1/10. Proximal-type epithelioid sarcoma in the pelvic soft tissues.A case of proximal-type epithelioid sarcoma arising in the pelvic soft tissues of a 46-year-old man is presented. The tumor showed a predominantly epithelioid component with rhabdoid features, cord-like arrangement, small nests, abundant eosinophilic cytoplasm containing intracytoplasmic, paranuclear, hyaline-like globules, large vesicular nuclei, and prominent nucleoli. There were foci with alveolar arrangement and a spindle-cell proliferation. The tumor cells were positive for cytokeratin CAM5.2, vimentin, epithelial membrane antigen, and CD34. flow cytometry showed a diploid dna content with high S-phase fraction. The patient had pelvic lymph-node metastases. He died of the disease 5 months after diagnosis. It is important to include proximal-type epithelioid sarcoma in the differential diagnosis of pelvic tumors.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
2/10. Thymic carcinoma with rhabdoid features.A case of thymic carcinoma with rhabdoid differentiation is presented. A 67-year-old man who presented with chest pain and hemoptysis was referred to The University of texas M. D. Anderson Cancer Center (Houston, TX). Radiologic studies revealed a large anterior mediastinal mass. After the initial biopsy and preoperative chemotherapy, a radical thymectomy revealed a stage III thymic carcinoma with a rhabdoid component. The rhabdoid component was characterized by large cells with an eccentric nucleus, prominent nucleolus, and typical paranuclear cytoplasmic inclusions. Immunohistochemical and electron microscopic studies confirmed the presence of rhabdoid cells with the paranuclear cytoplasmic inclusions staining for both pancytokeratin and vimentin. The patient was given postoperative chemotherapy and radiation. He has since developed metastases to the pelvis and is alive with disease at 20 months of follow-up. To our knowledge, this is the first reported case of thymic carcinoma with rhabdoid features.- - - - - - - - - - ranking = 9keywords = rhabdoid (Clic here for more details about this article) |
3/10. Proximal-type epithelioid sarcoma with elevated serum CA 125: report of a case with CA 125 immunoreactivity.Proximal-type epithelioid sarcoma is a recently described rare soft tissue neoplasm. It is commonly found in the pelvic, perineal, and genital areas in middle-aged or older adults, as compared with the classic type of epithelioid sarcoma, which arises in the distal portion of the extremities in adolescents and young adults. Proximal-type epithelioid sarcoma has a more aggressive clinical behavior than the classic type of epithelioid sarcoma. Proximal-type epithelioid sarcoma is histologically characterized by a diffuse proliferation of epithelioid cells with prominent rhabdoid feature. Recently, a few cases have been reported of epithelioid sarcoma with elevated serum CA 125 level and CA 125 immunoreactivity in neoplastic cells. These cases raise the possibility that serum CA 125 and CA 125 immunoreactivity could be a useful tumor marker for diagnosing and monitoring epithelioid sarcoma. We describe a case of proximal-type epithelioid sarcoma with elevated serum CA 125 level (up to 3395 U/mL [reference range, <35 U/mL]) in a 12-year-old girl who presented with a huge pelvic mass measuring 12 cm in greatest dimension. The serum CA 125 level dropped to 452 U/mL after a debulking operation of the mass. Immunostaining for CA 125 demonstrated a positive immunoreactivity in the neoplastic cells. She received one cycle of chemotherapy and died of the disease 2 months after diagnosis. This case represented a rare example of proximal-type epithelioid sarcoma with elevated serum CA 125 and immunoreactivity for CA 125 in the tumor cells. Based on the previous reported cases and the current case, serum CA 125 as well as immunohistochemical stain for CA 125 may be a useful tumor marker of proximal-type epithelioid sarcoma.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
4/10. Immunohistochemical localization of five classes of intermediate filament in a benign pelvic soft tissue tumor of rhabdoid appearance.A benign pelvic soft tissue tumor from a 50-year-old woman was examined by immunohistochemistry and electron microscopy. The tumor cells had abundant eosinophilic cytoplasm with a hyaline appearance, which was filled with large aggregates of intermediate-sized filaments (IF). The cells were positively immunostained by antibodies against cytokeratin, vimentin, desmin, glial fibrillary acidic protein, and neurofilament proteins. This case represents an extreme example of the simultaneous expression of IF by neoplastic cells, and exemplifies the limited applicability of immunohistochemical detection of IF antigens for pathological diagnosis of neoplasms.- - - - - - - - - - ranking = 4keywords = rhabdoid (Clic here for more details about this article) |
5/10. Extrarenal primitive malignant tumor with rhabdoid features: fine-needle aspiration cytology, immunocytochemistry, and electron microscopy of a case.We describe a malignant pelvic tumor with rhabdoid-like features that arose in the sacroiliac region of a 15-year-old girl. Fine-needle aspiration (FNA) cytology showed large cells with glassy cytoplasm, globular hyaline cytoplasmic inclusions, and prominent nucleoli. Immunocytochemical methods applied to FNA material revealed immunoreactivity for cytokeratin, epithelial membrane antigen, and vimentin; the cells failed to react with antibodies to desmin. This distinct appearance and phenotype was most consistent with a diagnosis of extrarenal malignant rhabdoid tumor. Electron microscopy confirmed this interpretation, showing whorled filamentous aggregates in the cytoplasm of the tumor cells.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
