnosis of ectopic pancreatitis in an isolated intestinal duplication was made. It is important to consider ectopic pancreatitis when serum pancreatic enzymes are elevated even when the pancreas appears normal.


Ischemic colitis (IC) results from reduced colonic vascular perfusion, accounting for 50-60% of all gastrointestinal ischemic episodes. IC leads to mucosal damage with clinical symptom severity developing based on the duration and extent of colonic injury. In rare cases IC may form a mass-like lesion mimicking malignancy. Here we present the case of a 55-year-old female with hematochezia and diarrhea, who on workup was found to have a mass-like lesion at the ileocecal valve. Multiple biopsies demonstrated ischemic change and mucosal injury without evidence of dysplasia or carcinoma. Two months later on follow-up imaging, after supportive treatment the lesion was completely resolved. It is critical for gastroenterologists and pathologists to be aware of this variant of IC to avoid unnecessary surgical procedures and treatment of patients. Ischemic colitis (IC) is a common vascular disorder of the intestines, usually affecting older patients . IC resuA 55-year-old female with past medical history significant only for hypothyroidism presented to the ED with hematochezia and diarrhea of unknown etiology. Her last colonoscopy, three years prior, was unremarkable. On colonoscopy, a mass-like lesion was identified at the ileocecal valve . NotablyThree months prior to this ED visit she was treated with steroids for presumed autoimmune hepatitis following a workup and admission for painless jaundice, decreased appetite, right upper quadrant pain, “mustard yellow” urine and pale stools, elevated transaminases, and hyperbilirubinemia. An ultrasound was unrevealing, and CT showed no intrahepatic or extrahepatic bile duct dilatation, with a contracted gallbladder with pericholecystic edema of uncertain etiology. There was a nonspecific periportal edema extending towards the porta hepatis and a short segment jejunojejunal intussusception. Pathology of her liver biopsy showed an acute hepatitis pattern of injury with mild lobular cholestasis. The precise etiology was not readily apparent with the differential diagnosis including acute viral hepatitis and drug-induced liver injury. Plasma cells were inconspicuous, and the patient reportedly had negative F-actin serology; as a result, autoimmune hepatitis was not favored, though it could not be completely ruled out. The patient underwent a repeat colonoscopy two months later, following supportive treatment for her symptoms, although she endorsed additional episodes of diarrhea. The procedure revealed complete resolution of the mass-like lesion at the ileocecal valve with no signs of colitis . After tClues to the diagnosis of this clinicopathologic variant of IC include acute onset, rapidly changing course, elderly female patient, location in the right colon, ischemic colitis on histology after thorough sampling, and discordant imaging and colonoscopy findings . Histolo


A 16-year-old female soccer player presented with an eight-month-old, insidious right-hip pain. The imaging workup demonstrated a cortically based lytic lesion at the level of the lesser trochanter with surrounding sclerosis, adjacent periosteal reaction, and a small soft-tissue mass. A biopsy revealed findings related to an osteoid-forming lesion with features of nuclear atypia. The patient's pathology referral returned a diagnosis of osteoid osteoma, and she was treated with CT-guided cryoablation. She had an excellent response and returned to full activities through nearly two years of surveillance. In similar patients approaching skeletal maturity, the differential diagnosis for bone lesions involving the lesser trochanter ranges from post-traumatic to primary oncologic processes. Given the implications of a missed malignancy, vigilance is required when treating young and active patients. Osteoid osteoma is a common benign bone lesion found in adolescents. It accounts for 10-12% of benign bone tumors and usually affects the long bones of the lower extremity, with two-thirds of the femoral lesions occurring in the pertrochanteric region . MultiplIn skeletally immature athletes, apophyseal avulsions of the anterior superior iliac spine (ASIS), anterior inferior iliac spine (AIIS), ischial tuberosity, or lesser trochanter can result from sudden, forceful contractions of originating muscle groups , 9-12. TIn adolescent athletes, an osteoid osteoma at the lesser trochanter may mimic an apophyseal avulsion fracture or an osteogenic sarcoma. We present the case of a 16-year-old female with such a presentation and discuss the diagnostic challenges. The patient and her family have given their consent for the publication of this case.A 16-year-old female presented with an eight-month old right-hip pain of insidious onset. She was an avid high-school soccer player but was unable to attribute the pain to a specific incident. She had initially consulted her primary care physician (PCP) after two months of symptoms and had been prescribed a 10-week course of physical therapy. When her symptoms failed to resolve and the pain was noted to further localize to the hip and buttock, plain radiographs were obtained. They demonstrated a cortically based lytic lesion at the level of the lesser trochanter with surrounding sclerosis and adjacent periosteal reaction (Figure Advanced imaging was then ordered, and the patient was referred to a local orthopedic surgeon. After evaluation, her care was transferred to an orthopedic oncologist. On presentation, her pain was noted to be worse at night and slightly alleviated by naproxen. She had pain with resisted hip flexion, but her exam was otherwise benign. An MRI with intravenous contrast was obtained Figure , along wAlthough the patient’s history and exam were suggestive of a benign or post-traumatic etiology, the findings of an adjacent periosteal reaction on plain radiographs Figure  and a soInitial review of the biopsy demonstrated findings regarding an osteoid-forming lesion with features of nuclear atypia Figure .Lesions in the differential included osteoid osteoma, osteoblastoma, osteosarcoma, and callus reaction from an avulsion fracture. These findings were corroborated by a consulting pathologist. The pathologist's opinion favored fracture and close follow-up or a repeat biopsy was recommended. A third opinion was sought from a national pathology referral center. Finally, combining history, radiographic differential, and histologic appearance, the diagnosis of osteoid osteoma was made. The patient was treated with CT-guided cryoablation Figure .She recovered from this procedure with a resolution of her symptoms and has returned to participation in high-school athletics. Surveillance radiographs and MRI were obtained after five months and 21 months following her ablation, and they all demonstrated resolution of the adjacent periosteal edema, bone marrow edema, and iliopsoas bursitis with restoration of the cortex Figure .The proximal femur is a common site for the development of an osteoid osteoma. In patients approaching skeletal maturity, the differential for lesions involving the lesser trochanter ranges from post-traumatic to primary oncologic processes. Despite a history suggestive of a benign process in the case under discussion, the imaging workup was equivocal. And the results of the biopsy only further confounded the treatment team as features of nuclear atypia, hypercellularity, and disorganized growth could be seen in both fracture callus and osteogenic malignancies . Osteoid osteoma is most frequently seen in adolescents and young adults. Most cases were reported in patients between the ages of 5-24 years , 4-5. WiIn skeletally immature athletes, the physis or apophysis is weaker than the adjacent attachments of ligaments and tendons. As such, injuries that might cause sprains, strains, or ruptures of ligaments or tendons in adults often lead to physeal or apophyseal injuries in children . Due to This difficult case required clinical correlation, an expert opinion from an experienced pathologist, and an interdisciplinary team including orthopedic oncology and musculoskeletal radiology to ensure proper diagnosis and treatment. Given the profound implications of a misdiagnosis, vigilance is required when treating young and active patients.


