Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 30-year-old male underwent radical nephrectomy for a right renal tumor 15 cm in diameter. On microscopic examination of initial 17 sections, the tumor consisted of pleomorphic giant cells and spindle neoplastic cells. There was no carcinomatous component. Immunohistochemically, the neoplastic cells were negative for keratin and epithelial membrane antigen but positive for vimentin. The giant cells were also scatteringly, weakly positive for myoglobin. At that time a diagnosis of rhabdomyosarcoma of the kidney was made. However, further microscopic examination of another eight sections revealed small areas of clear cell-type renal cell carcinoma (RCC) which transited to sarcomatous components and led to a diagnosis of sarcomatoid RCC. The patient underwent three cycles of adjuvant chemotherapy. He has been free of the disease for 14 months after nephrectomy.
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PMID:Sarcomatoid renal cell carcinoma with scant carcinomatous components. 1071 Feb 49

In our phase I reduced-intensity stem cell transplantation (RIST) study with fludarabine (180 mg/m2) or cladribine (0.66 mg/kg) plus busulfan (8 mg/kg), with or without anti-thymocyte globulin (ATG), a total of 85 patients who had a variety of hematological (n=68) or metastatic solid tumors (n=17) were treated. This presentation will further update the results of our RIST program, highlighting several burning issues in the treatment of patients with solid tumors. Pathological classification of solid tumor in this program included renal cell carcinoma (RCC, n=8), rhabdomyosarcoma (n=2), malignant melanoma (n=2), neuroblastoma (n=2), osteosarcoma (n=1), alveolar saft-part-tumor (n=1) and cholangiocarcinoma (n=1). All received PBSCT from an HLA-identical or one antigen-mismatched relative. Regimen-related toxicities were mild and >90% donor chimerism was achieved before day 30 in a majority of patients. The overall survival for hematological and solid malignancies was the same ca. 70% at 1 year.
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PMID:Mini-transplantation strategy for solid tumors. 1243 Aug 94

Eight separate neoplasms with five distinct morphologies are described in the present report. The spontaneous neoplasms were identified in farmed chinook salmon, Oncorhynchus tshawytscha (Walbaum), during processing. The masses were examined histologically and were classified as teratoma, rhabdosarcoma, biliary and renal cystadenomas, renal carcinoma and three leiomyomas. This represents one neoplasm detected for every 125000 fish processed during the sampling period.
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PMID:Sporadic neoplasms of farmed chinook salmon, Oncorhynchus tshawytscha (Walbaum), from New Zealand. 1294 8

Primary rhabdomyosarcoma (RMS) of the kidney in an adult is a very rare and unusual tumor in this site. The clinical signs associated with the flank tumoral syndrome, the histologic appearance of cytoplasmic double striation in rhabdomyoblasts and the immunohistochemical expression of skeletal muscle differentiation (desmin, myoglobin, myogenin) are described in the context of a rapidly evolving renal RMS in a 77-year old man. The differential diagnosis are mainly represented by sarcomatoid renal cell carcinoma. According to the neoplastic extent, the treatment includes radical nephrectomy, chemotherapy and surgery. The prognosis of primary renal RMS is extremely poor, with lymph node, hepatic, bone marrow and pulmonary metastasis and a short survival rate.
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PMID:[Primary pleomorphic rhabdomyosarcoma of the kidney in adults: unusual tumor]. 1465 Mar 5

A 71-year-old woman who presented with left abdominal pain was found to have a noncalcified renal mass with a perisplenic extension on imaging studies. Histologically, the tumor showed predominantly malignant spindle cells with extensive osteoid and chondroid matrix production. Various growth patterns resembling rhabdomyosarcoma, malignant fibrous histiocytoma, and fibrosarcoma were also observed. Immunohistochemistry showed positive staining of the neoplastic cells for cytokeratin and focally positive staining for CD10 and CD117 (c-Kit). Electron microscopic examination revealed a poorly differentiated neoplasm with both mesenchymal and epithelial features. The tumor was diagnosed as a sarcomatoid renal cell carcinoma with overgrowth of the sarcomatoid component (World Health Organization: renal cell carcinoma, unclassified). To our knowledge, sarcomatoid renal cell carcinoma with such a broad morphologic phenotype in a single case has not been documented. Furthermore, the CD117 expression in a sarcomatoid renal cell carcinoma that was observed in this case merits further investigation.
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PMID:Sarcomatoid renal cell carcinoma with divergent sarcomatoid growth patterns: a case report and review of the literature. 1604 2