6/10. Malignant rhabdoid tumor of the pelvis.An extrarenal malignant rhabdoid tumor (MRT) of the pelvis in a 14-year-old girl is described. The location of the tumor and age of the patient appear to be unique. The neoplasm is histologically and ultrastructurally identical to malignant rhabdoid tumor of the kidney seen in infants and very young children. The tumor had an aggressive clinical course, and the patient died with disseminated disease 13 months after diagnosis. The authors review previous reports of extrarenal MRT, discuss the occurrence of cytoplasmic filamentous inclusions, and contrast MRT with other pelvic sarcomas. Although the exact histogenesis is unknown, ultrastructural and immunohistochemical data suggest a mesenchymal origin.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
7/10. Malignant rhabdoid tumour of soft tissue. An ultrastructural and immunohistological study of a pelvic tumour.A case of extrarenal malignant rhabdoid sarcoma arising in the pelvic soft tissues of a 12-year-old girl is described. By routine light microscopy the tumour resembled, in some areas, an embryonal rhabdomyosarcoma and, in other areas, a neuroblastoma. Electron microscopy revealed characteristic cytoplasmic aggregates of intermediate filaments, often with central clusters of organelle membranes surrounded by these filaments. Immunohistochemical stains showed strong cytoplasmic reactivity for vimentin. Staining for cytokeratin, myoglobin, desmin, neurofilaments, neurone specific enolase, S-100 protein and leucocyte common antigen was negative. A histogenetic origin from primitive mesenchymal cells is favoured. We strongly support the use of electron microscopy for the definitive diagnosis of small round cell undifferentiated sarcomas of childhood.- - - - - - - - - - ranking = 13.646677528611keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
8/10. Paravertebral malignant rhabdoid tumor in infancy. in vitro studies of a familial tumor.Two female siblings died within three months after presenting with paravertebral tumors in the first year of life. The pathology of the two tumors was identical and characteristic of a malignant rhabdoid tumor. There were no identifiable tumor patterns within the kindred which have been associated with any hereditary cancer or precancer syndromes. fibroblasts were cultured from skin biopsies obtained from the second patient and both parents. Assays of growth kinetics associated with cellular transformation revealed that fibroblasts from the affected sibling can be distinguished from those of the parents and age-matched controls by increased in vitro occurrence of tetraploidy. Such evidence suggests that increased in vitro tetraploidy occurring spontaneously in cultured fibroblasts is an expression of a cancer-prone gene. Increased in vitro tetraploidy has previously been demonstrated in some kindreds with heritable colon cancer syndromes, and may extend our understanding of the genetic etiology of some childhood cancers.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
9/10. The desmoplastic round cell tumor: a new solid tumor of childhood.Three patients with a new, pathologically distinct solid tumor of childhood have been treated recently. The disease is characterized by male predominance, adolescent onset, an extensive abdominal primary tumor, and aggressive metastases to regional lymph nodes, liver, and lung. Two patients presented with vague abdominal pain and the third with testicular pain. All three noted fatigue and malaise of less than two months' duration with minimal associated weight loss. Computed tomography (CT) scans of the abdomen and chest were obtained for initial preoperative staging, and then all three underwent surgical exploration. Widespread disease was found in each case. In no instance was complete tumor extirpation possible because of extensive peritoneal spread and lymphatic and hepatic metastases. Histologically, all three tumors consisted of round blue cells with a dense desmoplastic reaction and focal rhabdoid features. Immunohistochemical markers for epithelial, neural, and muscle elements were positive. Aggressive multidrug chemotherapeutic regimens were used in each case, and all three patients are alive and well but with known residual disease. We conclude that in cases of the desmoplastic round cell tumor of childhood, CT scans underestimate the extent of disease, and exploratory laparotomy is necessary for diagnosis and appropriate staging. Surgery is usually palliative because of extensive spread. awareness of this newly recognized aggressive solid tumor of childhood is essential to define its natural history and guide the development of effective multidisciplinary therapeutic regimens.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
10/10. Synchronous occurrence of malignant rhabdoid tumor two decades after Wilms' tumor irradiation.We describe the first case of synchronous malignant rhabdoid tumor arising in the pelvis and the lung two decades after both sites were irradiated for Wilms' tumor. Although the malignant rhabdoid tumor phenotype is controversial as a specific clinicopathological entity, this case exhibited classic clinicopathological features of malignant rhabdoid tumor, including tissue features of a trabecular to alveolar growth pattern; cellular features of characteristic eosinophilic cytoplasmic inclusions exhibiting intermediate filament clusters, large nuclei with prominent central nucleoli, and a dual mesenchymal and epithelial immunocytochemistry profile; and clinical features of a rapidly deteriorating course leading to death 2 months after diagnosis. The occurrence of synchronous malignant rhabdoid tumors in sites irradiated for Wilms' tumor raise interesting questions concerning the relationship of radiation-induced malignancies to putative tumor suppressor gene defects, the distinction of synchronous secondaries from primary recurrences and metastases, and finally the quintessential relationship of malignant rhabdoid tumor to Wilms' tumor.- - - - - - - - - - ranking = 9keywords = rhabdoid (Clic here for more details about this article) |
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