Cases reported "Retroperitoneal Neoplasms"

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1/19. Primary pure intratesticular fibrosarcoma.

    Testicular sarcoma appears to be a rare stromal tumor usually of indolent course with potential for distant metastases. A pure primary intratesticular fibrosarcoma in a 71-year-old male is presented. By the time the tumor became evident metastases had already occurred widely. The course was rapidly fatal. A review of the literature yielded only one case reported previously. The present case illustrates that pure fibrosarcoma occurs occasionally as a stromal tumor and may be associated with a dismal prognosis. The diagnosis of pure fibrosarcoma should be made only after extensive sampling of the testicular tumor to rule out an associated germ cell component. The differential diagnosis also includes the fibroma of gonadal stroma origin and the unclassified sex cord-stromal tumor with a predominance of spindle cells.
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ranking = 1
keywords = stromal
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2/19. Case report of heterotopic bone formation in metastatic carcinoma of the colon.

    The case of an 83-year-old woman, who was operated on for an adenocarcinoma of the sigmoid colon and died with retroperitoneal matastases, is described. The post mortem examination showed widespread heterotopic bone formation in these metastases. Gastrointestinal cancers and their metastases are liable to calcify and ossify, and they do so more frequently than other malignant epithelial tumors. A search through the literature led to the discovery of 35 other cases of this type. The highest frequency of heterotopic bone formation occurs in cancers of the distal portion of the large intestine and in pulmonary and lymph node metastases. The results of the present case support the view that bone formation derives from the metaplasia of stromal fibroblasts into osteoblasts. The knowledge that gastrointestinal cancers can calcify and ossify has a definite diagnostic relevance for the radiologist and gastroenterologist.
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ranking = 0.33333333333333
keywords = stromal
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3/19. Endometrial stromal sarcoma of the retroperitoneum.

    Endometrial stromal sarcoma (ESS) is an uncommon neoplasm whose occurrence outside the uterus is extremely rare in the absence of metastasis or extension of a primary uterine neoplasm. When ESS occurs in such locations it is often associated with the uterine adnexa or serosal surface of various organs. Although rare, ESS is usually considered in the differential diagnosis of spindle cell neoplasms in the female patient. We report a case of ESS arising in the retroperitoneum and discuss the morphologic and immunohistochemical features in the context of the differential diagnosis of a retroperitoneal low-grade spindle cell neoplasm occurring in the female patient.
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ranking = 1.6666666666667
keywords = stromal
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4/19. Unusual aspects of desmoplastic small round cell tumor.

    desmoplastic small round cell tumor (DSRCT) is a neoplasia that occurs mainly in childhood and involves abdominal or peritoneal sites, coexpressing ectodermal and mesenchimal immunophenotypic markers, and is endowed with an impressive stromal desmoplasia that tends to decrease on tumor relapse. To date, over 150 cases have been collected in the literature. Its presumed neuroectodermal histogenesis has been challenged by cytogenetic findings different from those usually associated with neoplasms of neuroectodermal origin. The authors report a case bearing clinical and histologic aspects of typical desmoplastic retroperitoneal small cell tumor, with intense and diffuse nuclear immunopositivity for WT1, but lacking divergent immunophenotype. Ultrastructural investigation revealed that desmoplasia could result from fibrillary synthesis by neoplastic cells.
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ranking = 0.33333333333333
keywords = stromal
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5/19. Gastrointestinal stromal tumor of the retroperitoneum: CT and MR findings.

    Gastrointestinal stromal tumor (GIST) is a non-epithelial neoplasm arising from the wall of the gastrointestinal (GI) tract. Although the majority of GISTs are located in the GI tract, some GISTs occur in the omentum, mesentery and retroperitoneum. GIST arising in the retroperitoneum is extremely rare, and its radiological findings have not been well described. We report a case of primary retroperitoneal GIST and discuss its imaging findings.
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ranking = 1.6666666666667
keywords = stromal
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6/19. Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases.

    diagnosis of renal angiomyolipoma (AML) by fine-needle aspiration (FNA) may be difficult because cytological and radiological findings sometimes overlap with renal cell carcinoma (RCC) and liposarcoma. Five FNAs of AMLs were studied. Epithelioid and spindle stromal cells were arranged in loosely cohesive clusters and singly. The chromatin was evenly distributed and bland. Occasionally nuclear atypia was identified. Nuclei were oval to elongated, nucleoli were inconspicuous or absent, naked nuclei were present in three specimens, and intranuclear inclusions were present in two specimens. The cytoplasm was delicate and sometimes finely vacuolated. adipose tissue was observed in two specimens. Thick-walled vessels, mitoses, and necrosis were absent. Corresponding surgical material showed typical features of AML. In FNA, bland chromatin and inconspicuous nucleoli distinguish renal AML from RCC and liposarcoma. adipose tissue is not universally present. Cellular atypia in conjunction with overlapping radiological findings with RCC and liposarcoma are potential diagnostic pitfalls. Immunocytochemical (ICC) stains may elucidate the correct diagnosis.
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ranking = 0.33333333333333
keywords = stromal
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7/19. Endosonographic diagnosis of recurrent gastrointestinal stromal tumors associated with Carney's syndrome.

