1/46. A case of infantile rhabdomyofibrosarcoma with immunohistochemical, electronmicroscopical, and genetic analyses.A case of infantile rhabdomyofibrosarcoma arising on the buttocks of a 15-month-old boy is reported with histological, immunohistochemical, electronmicroscopical, and cytogenetic findings. Histological examination showed a proliferation of spindle-shaped cells in a fasciculated pattern, with occasional rounded rhabdomyoblastic cells with abundant eosinophilic cytoplasm. Immunohistochemically, the tumor cells expressed desmin and MyoD1 but were only weakly positive for myoglobin. No clear rhabdomyoblastic features were observed by electronmicroscopic examination. Chromosome analysis showed a clone of 46, XY, der(2)t(2;11)(q37;q13), different from any karyotypic abnormality in the original report of this neoplasm. loss of heterozygosity at 11p15.5, the most frequent genetic alteration in embryonal rhabdomyosarcoma, was not detected. The low degree of striated muscle differentiation and tumor localization supported the diagnosis of infantile rhabdomyofibrosarcoma rather than spindle-cell rhabdomyosarcoma in this case. The present case has been uneventful as of 25 months after surgery. The rather long recurrence-free period, which has not been reported in previous cases, may be attributable to chemotherapy-induced rhabdoid differentiation of the tumor cells.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
2/46. Rhabdoid epithelioid leiomyosarcoma of the uterine corpus: a case report and literature review.A case of epithelioid leiomyosarcoma of the uterus with rhabdoid phenotype and early rhabdomyoblastic differentiation is described. A 72-year-old woman with a 5-week history of increased abdominal girth was found to have a large pelvic mass. The uterus revealed a large intramyometrial and left adnexal necrotic tumor that had spread to the small bowel mesentery and to the anterior abdominal peritoneum. The tumor was an epithelioid leiomyosarcoma with rhabdoid phenotype and focal early rhabdomyoblastic differentiation, as confirmed by immunohistochemical and ultrastructural techniques. Also called composite extrarenal rhabdoid tumor (CERT), this lesion should be differentiated from malignant mixed mullerian tumor, rhabdomyosarcoma, endometrial stromal sarcoma, and pure rhabdoid tumors of the uterus. The recognition of a rhabdoid phenotype is of clinical importance since these tumors are prone to be aggressive.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
3/46. 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) |
4/46. rhabdoid tumor of the skin.A tumor in the skin of a 42-year-old man was analyzed by light and electron microscopic study and immunohistochemistry. The tumor cells were large and contained eosinophilic, periodic acid-Schiff (PAS)-positive inclusions in the cytoplasm. Immunohistochemically, the neoplasm was positive for intermediate filaments, cytokeratin, vimentin, desmin, and alpha-1-antichimotrypsin, and negative for S-100 and leukocyte common antigen (LCA). Ultrastructurally, the cytoplasm and cellular processes of the cells were inundated with intermediate filaments, some of which were tightly bundled. Junctional complexes and secretory granules were absent. These features suggest a rhabdoid tumor: a malignant tumor of uncertain origin.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
5/46. 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) |
6/46. Molecular analysis of a partial deletion of 22q in a central nervous system rhabdoid tumor.We previously reported the non-random occurrence of monosomy 22 in rhabdoid or atypical teratoid tumors of the brain in three young children. We now present cytogenetic and molecular studies of an additional rhabdoid tumor with the karyotype 46,XX,-9,-22, i(1q), der(22)t(9;22)(p13;q11)/45,XX,-9,-10,- 22, i(1q), der(22)t(9;22)(p13;q11). These studies further demonstrate the involvement of chromosome 22, and they begin to define the critical region containing a gene or genes involved in the development or progression of rhabdoid tumors of the brain.- - - - - - - - - - ranking = 7keywords = rhabdoid (Clic here for more details about this article) |
7/46. Malignant rhabdoid tumour of kidney. A case report.A case of malignant rhabdoid tumour of kidney is reported. Interesting features are bony metastasis at presentation and FNAB diagnosis of metastasis.- - - - - - - - - - ranking = 36.146257232364keywords = rhabdoid tumour, rhabdoid (Clic here for more details about this article) |
8/46. Paratesticular congenital malignant rhabdoid tumor diagnosed by fine-needle aspiration cytology. a case report.We report the FNA features of a congenital malignant extrarenal rhabdoid tumor (MERT) located in the right paratesticular area of a newborn full-term boy (39 wk gestation), with disseminated metastases in the liver and right parietal region. The diagnosis was suggested two days after birth by fine-needle aspiration biopsy (FNAB) of the parietal mass, which demonstrated an atypical large cell proliferation with vesicular nuclei, prominent nucleoli, and abundant cytoplasm exhibiting paranuclear dense inclusions. The diagnosis was confirmed by histopathologic and immunohistochemical examination of the primary paratesticular tumor. To the best of our knowledge, this is the third MERT reported in the paratesticular region, one of the few congenital extrarenal non-central nervous system cases, and the third congenital case (renal or extrarenal) primarily diagnosed by FNAB. We emphasize the characteristic cytologic features of a congenital rhabdoid tumor, which must be known by pathologists because of the clinical and prognostic implications. Diagn. Cytopathol. 2004;30:46-50.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
9/46. Cutaneous extrarenal rhabdoid tumor with myogenic differentiation.BACKGROUND: The rhabdoid phenotype is characterized by large epithelioid cells with abundant eosinophilic cytoplasm and paranuclear inclusions of intermediate filaments. Although originally described in tumors from pediatric kidneys, the rhabdoid phenotype has since been described in a variety of patient ages and extrarenal sites. Extraordinarily, the rhabdoid phenotype has emerged in cutaneous neoplasms, either as a pure extrarenal rhabdoid tumor or a composite phenotype coupled with another malignancy. Regardless of the clinical setting, the rhabdoid phenotype is uniformly associated with aggressive biological behavior. We report the findings from a rare and very aggressive primary extrarenal rhabdoid tumor of the skin with myogenic differentiation. CASE REPORT: A 53-year-old woman presented with an ulcerated nodule on her right medial calf and ipsilateral inguinal lymphadenopathy. Histological examination of the skin nodule as well as cytologic examination of a lymph node disclosed the characteristic rhabdoid phenotype. Electron microscopy demonstrated paranuclear globules of intermediate filaments; special stains suggested an underlying myogenic histogenesis. The patient was aggressively treated with chemotherapy but ultimately died of her disease 8 months after presentation. CONCLUSIONS: The rhabdoid phenotype is rarely seen in cutaneous neoplasms, but when present, portends a poor prognosis adding particular importance to its recognition.- - - - - - - - - - ranking = 12keywords = rhabdoid (Clic here for more details about this article) |
10/46. Malignant rhabdoid tumor of the liver. A distinct clinicopathologic entity.A malignant rhabdoid tumor occurring as a primary hepatic neoplasm in a six-month-old white infant is reported. It was treated by an attempt at total resection involving right hepatic lobectomy and by chemotherapy with cis-platinum, VP-16, and Adriamycin. Despite this, recurrence of the tumor resulted in the girl's death within three months. The neoplasm showed typical light microscopic features of malignant rhabdoid tumor as well as filamentous cytoplasmic inclusions by electron microscopic examination and staining for both cytokeratin and vimentin by immunohistochemistry. The classic clinicopathologic features of this tumor support the concept that malignant rhabdoid tumors similar to those of the kidney may occur in extrarenal sites.- - - - - - - - - - ranking = 7keywords = rhabdoid (Clic here for more details about this article) |
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