Introduction: Here we present a 75-year-old patient who was admitted with acute-onset right-sided hemiparesis, dysphagia, dysarthria and nystagmus. Repeated MRI scans showed two lesions with contact to one another: one solid stationary extra-axial lesion at the caudal part of the clivus and a rapidly growing intra-axial cystic lesion at the level of the medulla oblongata. Biopsy of the solid lesion demonstrated a low-grade chondrosarcoma, while no tissue sample of the cystic lesion could be retrieved. After initiation of dexamethasone therapy the cystic lesion markedly regressed.Background: A literature search on published cases with the same combination of a stationary solid extra-axial mass at the caudal part of the clivus and a growing intra-axial cystic mass in the medulla oblongata was negative, indicating that the case described here is both unique and novel.Discussion: Considering the rapid progression of symptoms and growth on MR-imaging in combination with the marked response to steroids, an inflammatory response linked to the chondrosarcoma is most likely. At the same time other possible explanations as a second neoplasm, an abscess or an ischemic lesion seem unlikely.Concluding remarks: This case underlines an unusual complication of a rare brainstem tumor and outlines both the differential diagnosis and potential treatment options. For such cystic lesions in combination with chondrosarcoma, a treatment course with steroids should be considered along with surgical exploration necessary to obtain the diagnosis and for potential reduction of mass-effect on the medulla oblongata. A 75-year-old womanwas admitted to another hospital with progressive right-sided hemiparesis for 24 h. She had no headache, nausea, vomiting, or dizziness. On examination, slight facial asymmetry and mild-to-moderate right-sided hemiparesis were noted. A non-contrast CT-scan  showed no signs of intracranial hemorrhage, ischemia or mass lesions. On the next day, an MRI of the brain was obtained. Besides moderate-to-severe chronic vascular leukoencephalopathy a cystic, T2-hyperintense intra-axial lesion Figure  in the mOn physical examination, the patient had slightly anisocoric pupils (right>left) equally reactive to light, saccadic smooth pursuit and hypometric saccades, gaze-evoked nystagmus, upbeat-nystagmus, right-sided ptosis, right-sided weakness of the buccal branch of the facial nerve, dysarthria, dysphagia, uvula, and tongue deviation to the left and moderate-to-severe right-sided hemiparesis with a positive Babinski sign. A lumbar puncture showed 6 cells/μl  and slightly elevated protein levels . The increased cell count and the elevated protein level were suggestive of an inflammatory response and therefore dexamethasone was started. Repeated MRI day 10) showed peripheral contrast-enhancement of the cystic lesion (25 × 11 × 9 mm) and progressive perifocal edema  was completed 6 months after the diagnosis was made and since then no further disease progression was noted.No ethical approval was obtained as this was a single case report. Written informed consent for publication of this case report was retrieved from the patient.We performed a literature search on published cases with the same combination of a stationary solid extra-axial mass at the caudal part of the clivus and a growing intra-axial cystic mass in the medulla oblongata. However, to our best knowledge, we could not identify comparable cases previously described in the literature. As the differential diagnosis of the case presented here is the main focus of this manuscript, we will address previously published cases with certain similarities in the Discussion section.This 75-year-old patient presented with rapidly progressive hemiparesis, nystagmus, dysphagia, and dysarthria due to a growing intra-axial cystic mass in the medulla oblongata as well as a stationary solid extra-axial mass at the caudal part of the clivus identified as chondrosarcoma. Based on a recently published case series of isolated medullary lesions, we categorized the intra-axial lesion as suggested by Prakkamakul et al. . PresentThis includes a second, independent intra-axial neoplasm. Since the cystic lesion responded to dexamethasone and with absent clinical and laboratory features of systemic lymphoma, the first suspicion was lymphoma of the central nervous system (PCNSL). PCNSL are a rare form of lymphoma and very rarely located in the brainstem. They usually show homogenous contrast-enhancement, but ring-like contrast-enhancement is possible . This diDue to the cystic appearance, an endodermal cyst, abscess or hemangioblastoma should be included in the differential diagnosis. Endodermal cysts are usually located extra-axially in the posterior fossa , whereasThe rapid onset would be consistent with an