Cases reported "Peritoneal Neoplasms"

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1/42. Stromal sarcoma arising on endometriosis. A clinicopathological and immunohistochemical study of 4 cases.

    The development of stromal sarcomas on the foci of endometriosis is extremely rare and the differential diagnosis from other tumors of myogenic, vascular, hemopoietic or epithelial origin may present great diagnostic difficulties. We investigated the clinicopathological and immunohistochemical characteristics of 4 cases of endometrial stromal sarcoma that developed on endometriotic foci of the uterus, vagina and omentum. Thye were classed as high grade (1/4) or low grade (3/4) malignant potential tumors, according to their mitotic activity. Immunohistochemically these tumors gave a positive reaction to vimentin, but were negative to desmin, smooth muscle actin, factor viii, EMA and LCA. These characteristics permit their identification and a proper therapeutic approach.
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keywords = stromal
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2/42. Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.

    central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.
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keywords = stromal
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3/42. desmoplastic small round cell tumor--report of 3 cases and review of the literature.

    desmoplastic small round cell tumor (DSRCT) is a rare neoplasm with distinctive histological features, characterized by extensive stromal tissue around islands of small and undifferentiated cells revealing the desmoplastic appearance. It was first described by Gerald and Rosai. It mainly the peritoneum and is found predominently in adolescents and young adults, and it is much more common in males than in females. The histological and immunhistochemical features are very distinct but may be misdiagnosed as other undifferentiated neoplasm if the pathologist has no idea of this entity. We report the experience of three cases here. The first case was diagnosed as malignant neuroendocrine neoplasm orginally and revised to DSRCT later. Case 2 had been diagnosed as Atypical carcinoid and received surgery and chemotherapy. It was revised as DSRCT 5 years later by an other pathologist. Case 3 carried the characteristic feature whereby diagnosis could be made even in frozen section.
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keywords = stromal
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4/42. Primary peritoneal mesodermal adenosarcoma: report of a case and review of the literature.

    BACKGROUND: Mesodermal (mullerian) adenosarcoma arising from the peritoneum is rare and is thought to arise from pluripotent mesothelial and mesenchymal cells of the pelvic cavity or from endometriotic deposits. CASE: A case of primary peritoneal mesodermal adenosarcoma arising from the omentum is described. A 50-year-old woman presented with sudden abdominal distension. Initial laparotomy revealed a 13-kg mass arising from the omentum, which was determined from frozen and paraffin sections to be serous cystadenofibroma. The tumor recurred within 10 months and weighed 18 kg at a second laparotomy. Histopathology and review of the original tumor established the correct diagnosis of mesodermal adenosarcoma. The patient died from disseminated disease 6 months later. CONCLUSION: Adenosarcomas are difficult to differentiate from adenofibromas or endometriosis histologically because of the presence of large areas of low cellularity and infrequent mitotic figures. In such cases, stromal nuclear atypia and periglandular stromal cuffing are features that are diagnostic of adenosarcoma.
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keywords = stromal
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5/42. Ovarian endometriosis and clear cell carcinoma, leiomyomatosis peritonealis disseminata, and endometrial adenocarcinoma: an unusual, pathogenetically related association.

    A 42 year-old female with a preoperative clinical diagnosis of ovarian cancer underwent laparotomy which revealed leiomyomatosis peritonealis disseminata (LPD) in the peritoneum and omentum and a left ovarian endometriotic cyst associated with a clear cell carcinoma. A grade 1, superfically invasive villoglandular endometrial endometrioid adenocarcinoma was also found. Microscopically, the endometriotic cyst wall contained an extensive peripheral band-like condensation of stromal cells. These cells were strongly positive for alpha inhibin and may have been the hormonal source responsible for the induction of the simultaneous LPD and endometrial adenocarcinoma. It is proposed that endometriosis is not only a precursor of clear cell carcinoma but, through secondary hormonal induction of the surrounding ovarian stroma, may also provide a hormonal stimulus for diverse proliferative processes.
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keywords = stromal
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6/42. Gastrointestinal stromal tumor of the lesser omentum: report of a case.

