¹8F-fluorodeoxyglucose PET/CT features and correlations with histopathologic characteristics in sclerosing epithelioid fibrosarcoma.

Sclerosing epithelioid fibrosarcoma (SEF) is a clinicopathologically distinct variant of fibrosarcoma that is capable of recurrence and metastasis. Awareness of imaging features and histopathologic characteristics will be helpful for differential diagnosis from other common tumors. Here, we report a case of SEF metastasizing to the pancreas as a solitary mass mimicking primary pancreatic cancer, and summarize the reported cases with FDG PET/CT from the literature (n=4). PET/CT showed abnormal FDG accumulation (n=2), mild FDG uptake (n=1), or photopenic (n=1). The FDG PET/CT features are closely related to histopathologic characteristics regarding its differentiation and aggressiveness.

Primary sclerosing epithelioid fibrosarcoma of bone: analysis of a series.

Sclerosing epithelioid fibrosarcoma (SEF) is a rare, aggressive malignant neoplasm characterized by small nests and linear arrays of epithelioid cells embedded in a dense collagenous matrix. Very few primary SEFs of bone have been reported. Recognition is critical, as the dense extracellular collagenous matrix can be interpreted as osteoid, leading to misdiagnosis as-osteosarcoma. MUC4 and SATB2 are 2 recently characterized immunohistochemical markers for SEF and osteosarcoma, respectively. In reports to date, osteosarcomas are positive for SATB2 and negative for MUC4, whereas soft tissue SEFs have shown the opposite immunohistochemical profile (SATB2-/MUC4+). The purpose of this study was to characterize the clinicopathologic and immunohistochemical features of 8 primary SEFs of bone. The patients presented at a wide range of ages (25 to 73 y; median 52 y). Tumors mostly involved long bones of the extremities, with 3 cases involving the femur, 2 involving the ulna, and 1 involving the humerus. Other sites of involvement included the second rib (1) and the C6 vertebra (1). Follow-up information was available for 7 patients, 3 of whom developed metastases within 2 years of diagnosis. The other 4 patients were free of local recurrence or metastases at 1, 5, 12, and >84 months of follow-up, respectively. Radiographically, the tumors were predominantly lytic and poorly marginated. Histologically, 6 tumors showed pure SEF morphology, and 2 showed hybrid SEF/low-grade fibromyxoid sarcoma morphology. Focal dystrophic mineralization was seen in 1 case but was limited to areas of necrosis. None of the tumors showed the lace-like pattern of mineralization typical of osteosarcoma. The majority (6/8) of the tumors strongly expressed MUC4. SATB2 was negative in all but 1 case, which showed variable weak to moderate staining in ∼50% of nuclei. In general, the combination of morphology, MUC4 expression, and the absence of SATB2 expression was highly useful in arriving at the correct diagnosis.

Imaging appearance of renal epithelioid angiomyolipomas.

OBJECTIVE: This study aimed to describe the computed tomographic (CT) imaging appearance of renal epithelioid angiomyolipomas (eAMLs). METHODS: The CT scans and electronic medical records of 8 patients with histologically confirmed eAMLs identified by biopsy and/or surgical excision who had available imaging performed between 1995 and 2012 were reviewed. Preoperative CT imaging appearance, histologic features, and clinical follow-up were recorded for each patient. RESULTS: Macroscopic fat was identified in 3 (38%) of 8 eAMLs on preoperative CT imaging. Seven of the eAMLs demonstrated postcontrast enhancement of greater than 20 Hounsfield units. None of the eAMLs showed evidence of local invasion, vascular involvement, or distant metastases on the initial preoperative CT; however, 1 patient developed local recurrence and another developed distant metastatic disease on follow-up imaging. CONCLUSIONS: Epithelioid angiomyolipomas may or may not demonstrate macroscopic fat. Those with macroscopic fat do not possess any CT imaging characteristics that allow them to be distinguished from typical angiomyolipomas. Epithelioid angiomyolipomas without macroscopic fat are indistinguishable from renal cancers.

Synchronous pure epithelioid angiomyolipoma of the kidney and retroperitoneal schwannoma in the same patient on 18F-FDG PET/CT imaging.