underlying vascular pathology such as brainstem ischemia or hemorrhage . Yet, the progressive clinical course and MR-imaging do not support this etiology. With the intra-axial lesion being hyperintense on T2 rather than hypointense  and being negative on SWI-weighted imaging, this speaks against a vascular pathology. Furthermore, such a vascular lesion would not have resolved by administration of dexamethasone or surgical decompression of the medulla , 8.Alternatively, the cystic lesion could have been caused by an inflammatory process as the cerebrospinal fluid cell count was slightly elevated and as the patient improved clinically and radiologically on dexamethasone. The inflammatory process could have been caused by compression of the medulla oblongata due to the mass effect of the solid clival lesion. This hypothesis is supported by the fact, that the cystic lesion regressed after surgical reduction of the mass effect on the medulla oblongata. However, regression already started before the surgical intervention. Therefore, rather than pressure, induction of inflammatory processes by the chondrosarcoma itself must be considered as well. Even though unusual, tumor-associated infiltration of inflammatory cells has been linked to chondrosarcomas , 10. FurIn conclusion, in this 75-year-old patient with a cystic intramedullary mass and a clival chondrosarcoma causing symptoms of the lower cranial nerves and hemiparesis, the most plausible etiology of the cystic lesion is an inflammatory process as for several reasons two distinct neoplasms or underlying vascular pathologies seem highly unlikely. For such cystic lesions in combination with chondrosarcoma, a treatment course with steroids should be considered along with surgical exploration necessary to obtain the diagnosis and for potential reduction of mass-effect on the medulla oblongata.JW was involved in the conceptualization of the case report, the clinical assessment of the patient, drafted and critically reviewed the manuscript. SK was involved in the clinical assessment of the patient and critically reviewed and edited the manuscript for content. NK performed the surgery, contributed the intraoperative images and critically reviewed and edited the manuscript for content. AT was involved in the conceptualization of the case report, the clinical assessment of the patient and critically reviewed and edited the manuscript for content. All authors read and approved the final version of the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


We report a rare case of a woman with optic disk melanocytoma (ODMC) in conjunction with polypoidal choroidal vasculopathy (PCV). We also present, for the first time in literature, the clinical and morphological outcomes of the applied treatment, consisting of a session of photodynamic therapy (PDT) and three monthly intravitreal aflibercept injections.An 83-year-old Greek woman, complaining for visual decline at her left eye, referred to our department and was diagnosed with ODMC associated with PCV. At presentation, best corrected visual acuity (BCVA) was 2/10, fundus examination revealed a pigmented lesion covering partially the optic nerve head and extending into the peripapillary choroid and retina, while hard exudates were observed temporal to it. Blocked hypofluorescence in the area covered by the lesion and diffuse hyperfluorescence at its temporal rim were shown by fluorescein angiography (FA). Indocyanine green angiography (ICGA) identified 3 hyperfluorescent polypoidal lesions arising from the choroidal vasculature. Optical coherence tomography (OCT) revealed subretinal fluid and retinal pigment epithelium detachment (RPE) at the region corresponding to polyps. The treatment included a PDT session combined with 3 monthly intravitreal aflibercept injections. Three months since the treatment initiation, new BCVA was 5/10, ICGA demonstrated total polyps occlusion, while OCT detected RPE detachment without subretinal fluid. Ten months later, ODMC was stable, BCVA rose to 7/10, no polyps were present, and total resolution of RPE detachment was achieved.This is the first case report of PCV coexisting with ODMC, presenting both ICGA and OCT findings, and the applied treatment and its outcomes. Furthermore, we demonstrated that PDT combined with intravitreal aflibercept injections seems to be a promising treatment for PCV. Optic disc melanocytoma (ODMC) is a rare ophthalmic tumor arising from melanocytes, and is considered to be a variant of the melanocytic nevus . ODMC isPolypoidal choroidal vasculopathy (PCV), first described by Yannuzzi et al. , is a clIn this report we describe a case of a woman with ODMC along with PCV. To the best of our knowledge it has been previously described only by Bartlett et al. and El-Haddad et al. , 11. HowAn 