    We describe herein an extremely unusual case of a gastrointestinal stromal tumor (GIST) of the lesser omentum. A 45-year-old man was admitted to our hospital with an intra-abdominal mass that was subsequently misdiagnosed as a submucosal tumor of the stomach. The tumor arose from the lesser omentum and was removed without difficulty. Histologically, the tumor was composed of spindle-shaped cells with an interlacing bundle pattern, and immunohistochemical examination showed that it was positive for myeloid stem cell antigen (CD34), but negative for HHF35 and S-100 protein. These findings were consistent with a GIST lacking myogenic features and neural attributes. The patient had an uneventful postoperative course, and was free of recurrence when last seen 11 months after his operation.
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ranking = 28.244220102146
keywords = gastrointestinal stromal, stromal
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7/42. Peritoneal endometriosis in the broad ligament presenting as a large tumor.

    Peritoneal endometriosis presenting as a tumor is very rare. A case of peritoneal endometriosis in the broad ligament presenting as a large tumor is reported. A 39-year-old woman had a solid and cystic tumor with many microcysts, measuring 17 x 13 x 3.5 cm, mainly located in the right posterior broad ligament. Histologically, the tumor consisted of many endometrial glands associated with various amounts of endometrial stroma. Neither the glands nor the stromal cells had cellular atypia. We diagnosed peritoneal endometriosis presenting as a tumor. Among the six cases (five previously reported cases and our case) of tumor-like endometriosis in the peritoneum, two cases (33%) had received tamoxifen therapy and four cases (67%) had cystic or solid and cystic tumor. Among them, the tumor size ranged from 3 to 17 cm in diameter, and our case was the largest. In conclusion, this case is rare, but it is important for pathologists to be aware of the phenomenon (that extensive peritoneal endometriosis produces a large tumor) in the histological diagnosis of a tumor in the peritoneum.
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keywords = stromal
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8/42. Primary mesenteric tumor with phenotypical features of gastrointestinal stromal tumors.

    We report a case of a primary mesenteric tumor that was discovered in a 71-year-old man. Abdominal US accurately visualized the internal structure of the tumor but failed to determine the site of origin. Computed tomography suggested the mesenteric origin of the tumor before resection. The mass was removed and histopathological examination (including immunohistochemical and ultrastructural studies) was consistent with a primary gastrointestinal stromal tumor (GIST) of the mesentery, a very rare neoplasm scarcely reported in the radiology literature. Clinical, radiological, and pathological findings of this case are presented and the topic of primary mesenteric tumors with phenotypical features of GIST is reviewed.
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ranking = 131.22110051073
keywords = gastrointestinal stromal, stromal
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9/42. Urachal adenocarcinoma in situ with pseudomyxoma peritonei: a case report.

    A 54 year old man presented with a six month history of abdominal pain. A computerised tomography scan showed a well defined intra-abdominal unilocular mass with a calcified wall just superior to the bladder. At laparotomy, pseudomyxoma peritonei was discovered, together with a midline abdominal mass adherent to the anterior abdominal wall originating from the fundus of the bladder. The specimen consisted of a cystic mass measuring 14 x 9.5 x 7 cm overall, which contained mucoid material. Histological examination revealed that the cyst was lined by mucinous epithelium, which in areas varied from having bland morphology to showing pronounced nuclear and architectural atypia. There was abundant extracellular mucin. The specimen was extensively sampled but there was no evidence of invasion. This tumour has many unusual features, namely: the absence of destructive invasion, association with pseudomyxoma peritonei, areas of dysplasia and cystadenoma, and stromal osseous metaplasia within the wall.
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ranking = 0.5
keywords = stromal
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10/42. Gastrointestinal stromal tumor of the rectal mesentery.

    We report a case of gastrointestinal stromal tumor (GIST) arising from the rectal mesentery. GIST of the large intestine is a rare tumor that accounts for only 0.1% of all colorectal cancers. The patient presented to our hospital with constipation and abdominal distension. Computed tomography (CT) revealed a huge mass in the pelvic cavity, and laparotomy disclosed diffuse peritoneal dissemination from the primary tumor. Radiochemotherapy was commenced, but the patient became too ill to complete it and died of the disease 2 months after surgery. A large high-grade tumor with diffuse dissemination was recognized as an indicator of poor survival in this patient.
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ranking = 28.244220102146
keywords = gastrointestinal stromal, stromal
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