1/20. Extraskeletal osteosarcoma of the scalp.A rare case of extraskeletal osteosarcoma of the scalp in a 56-year-old woman is described. At presentation she was found to have an 8-cm diameter, tender, firm, exophytic scalp tumor. MRI scan confirmed absence of underlying skeletal origin and showed extension along the subcutaneous plane. The tumor was excised and the patient received post-operative chemotherapy. Histologically, the tumor showed classical features of an osteogenic osteosarcoma with focal fibroblastic areas. In addition, there were rhabdoid cells present, which showed paranuclear cytoplasmic immunoreactivity for epithelial membrane antigen. The patient developed metastatic disease 6 months after surgical excision.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
2/20. Aspiration cytopathology of epithelioid angiosarcoma.BACKGROUND: Epithelioid angiosarcoma (EA) is an uncommon neoplasm readily mistaken for carcinoma. In contrast to the histopathology of this tumor, the cytopathology as obtained using fine-needle aspiration (FNA) biopsy has rarely been described. methods: Three patients with histologically and immunohistochemically proven EA each underwent FNA using standard technique before surgical resection. RESULTS: Aspirate smears were obtained from 3 males (ages 47, 63, and 15 years) each of whom presented with a solitary palpable soft tissue mass, 1 from the left calf and 2 from the right popliteal region. No patient had a history of malignancy or had been exposed to prior radiation therapy. Smears were relatively hypocellular due to the dilutional effects of abundant blood. Cells were scattered on slides primarily in a single cell dissociated pattern; small aggregates were present in a fraction of the slides. Malignant cells generally monotonous in size and averaging three to four times the dimension of a mature lymphocyte had a rounded so-called epithelioid configuration. Cells possessed primarily rounded, single nuclei often eccentrically placed, with some anisokaryosis, and smooth nuclear borders. Binucleated cells with mirror-image nuclei were much less frequent, and cells with three or four nuclei were even more scarce. Cells contained large single nucleoli or more often multiple misshapen smaller nucleoli. cytoplasm was abundant and finely granular in virtually all cells. In some, the cytoplasm acquired a central spheric density thus producing a "rhabdoid" appearance that was only seen with air-dried Diff-Quik (Fisher Scientific, Biochemical Sciences, Inc., Swedesboro, NJ) stained smears. Mitoses were readily found. Immunostaining of the cell block in one case permitted a specific diagnosis of EA before subsequent surgical excision. CONCLUSIONS: Epithelioid angiosarcoma may display a rhabdoid morphology in FNA biopsy smears, and this cytopathology can closely mimic that of nonsmall carcinoma, malignant melanoma, and other epithelioid types of soft tissue tumors. immunophenotyping is essential for definitive diagnosis. Cancer (Cancer Cytopathol) copyright 2000 american cancer society.- - - - - - - - - - ranking = 2keywords = rhabdoid (Clic here for more details about this article) |
3/20. Mutations of the p53 gene in malignant rhabdoid tumors of soft tissue and the kidney: immunohistochemical and dna direct sequencing analysis.Malignant rhabdoid tumor (MRT) is characterized by the presence of intracytoplasmic eosinophilic inclusions composed of whorls of intermediate filaments. This tumor was originally described as an entity of the abortive type of Wilms' tumor in childhood. Recently, it has been proved that these rhabdoid cells can be observed in various types of malignant tumors, including soft tissue sarcoma or carcinoma. To investigate the oncogenesis of this tumor, we examined the p53 gene alteration by means of immunohistochemical analysis and dna direct sequencing in three cases of malignant rhabdoid tumor (MRT) of the soft tissue and three cases of MRT of the kidney. All the cases of MRT of the soft tissue and two of the cases of MRT of the kidney showed immunopositivity for p53 protein. Among them, one of the cases of MRT of the soft tissue and two of the cases of MRT of the kidney showed missense mutations of the p53 gene. These results strongly suggest that p53 gene alterations may have an important role to play in the aggressive biological behavior and poor prognosis of this tumor.- - - - - - - - - - ranking = 7keywords = rhabdoid (Clic here for more details about this article) |
4/20. Malignant rhabdoid tumor of soft tissues: a cytopathological and immunohistochemical study.We describe the cytopathological and immunohistochemical features of a primary malignant rhabdoid tumor (MRT) located in the left forehead region of an 8-mo-old female. The cardinal characteristics are hyaline cytoplasmic inclusions, rounded or bean-shaped nucleus, vesicular chromatin, central prominent nucleolus, and poorly defined cytoplasmic profile. Both the cytoplasm and the hyaline inclusion are vimentin and keratin positive, but negative with desmin, S-100 protein, neuron-specific enolase, (NSE) myoglobin, leukocyte common antigen (LCA), and alpha-1-antichymotrypsin. This immunohistochemical pattern, although not exclusive to MRT, is fairly typical of MRT, and when it is assessed in conjunction with cytopathological findings and clinical data permits a confident diagnosis of MRT. We emphasize the utility of immunohistochemical techniques in making the differential diagnosis of MRT against other childhood round-cell tumors.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