83-year-old Greek woman, without any prior significant medical history, referred to the outpatient department of the 2nd Department of Ophthalmology of the Medical School of Athens, complaining for visual deterioration at her left eye (OS). At presentation she underwent a complete ophthalmological examination. Best corrected visual acuity (BCVA) was 10/10 in her right eye (OD) and 2/10 in OS. Slit lamp examination did not identify any abnormalities in the anterior segments in both eyes. Her intraocular pressure was normal bilaterally.Funduscopy and fundus photography revealed an unremarkable retina in OD. However, in OS a dark brown lesion, with “fuzzy” borders was identified, which covered partially the optic nerve head and extended temporal into the adjacent choroid and retina Fig. . FurtherFluorescein angiography (FA) Fig.  and ICGAThe pigmented lesion was shown in optical coherence tomography (OCT)  as a gradually sloped nodular elevation, with hyperreflective anterior surface and dense posterior shadowing. OCT also revealed subretinal fluid, RPE detachment, and a round protrusion attached beneath the posterior surface of the detached RPE, at the site corresponding to polypoidal lesions in ICGA Figs.  and 3.FiA diagnosis of PCV associated with ODMC was made.2 body surface area), its irradiation was performed using an ocular photoactivation diode and a laser-linked slit lamp. The treatment spot diameter, based on the ICGA findings, was approximately 1500 μm  in combination with 3 monthly intravitreal aflibercept injections. PDT was performed according to the standard TAP guidelines . Fifteen μm Fig.  and it wPDT session was performed 7 days after the first intravitreal aflibercept injection. One and two months later, the several injections were applied.Three months after the treatment initiation, significant improvement was observed. Patient’s new BCVA was 5/10, while ICGA demonstrated total polyp regression Fig. . In OCT Thirteen months since the beginning of the treatment, the patient was re-examined. ODMC remained stable, her BCVA rose to 7/10, no polyps were detected, while total resolution of RPE detachment was achieved Fig. .Fig. 6FuThis is the first report in literature presenting a rare clinical case of ODMC coexisting with PCV, in which the applied treatment and its clinical outcomes are described, and the findings of SPECTRALIS OCT, which depicts the clinical lesions with more accuracy, are provided.Several therapeutic strategies have been applied in PCV treatment. PDT has been proven to be effective in inducing polyps occlusion and reducing exudative phenomena in PCV eyes, improving thus visual acuity –15. HoweAflibercept is a recombinant fusion protein, binding to all isomers of vascular endothelial growth factor (VEGF)-A family, to VEGF-B and to placental growth factor , 19. ItsOur treatment strategy, consisting of PDT along with three intravitreal aflibercept injections, seems to be effective, since our patient experienced significant vision and anatomical gains. Polyps closed completely, their recurrence was prevented in a thirteen-month period, while resolution of RPE detachment occurred.As for ODMC, no changes were detected during the 12-month follow up. ODMC is regarded to be a benign and stable lesion, while only 1–2% of the cases undergo a malignant transformation . In the To the best of our knowledge, no previously published data describe a case of PCV coexisting with ODMC, displaying both ICGA and OCT findings. Furthermore, this is also the first report in literature that any form of treatment is applied for PCV associated with ODMC. Last but not least, we present the combination of PDT and intravitreal aflibercept injections as a promising treatment for PCV.


Beckwith-Wiedemann syndrome (BWS) is a rare congenital overgrowth disorder. A major feature is lateralized overgrowth, which can variably involve a single body district up to the entire hemisome. Visceral asymmetrical involvement has been observed, commonly represented by enlargement of one kidney or adrenal gland, rather than one gonad.We report the case of a pubertal boy affected by BWS, who developed a progressive testicular enlargement, ipsilateral to the pre-existing external body overgrowth. Asymptomatic unilateral testis enlargement started after regular pubertal onset and worsened over time, without any associated pathological findings in a long-term follow-up. Since biopsy is not indicated in case of benign macro-orchidism, we hypothesize that this asymmetric enlargement could be an expression of visceral lateralized overgrowth in BWS.At the best of our knowledge, this is the first detailed report of unilateral testicular overgrowth in BWS. We revised common causes of painless unilateral scrotal masses in the pediatric age. Considering both the overall frequency of neoplasia and the malignancies predisposition in BWS, a testicular cancer should be carefully ruled out through a close follow-up, before stating a benign condition. A normal ultrasound pattern, together with normal serum hormonal levels and negative tumor markers, make testicular neoplasms highly unlikely. Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth disorder with heterogeneous genetic background and variable phenotypic expression, more recently defined as Beckwith-Wiedemann Spectrum (BWSp) . The conA boy affected by BWS has been followed in our Pediatric Endocrinology Unit to monitor growth and pubertal development. Given the neonatal findings of macrosomia (birthweight >2SDS), transient hyperinsulinemic hypoglycemia and umbilical ‘hernia permagna’ , a diagnosis of BWS was hypothesized and further confirmed by molecular analysis, that revealed a mosaic paternal isodisomy of chromosome 11p15. Family history was unremarkable. Over the years, linear growth assessed on the 90th centile, with an increasingly evident right-sided overgrowth of the trunk and limbs . No embryonal tumors occurred. Pubertal development started regularly at 11 years of age with bilateral testicular volume of 5 ml, associated with phallus enlargement. At the age of 13 years, a slight testicular asymmetry was noticed. At 14 years testicular asymmetry was prominent: clinically estimated testicular volume was 18 ml on the right and 12 ml on the left side. The bigger testis appeared of normal consistency, without intra or paratesticular nodular masses. Scrotal transillumination was opaque. The boy was asymptomatic and denied traumas. Growth rate velocity was consistent with normal pubertal spurt.Testicular ultrasound confirmed the clinical asymmetry  and showed a regular echostructure of both testes and epididymides, excluding focal lesions. Abdomen ultrasound was negative for alterations. Laboratory studies, including complete blood count, lactic dehydrogenase, serum tumor markers , testicular, adrenal and thyroid hormones were repeatedly normal. In the absence of clinical signs of malignancy, a watch and wait approach was preferred to a biopsy. Asymmetry slightly increased during the follow-up: at 15 years of age, right testis measured 54.5 × 20.2 × 34.3 mm by ultrasound with an estimated volume of 19.7 ml and left testis measured 46.2 × 18.6 × 29.1 mm with an estimated volume of 13.3 ml  , this coAs the history of our patient was negative for cryptorchidism and testicular torsion, compensatory hypertrophy was excluded. On the other hand, by considering that asymmetric overgrowth has initiated at pubertal time and given the oncogenic risk in BWS, especially in case of chromosome 11p15 UPD [Without a biopsy, pathological findings cannot be assessed and an accurate final diagnosis cannot be made, but considering genetic predisposition and pre-existing body asymmetry, we cannot exclude that this event was an expression of lateralized visceral overgrowth in BWS.In conclusion, we report an uncommon case of progressive asymptomatic testis enlargement in an adolescent affected by BWS: it occurred slowly and asymptomatically, within a regular pubertal development and consensually to a pre-existing body asymmetry. In a long term follow-up, a completely normal ultrasound examination together with persistent negative evaluation for tumor markers strongly suggest a benign condition. A conservative approach may be acceptable if a strict follow-up is available, considering that this syndromic disorder is associated with an increased risk of tumors.


T1 weighted images and hyperintense on short tau inversion-recovery images. It demonstrated homogeneous contrast enhancement. The lesion was biopsied, and histopathology revealed an intraosseous hibernoma composed of brown fat cells. Intraosseous hibernomas are rare and demonstrate non-specific imaging findings requiring biopsy for diagnosis, although most hibernomas are incidental and asymptomatic. Initial treatment with microwave ablation and cementoplasty improved the patient’s symptoms temporarily but cementoplasty caused radicular symptoms, and eventually, cement removal, bone curettage, grafting and sacral nerve root decompression were required for symptom remission.We report the imaging findings and histopathology of a rare case of sacral hibernoma in a female presenting with right buttock pain while sitting. The lesion was occult on radiographs and CT scan. A small, rounded right S2 lesion was hypointense on  T1 weighted images, hyperintense on T2 weighted and short tau inversion-recovery images (A 36-year-old female presented complaining of months of worsening back and right buttock pain exacerbated by sitting. She did not respond to initial management with conservative therapy of