A 21-year-old woman was admitted to our hospital after incidental detection of a left renal lesion on ultrasound scan. Abdominal contrast-enhanced CT scan revealed a moderately enhanced lesion in the left kidney and a round mass with slight enhancement in the retroperitoneum. FDG PET/CT imaging was performed for whole-body survey, showing increased tracer accumulation in both the left renal and the retroperitoneal lesions. Left renal cell carcinoma with retroperitoneal metastasis was suspected. Synchronous pure epithelioid angiomyolipoma of the left kidney and retroperitoneal schwannoma were diagnosed by pathology. This case highlights that epithelioid angiomyolipoma should be considered as one of the differential diagnosis in renal lesions with increased FDG uptake.

Endoscopic ultrasound fine needle aspiration biopsy of celiac ganglia.

OBJECTIVE: Celiac ganglia are normal anatomic structures located in the region of the celiac artery and can vary in size from 5 mm to 4.5 cm. These nodules can be visualized by endoscopic ultrasound (EUS) and be subject to fine needle aspiration (FNA) biopsy. They can be identified in patients with and without underlying pathology and present a challenge in accurate interpretation. STUDY DESIGN: The hospital database was retrospectively searched for patients who had undergone EUS FNA and where a diagnosis of 'ganglia' was rendered. Clinical and demographic data were collected and the cases reviewed for cytomorphologic features. RESULTS: Aspirate smears were generally hypocellular with variable cellularity between aspirate passes, showing dispersed single large epithelioid cells with cytoplasmic pigmentation, small groups of bland stroma and spindled cells, as well as 'grape-like' groups with peripheral epithelioid cells and central stroma with spindled elements. CONCLUSION: EUS cannot always definitively identify celiac lesions and the use of EUS FNA can help to accurately classify these cases. In patients with known or suspected malignancy, the accurate identification of celiac ganglia by EUS FNA can have important patient management implications. EUS FNA can identify celiac ganglia and show the distinctive features of large pigmented epithelioid ganglion cells and background neural elements.

CT imaging and histopathological features of renal epithelioid angiomyolipomas.

AIM: To describe computed tomography (CT) imaging and histopathological manifestations of renal epithelioid angiomyolipomas (EAMLs) for better understanding and cognition in the diagnosis of this new category of renal tumours. MATERIALS AND METHODS: Clinical data and CT images from 10 cases of EAML were retrospectively analysed. All patients underwent CT with and without contrast medium administration, with multiplanar reconstruction (MPR) when needed. RESULTS: Plain CT manifestations of EAMLs were a higher density of mass (10-25 HU) than renal parenchyma, bulging contour of the involved kidney, absence of fat, distinct edges without a lobulate appearance. Contrast-enhanced CT features were markedly heterogeneous enhancement (from rapid wash-in to slow wash-out), large tumour size without lobular appearance, complete capsule with distinct margins and frequent mild necrotic areas. Histopathological features were epithelioid cells with eosinophilic cytoplasm, large and deeply stained nuclei, and dense arrangement of tumour cells with patchy necrosis; diffuse sheets of epithelioid cells were positive for HMB-45 (melanoma-associated antigen) and negative for epithelial membrane antigen (EMA) staining. CONCLUSION: Multiple specific CT features correlated well with the histopathology and may play an important role in the primary diagnosis of EAMLs.

Intratumoral blood flow analysis in epithelioid trophoblastic tumors.

OBJECTIVE: The purpose of this series was to describe the transvaginal color pulsed Doppler sonographic features of epithelioid trophoblastic tumors (ETTs) and to evaluate whether there were specific sonographic criteria to accurately distinguish them from other lesions. METHODS: Seven cases of ETTs treated in the Women's Hospital of Zhejiang University were retrospectively analyzed. Doppler indices, including the Pourcelot resistive index (RI), pulsatility index (PI), and peak systolic to diastolic velocity (S/D) ratio from blood flow signals within the tumors were calculated from each waveform sample by using the software of the ultrasound machines. RESULTS: Patients with ETTs had heterogeneously echoic masses and highly abnormal flow patterns. The mean PI, RI, and S/D ratio for the patients were 0.57 (range, 0.22-1.09), 0.42 (range, 0.2-0.7), and 1.89 (range, 1.25-3.40), respectively. CONCLUSIONS: The clinical usefulness of intratumoral blood flow assessment in ETTs is yet to be established. However, the multiparameter sonographic approach can help in diagnosis of an ETT.