We have recently isolated a gene, Ankyrin-repeated protein with a proline-rich region (ARPP), that is highly expressed in the skeletal and cardiac muscle. Our previous immunohistochemical analysis revealed that ARPP expression was augmented in rhabdomyosarcoma but scarcely detectable in leiomyosarcoma, showing that ARPP is a useful marker for rhabdomyosarcoma. In the present study, we generated the anti-ARPP monoclonal antibody, YAS11, immunoreactive with the N-terminal region (amino-acids residues 1-145) of the ARPP protein. Further, we immunohistochemically analyzed 100 renal tumors including 14 oncocytomas, and 86 renal cell carcinomas (RCCs). We found that ARPP was highly expressed in 12 of the 14 (85.7%) oncocytomas, but was detectable in only four of the 86 (4.7%) RCCs. Interestingly, ARPP was not detected in any of 11 chromophobe RCCs, suggesting that ARPP may be useful for differential diagnosis between oncocytoma and chromophobe RCC. Furthermore, we found that ARPP was selectively expressed in part of the distal renal tubule in normal kidney. Immunoelectron microscopy with anti-ARPP antibody revealed that ARPP was localized in mitochondria and nuclei in both the normal distal renal tubule and oncocytoma, suggesting that oncocytoma may be derived from the distal nephron, and probably from part of the distal renal tubule.
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PMID:ARPP protein is selectively expressed in renal oncocytoma, but rarely in renal cell carcinomas. 1720 5

In the 10 years to November 1987, 33 different types of naturally occurring neoplasms were diagnosed in 2281 domestic fowls submitted for necropsy examination. The most prevalent type was lymphoma (1825/2281 = 80%) including two cases in chickens from a flock free from Marek's disease virus, exogenous avian leukosis viruses and reticuloendotheliosis virus; the remaining lymphomas were diagnosed on gross and/or histological criteria as Marek's disease (1069) or lymphoid leukosis (754). Of the non-lymphoid neoplasms, leiomyomas of the ventral ligament of the oviduct (99) were the most common followed closely by metastatic abdominal adenocarcinomas (93) and haemangiomas or haemangiosarcomas (91). A large number of fibromas and fibrosarcomas (40), and a smaller number of myxomas and myxosarcomas (17) were observed. Three fibrosarcomas and two myxosarcomas were presented as metastatic abdominal neoplasms, and two fibromas and seven myxomas were found on the rostral extremity of the upper beak of hens, including five cases of myxoma from one flock. Other less prevalent neoplasms described were myelocytomas or myeloblastomas (12), avian keratoacanthomas (11), granulosa-theca cell tumours (11), oviduct adenomatosis and adenocarcinomas (10) and ovarian adenocarcinomas (8). Small numbers of some neoplasms or well-characterized variants were encountered, namely histiocytic sarcomas (5), lipomas (4), liposarcomas (2), reticulum cell sarcomas (3), plasmacytomas (3), intracutaneous keratinizing epitheliomas (3), feather folliculomas (2), proventricular adenomas (2), hepatocellular adenocarcinomas (2), cholangiocellular adenomas and adenocarcinomas (6), pancreatic adenocarcinomas (3), nephroblastomas (7), astrocytomas (5), pinealomas (3), Schwannomas (3) and multifocal melanomas (3). Single examples were found of osteoma, osteosarcoma, rhabdomyosarcoma, leiomyoma of the gizzard wall, intestinal adenocarcinoma, renal adenocarcinoma, adrenocortical adenoma and ultimobranchial cyst.
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PMID:Some observations on naturally occurring neoplasms of domestic fowls in the State of Victoria, Australia (1977-87). 1864 70