non-steroidal anti-inflammatory medications. Radiographs of the pelvis were unremarkable. Subsequently, MRI of the lumbar spine was performed and showed a partially visible sacral lesion. Later, MRI of the pelvis demonstrated a rounded, non-destructive lesion involving the right sacrum at the S2 level. The sacral lesion was hypointense on y images  and enhay images . CT imagy images  did not T2 hyperintensity and enhancement. In the initial evaluation of our case, haemangioma was the suspected diagnosis. An intraosseous hibernoma may also be considered as a possible entity, albeit rare, with T1 weighted hypointensity, T2 weighted hyperintensity and homogeneous enhancement. Other possible aetiologies include skeletal metastases, particularly if these are 18-fludeoxyglucose avid on positron emission tomography/CT scan, although these are not expected to be radiographically occult.1 One recently recognized entity capable of exhibiting a similar intraosseous appearance within the sacrum is a benign notochordal cell tumour; these benign lesions may lead to chordoma and present similar to our case as radiographically occult lesions with low T1 weighted signal and high T2 weighted signal and may exhibit sclerosis on CT scan.The differential diagnosis for this sacral lesion includes osseous haemangioma, generally with CT-guided biopsy was performed by correlating MRI findings with bony landmarks . Gross pT1 and T2 signal. There was no clear evidence of residual tumour or recurrence.Initially, the patient’s sacral lesion was treated with microwave ablation and cementoplasty with initial symptom relief. A follow-up MRI of the pelvis  demonstrApproximately 10 months after initial ablation and cementoplasty, the patient developed worsening pain in the right buttock radiating into medial and posterior thigh with associated numbness. These radicular symptoms with numbness were different from the patients initial presentation with greater severity. This pain was attributed to encroachment of cement and treatment-related changes within the right S3 nerve root suggested by earlier post-operative imaging . SurgeryT1 weighted hypointense, T2 weighted hyperintense and homogeneous enhancement properties. Biopsy suggested an intraosseous hibernoma. Hibernoma is a rare, benign soft-tissue tumour composed of brown fat most often manifesting as subcutaneous soft-tissue tumours involving the thigh, shoulder, back, neck, chest, arm and retroperitoneum.4 They typically occur as slow-growing masses that are mostly asymptomatic. This case of probable sacral intraosseous hibernoma adds to the handful of reported intraosseous cases.We describe a unique case of a symptomatic sacral intraosseous hibernoma that was occult on radiographs and CT scan. This right S2 lesion had a non-specific MRI appearance with T1 weighted hypointensity, T2 weighted hyperintensity and homogenous enhancement, which is consistent with previously described intraosseous hibernomas. Nevertheless, hibernoma within a bone is rare and other conditions may demonstrate a similar imaging appearance. Interestingly, all reported cases of intraosseous hibernoma with CT or radiographic examination have demonstrated sclerosis.1, we suggest broadening the differential diagnosis of intraosseous hibernoma to include non-sclerotic osseous lesions. It may be that our case was a developing hibernoma that had not yet elicited a sclerotic reaction, especially as our patient was much younger than the middle-aged and elderly patients more commonly reported. Cases of unusual imaging appearance such as ours require histopathological confirmation from bone biopsy to ensure appropriate diagnosis and treatment. Soft-tissue hibernoma arising from brown fat is a rare entity in itself and intraosseous hibernoma is even more rare, with only 14 cases documented previously.1The sacral lesion in this case demonstrated a non-specific appearance with 13 The pathology of intraosseous hibernoma in our case is consistent with that of others, suggesting a common pattern of benign, neoplastic proliferation of brown fat cells in a manner similar to more common, yet still infrequent, soft-tissue hibernomas.9 Since our biopsy sample was small and was obtained under CT guidance from an area where the lesion was occult, it could be argued that the specimen could reflect incidental residual brown fat in marrow adjacent to the lesion as opposed to a space-occupying mass composed of brown fat or hibernoma. The biopsy and ablation site on CT scan correspond to the initial lesion site on MRI, indicating that the original biopsy was obtained properly and represented a sample of the space-occupying mass, thereby supporting the diagnosis of intraosseous hibernoma.The exact aetiology and mechanism by which hibernomas occur is unclear. It appears that