    This is a report of a patient who manifests all of the features of Carney's syndrome, including gastrointestinal stromal tumors (GISTs), extra-adrenal paragangliomas, and pulmonary chondromas. The patient underwent surgical resection of a gastric GIST; a retroperitoneal, nonfunctional paraganglioma; and a mediastinal, catecholamine-secreting paraganglioma (pheochromocytoma). Recently, new gastric GISTs were diagnosed by endoscopic, ultrasound-guided, fine-needle aspiration (EUS-FNA) biopsy and were resected. recurrence of stromal tumors following complete resection is common in Carney's syndrome and presents considerable management challenges. This case illustrates several important points: (1) Carney's syndrome, although rare, should be considered in patients with foregut GISTs; (2) GISTs associated with Carney's syndrome, like sporadic gastric GISTs, may have a more indolent clinical course; and (3) EUS-FNA may be useful for the diagnosis and management of GISTs in this syndrome.
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ranking = 62.876666432687
keywords = gastrointestinal stromal, stromal
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8/19. Cytogenetic confirmation of a gastrointestinal stromal tumor and ewing sarcoma/primitive neuroectodermal tumor in a single patient.

    We report a rare case in which two tumor entities, a gastrointestinal stromal tumor (GIST) and Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET), with distinct cytogenetic features occurred in a single patient. The patient was a 72-year-old woman. The first tumor was a submucosal gastric tumor and was diagnosed as a low-risk group GIST based on morphological characteristics and the results of an immunohistochemical analysis for c-kit and CD34. Further cytogenetic analysis revealed that this tumor had a point mutation (D842V substitution) in exon 18 of the platelet-derived growth factor receptor alpha gene. The second tumor was found more than 4 years after the appearance of the first tumor. ES/PNET was highly suspected both morphologically and immunohistochemically, and the diagnosis was confirmed by the detection of an EWS rearrangement using a fluorescence in situ hybridization technique. Although the cytogenetic correlations of these two tumors are unclear, accurate histologic recognition is of clinical importance because the treatments for these two tumors differ.
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ranking = 62.543333099353
keywords = gastrointestinal stromal, stromal
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9/19. Myxoid variant of adrenocortical carcinoma: report of a unique case.

    Myxoid variant of adrenocortical carcinomas (ACC) are rare, there being only 11 cases in the literature to date. Reported herein are the findings of a case, which in contrast to all previously reported myxoid ACC, was devoid of typical non-myxoid areas. The patient was a 61-year-old man in whom a left adrenal mass was detected during investigation of Cushing's syndrome. The adrenal was replaced by malignant cells and expanses of myxoid material. The cells were positive for melan-A, synaptophysin, vimentin and alpha-inhibin. The ultrastructural features of the cells were typical of adrenal cortical differentiation. The differential diagnosis of myxoid ACC includes extraskeletal myxoid chondrosarcoma, chordoma, myxoid adenocarcinoma, myxoma, lipomatous tumors, nerve sheath tumors, smooth muscle tumors, gastrointestinal stromal tumor and other sarcomas. The presence of myxoid material in a retroperitoneal lesion raises a broad differential diagnosis in which myxoid adrenocortical neoplasms should be included. Clinicoradiological correlation may be helpful, but special stains, immunohistochemistry and ultrastructural examination may be necessary to establish the diagnosis.
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ranking = 12.508666619871
keywords = gastrointestinal stromal, stromal
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10/19. Gastrointestinal stromal tumor with diffuse mesenteric metastases.

    Although malignant gastrointestinal stromal tumors (GISTs) sometimes show peritoneal dissemination, diffuse metastasis to only the mesentery is rare. We describe the unusual case of GIST in a 69-year-old woman who showed multiple nodules restricted only to the mesentery except the surface of the small and large bowel, omentum, and abdominal wall. These small nodules were similar to those seen in leiomyomatosis peritonealis disseminata. Histological and immunohistochemical findings were consistent with GIST. This case of GIST shows an apparently unique and rare spreading pattern.
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ranking = 13.841999953204
keywords = gastrointestinal stromal, stromal
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