Primary gastric lymphoma with florid granulomatous reaction.

Epithelioid cell granulomas are more commonly seen in Hodgkin's disease and T cell lymphomas. Rarely florid granulomatous reaction with necrosis may be a prominent feature in lymphoma. To the best of our knowledge, a total of 11 cases of Burkitt's lymphoma with florid necrotizing granulomas have been reported in the English literature. None of these cases have previously had the stomach involved. Here we report a gastric Burkitt's lymphoma with florid granulomatous reaction diagnosed following a partial gastrectomy. The initial gastric biopsy showed granulomatous gastritis but the radiological and endoscopic appearance was that of a gastric stromal tumour. We conclude that in the presence of a mass lesion, the finding of epithelioid granulomas should warrant re-biopsy to establish an accurate diagnosis and exclude a concurrent malignant process. Hence, major surgery and postoperative complications can be avoided and appropriate treatment regimen can be initiated.

Epithelioid hemangiomas of bone: spontaneous clinical and radiographic remission.

A case of pathologic multifocal epithelioid hemangioma which occurred in the perigestational period is reported. This is the only reported case in which an epithelioid hemangioma, without treatment, went into radiographic and clinical remission. This case may suggest an alternative treatment for epithelioid hemangioma. Rather than radiation therapy or amputation, close observation of this benign neoplasm may be indicated.

Epithelioid angiosarcoma of the splenic capsule. Report of a case reiterating the concept of inert foreign body tumorigenesis.

We report an epithelioid angiosarcoma involving the splenic capsule. This neoplasm developed because of a gauze sponge, retained for 38 years following left-sided nephrectomy. Clinical, radiological, and histological features of this angiosarcoma add to the validity of the concept of inert foreign body tumorigenesis in humans.

Epithelioid variant of malignant peripheral nerve sheath tumor (malignant schwannoma) of the urinary bladder.

Sarcoma represents less than 2% of all neoplasms diagnosed or recognized in effusions. Epithelioid peripheral nerve sheath tumor is a rare tumor that is difficult to differentiate from other epithelioid tumors without the use of ancillary studies. A 39-year-old paraplegic man presented with hematuria and a bladder mass that extended to involve the pelvic peritoneum. Light microscopy using hematoxylin-eosin, Papanicolaou, and immunohistochemical stains as well as transmission electron microscopy showed features of epithelioid malignant peripheral nerve sheath tumor with rhabdoid features and an accompanying eosinophilic infiltrate. Cytologic smears confirmed the similarities between the primary tumor in the bladder and the cells in the pelvic fluid and excluded the possibility of reactive changes related to postsurgical radiation. Ancillary studies were critical in narrowing the differential diagnoses and reaching the final conclusion.

Diagnostic significance of epithelioid granulomas in Crohn's disease in children. Multicenter Paediatric Crohn's Disease Study Group.

Out of 528 children with Crohn's disease in a Multicenter Paediatric Crohn's Disease Study Group, 37 cases had epithelioid granulomas but did not fulfill defined radiographic criteria of the disease. Follow-up studies including clinical, biochemical, radiological, endoscopic, and histological investigations were done in these patients. Initially, all patients showed clinical symptoms and 27 of them had biochemical signs of chronic inflammation. After a mean follow-up of 3 years, all 37 children treated for Crohn's disease got a complete upper gastrointestinal series with small bowel followthrough and 8 children in addition had barium enemas. Colonoscopies were done in 23 patients. Radiographic examination revealed Crohn's disease in 14 and endoscopy additionally confirmed Crohn's disease in 8 further cases. One child was diagnosed as having chronic granulomatous disease. Thirteen children still remained unclassified after these follow-up studies including radiographs and endoscopy. An interval of 3 years may in some cases be too short to express the complete radiographic pattern of Crohn's disease. Our studies demonstrate that in addition to initial radiological, endoscopic, and histological investigations, a thorough follow-up is necessary in early diagnosed patients. In these children, epithelioid granulomas are of high diagnostic validity preceding radiological changes of Crohn's disease often for years.