most cases are incidental and asymptomatic. Symptomatic presentation is more common where lesions compress adjacent anatomical structures such as nerve roots, although the aetiology of intraosseous hibernoma-related pain can be unclear. In our case, the aetiology of pain is difficult to establish in the absence of an expansile lesion but may be similar to that seen in bone metastases such as increased intraosseous pressure, periosteal stretching or microfracture.1 were incidentally discovered on imaging studies for other pathologies such as cancer staging CT examinations. Only 2 of the 14 previously reported cases presented with back or buttock pain similar to our patient.9 demonstrated a similar presentation with an initial lumbar spine MRI ordered for evaluation of low back and buttock pain, which demonstrated a lesion within the right ilium. Another case of sacral intraosseous hibernoma presented with lower back pain radiating down the ipsilateral foot.11 CT-guided radiofrequency ablation was performed in this case with complete pain relief at 9 months.11Treatment of intraosseous hibernoma is not established owing to its rarity and generally asymptomatic, benign natural course. All of the five intraosseous hibernoma cases reported by Bonar et alOur case highlights the difficulties encountered in treating intraosseous hibernoma. No standardized treatment method exists, and symptomatic cases may benefit from ablation or surgical excision. Management approaches range from conservative methods of analgesic medication and steroid injections to ablation and surgical excision. In our case, microwave ablation with cementoplasty initially provided symptomatic relief, but then pain recurred with worsening symptoms, including paresthesias and numbness in a S3 distribution likely because of compressive effects of cementoplasty on the nerve root. Surgical removal of the bone cement and decompression of the S1–S3 nerve roots resulted in improvement of symptoms. Treatment should be tailored to the lesion location and symptomatic status of each patient on an individual basis.Intraosseous hibernoma is a rare benign brown fat tumour that is generally asymptomatic but may cause symptoms owing to compressive effects on local structures.T1 weighted hypointense, T2 weighted hyperintense enhancing bone lesion may represent metastases, haemangioma, benign notochordal tumour or intraosseous hibernoma.A This case is unique as the lesion was occult on initial radiographs and CT scan, whereas most cases of intraosseous hibernoma are reported to be sclerotic.


Tumefactive Multiple Sclerosis (TMS) is a rare variant with 1 per 1000 cases of MS and 3 per million cases per year. TMS can mimic clinical and radiological features of a neoplasm, infarction, or abscess and therefore can be diagnostically challenging for clinicians. We present a clinical scenario of a patient presenting with left homonymous hemianopia with atypical radiological features initially thought to be more consistent with neoplasm or infraction. Ultimately, biopsy was done which led to the diagnosis of tumefactive multiple sclerosis. Multiple Sclerosis (MS) is a chronic, inflammatory demyelinating disease of the central nervous system, with a relapsing and remitting quality . TumefacA 29-year-old female with no medical history presented to the hospital for acute left-sided vision loss. Associated symptoms included photophobia, floaters, and bifrontal headache. On examination, she had left homonymous hemianopia, but no other neurologic deficits. Computed tomography revealed acute ischemia involving the right parieto-occipital lobe with vasogenic edema. Magnetic resonance imaging  revealedThis is a unique case of TMS manifesting as homonymous hemianopia. Studies have shown only 10% of patients with TMS present with visual deficits. Diagnosis of TMS is difficult but should be strongly considered in patients with neurologic deficits and supportive imaging findings. These include mass greater than 2 centimeters, vasogenic edema, ring enhancement, restricted diffusion, and T2 hypointensity. Typically, patients follow a relapsing-remitting course although studies have shown that a mass greater than 5 centimeters, as seen in our patient, is a poor prognostic factor.In a large study of 168 patients with biopsy confirmed diagnosis of MS, radiographical and clinical information was gathered and patient characteristics, clinical course, MRI findings, and prognosis were evaluated. The ratio of female: male was 1.2:1, median age of onset was 37 years, duration between symptom onset to biopsy was 7.1 weeks, and total disease duration was 3.9 years. From clinical course prior to biopsy, this was first neurological event in 61% of patients, relapse remitting presentation in 29%, and progressive in 4% , 11. Fin