5/20. Malignant rhabdoid tumor arising from soft parts of the right thigh with unusual neurologic manifestation: report of a case.A case of malignant rhabdoid tumor (MRT) arising from the soft tissue of the right thigh in a 49-year-old Chinese female with peripheral neuropathy is reported. The tumor, exhibiting the salient features of MRT, was composed of sheets and nests of polygonal cells with prominent nucleoli and characteristic paranuclear inclusion-like hyaline globules under light microscopy which corresponded to aggregates of intermediate filaments under electron microscopy. The results of immunohistochemical studies of the tumor cells were also characteristic: cytokeratin ( ), vimentin ( ), epithelial membrane antigen (EMA) ( ), desmin (-), myoglobin (-), leukocyte common antigen (LCA) (-), kappa (-), lambda (-), IgG (-) and IgA (-). Serologic study revealed an M-component of IgA. The clinical evolution of the patient was highly aggressive and inevitably lethal. An adult malignant rhabdoid tumor is unusual, and its association with peripheral neuropathy and the coexistence of an M-component of IgA in this case appears to be unique. In this report, the differential diagnosis of histopathologic features, the association of peripheral neuropathy and the coexistence of an M-component of IgA are discussed.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
6/20. Soft-tissue extrarenal rhabdoid tumor with a unique long-term survival.Rhabdoid tumors of kidney and extrarenal rhabdoid tumors are identified by a round-epithelioid cell morphology and a bland immunophenotype, but a distinctive ultrastructure dominated by paranuclear whorls of intermediate filaments, most usually of vimentin. These tumors are also known to be highly aggressive malignancies, which, typically, bear a poor prognosis, frequently measured in months following initial presentation. The authors record the case a soft-tissue rhabdoid tumor in a 12-year-old boy with a unique long-term survival in excess of 16 years. The features of this case are documented, with a brief summary of histological, immunohistochemical, ultrastructural, and genetic characteristics of this entity.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
7/20. Proximal-type epithelioid sarcoma: report of two cases in the perineum: differential diagnosis and review of soft tissue tumors with epithelioid and/or rhabdoid features.The authors report two cases of perineal proximal-type epithelioid sarcoma in middle-aged men, age 51 and 43 years old. Both tumors were located in the right side. In the first patient a 7.5-cm, well-encapsulated tumor was completely excised. The second patient was a referral case with incomplete excision, but the computed tomography scan and magnetic resonance imaging showed a 14-cm nonencapsulated tumor involving the soft tissues of the inner thigh and perineum, as well as metastasis in right inguinal and retroperitoneal lymph nodes. Both neoplasms had a predominant solid pattern alternating with occasional discohesive areas. Both were composed of large oval to polygonal cells with vesicular nuclei, conspicuous nucleoli, and amphophilic to eosinophilic cytoplasm. Rhabdoid phenotype was identified in the second case only. The first neoplasm displayed 15% necrosis, 7 mitoses per 10 high-power field, focal vascular invasion, and no extracapsular invasion. The other exhibited 60% necrosis, 12 mitoses per 10 high-power fields, extensive vascular invasion, no distinct capsule, and invasion of the surrounding fatty tissue. Both were positive for vimentin, cytokeratin, epithelial membrane antigen, and CD34. Muscle-specific actin was negative in the first case and focally positive in the second. CD56 was positive in the second case and negative in the first case. desmin, CD45, CD30, factor viii, CD31, S100, HMB45, calretinin, and synaptophysin were negative in both. Since proximal-type epithelioid sarcoma can be confused with a number of other soft tissue tumors with epithelioid and/or rhabdoid features, the authors emphasize the immunohistochemical differential diagnosis.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
8/20. 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) |
9/20. 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) |
10/20. Establishment of a rhabdoid tumor cell line with a specific chromosomal abnormality, 46,XY,t(11;22)(p15.5;q11.23).The malignant rhabdoid tumor is a rare, poorly understood tumor which occurs primarily in children. The kidney is a frequent primary site of origin, but the tumor has arisen in other mesodermally derived tissues as well. Controversy exists regarding the embryonic origin of the rhabdoid tumor and recent histopathologic studies suggest that it may be of neuroepithelial origin. Our immunohistochemical and electron micrographic studies support this theory. No consistent chromosome abnormalities have been reported in this tumor and no cell lines are available for study. We have established and characterized the first rhabdoid tumor cell line. It possesses a specific chromosomal abnormality, 46,XY,t(11;22)(p15.5;q11.23). The translocation may provide an important clue to the pathogenesis of the tumor as well as an opportunity for further study of the involved chromosome regions.- - - - - - - - - - ranking = 7keywords = rhabdoid (Clic here for more details about this article) |
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