From March 1991 through 31st December 2007, 2042 patients underwent stem cell transplantation at the Hematology-Oncology and Stem Cell Transplantation Research Center, affiliated to Tehran University of Medical Sciences. These transplantations included 1405 allogeneic stem cell transplantation, 624 autologous stem cell transplantation, and 13 syngeneic stem cell transplantation. Stem cell transplantation was performed for various diseases including acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, thalassemia major, sickle cell thalassemia, sickle cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combine immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. We had 105 cellular therapies for postmyocardial infarction, multiple sclerosis, cirrhosis, head of femur necrosis, and renal cell carcinoma. About 30 patients were retransplanted in this center. About 74.9% of the patients (1530 of 2042) remained alive between one to 168 months after stem cell transplantation. Nearly 25.1% (512 of 2042) of our patients died after stem cell transplantation. The causes of deaths were relapse, infections, hemorrhagic cystitis, graft versus host disease, and others.
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PMID:Stem cell transplantation; Iranian experience. 1911 Oct 33

Nestin is an intermediate filament that was first identified in neuroepithelial stem cells. During embryogenesis, nestin is expressed in a number of cell types, including neural crest cells and developing myocytes. We have recently shown that nestin is expressed in human podocytes and nephrogenic blastema. We sought to determine the utility of nestin expression in distinguishing pediatric tumors in the region of the kidney. Cases studied included Wilms tumor (n=24), nephroblastomatosis (n=6), renal cell carcinoma (n=19), renal clear cell sarcoma (n=9), mesoblastic nephroma (n=9), neuroblastoma (n=11), malignant rhabdoid tumor (n=8 including 2 renal), Ewing sarcoma (n=16 including 1 renal, 7 soft tissue, and 8 bone), intra-abdominal desmoplastic small round cell tumor (n=5), and rhabdomyosarcoma (n=8, all extrarenal). Nestin expression was assessed semiquantitatively by immunohistochemistry and then scored as positive or negative. All cases of Wilms tumor, mesoblastic nephroma, rhabdomyosarcoma, neuroblastoma, malignant rhabdoid tumor, and desmoplastic small round cell tumor were nestin-positive. In Wilms tumor and nephroblastomatosis, nestin was expressed in blastema and glomeruloid structures, but not tubules. In neuroblastoma, positive staining was detected regardless of degree of differentiation. The majority of Ewing sarcoma and renal cell carcinoma were negative. Expression in clear cell sarcoma was variable with 5 cases negative and 4 cases positive. Thus, nestin is a highly sensitive, but nonspecific, marker of Wilms tumor in the context of tumors that may occur in or around the kidney. Nestin reactivity may be useful in differentiating Wilms tumor from Ewing sarcoma, renal cell carcinoma, or nestin-negative clear cell sarcoma.
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PMID:Diagnostic utility of nestin expression in pediatric tumors in the region of the kidney. 1941 21

Mesenchymal neoplasms of the kidney in adults cover a wide spectrum with characteristic ontogeny and histologic findings and variable biologic profiles and imaging findings. Benign mesenchymal renal tumors include angiomyolipoma, leiomyoma, hemangioma, lymphangioma, juxtaglomerular cell tumor, renomedullary interstitial cell tumor (medullary fibroma), lipoma, solitary fibrous tumor, and schwannoma. Malignant renal tumors of mesenchymal origin include leiomyosarcoma, rhabdomyosarcoma, angiosarcoma, osteosarcoma, synovial sarcoma, fibrosarcoma, malignant fibrous histiocytoma, and solitary fibrous tumor. Cross-sectional imaging findings for mesenchymal renal tumors in adults are varied. Although angiomyolipomas and lipomas show macroscopic fat, lymphangiomas are cystic in appearance. Renal hemangioma may show phleboliths and a characteristic enhancement pattern. Leiomyoma typically arises from the capsule and causes buckling of the renal cortex. Although osteosarcoma may demonstrate characteristic dense ossification, most renal sarcomas demonstrate imaging features that are indistinguishable from the more common renal cell carcinoma. Although some renal mesenchymal tumors have typical imaging findings, biopsy is warranted to establish a definitive diagnosis. Awareness of the various mesenchymal renal tumors and familiarity with their imaging findings permit optimal patient management.
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PMID:Mesenchymal neoplasms of the kidney in adults: imaging spectrum with radiologic-pathologic correlation. 2107 73


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