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)

Eighteen poorly differentiated, small and dark cell malignancies afflicting young individuals without light-microscopic evidence of a rhabdomyoblastic differentiation or a growth pattern characteristic of rhabdomyosarcoma were analyzed and compared with a series of 30 alveolar rhabdomyosarcomas of varying differentiation, where the diagnosis could be established light-microscopically. The study comprised clinical data, light and electron microscopy and immunohistochemistry, using a battery of mono- and polyclonal antibodies against intermediate filaments, myoglobin, epithelial membrane antigen, neuron-specific enolase, S-100 and leucocyte common antigen. All 30 alveolar rhabdomyosarcomas were positive for desmin, while a minority were positive for myoglobin, using monoclonal antibodies. In 8 of the 18 small and dark cell malignancies, support for a rhabdomyoblastic differentiation was obtained by a positive staining for desmin. In only 3 of these 8 cases was there ultrastructural evidence of rhabdomyosarcoma. The results of the investigation indicate that immunohistochemistry is a more useful tool than electron microscopy in the diagnosis of poorly differentiated rhabdomyosarcoma and that the criteria for the diagnosis of poorly differentiated rhabdomyosarcoma may need to be reformulated.
APMIS 1988 Sep
PMID:Alveolar and poorly differentiated rhabdomyosarcoma. A clinicopathologic, light-microscopic, ultrastructural and immunohistochemical analysis. 316 9

Rhabdomyosarcoma is a diagnostic and therapeutic problem in the management of childhood tumours. A case of embryonal rhabdomyosarcoma affecting the sphenoid sinus and involving the cavernous sinus is presented. It has become evident that modern combined modality therapy, including surgery when required, radiation and adjuvant multi-drug chemotherapy offers the best chance of survival.
J Laryngol Otol 1988 Sep
PMID:Embryonal rhabdomyosarcoma of the sphenoid sinus. 317 80

A case of rhabdomyosarcoma of the right kidney is presented. A 78-year-old man was admitted with the complaint of abdominal pain and abdominal fullness on March 15, 1985. Radiological examination showed a giant tumor of the right kidney. Radical nephrectomy and right hemicolectomy were performed. Histological findings were embryonal rhabdomyosarcoma of the kidney. Residual tumor in the duodenal area recurred and he died of peri-duodenal abscess 2 months after admission. This case is the 17th case of rhabdomyosarcoma of the kidney in Japan. The literature is reviewed and discussed.
Hinyokika Kiyo 1987 Sep
PMID:[Rhabdomyosarcoma of the kidney: report of a case]. 332 38

To investigate histologic prognostic factors in soft tissue sarcomas (STS), multivariate analysis was performed on 236 patients with complete clinical information. These included 141 males and 95 females with an age range from 1 to 85 years (median, 47.6 years). Histologically, malignant fibrous histiocytoma (33.5%) was the most common type followed by synovial sarcoma (11%), liposarcoma (10%), rhabdomyosarcoma (7%), and leiomyosarcoma (7%). Monofactorial analysis revealed that sex, depth, location, histologic grade, cellularity, frequency of mitosis, and necrosis were significant prognostic factors. By multivariate analysis, only frequency of mitosis and depth of tumors were proved to be of prognostic significance. The prognostic importance of the frequency of mitosis as shown in previous and the present studies indicates that further investigation of the cell proliferation in patients with STS is required.
Cancer 1988 Sep 01
PMID:Multivariate analysis for histologic prognostic factors in soft tissue sarcomas. 340 80

BA-HAN-1C is a clonal rat rhabdomyosarcoma cell line composed of proliferating mononuclear cells, which partly fuse to terminally differentiated postmitotic myotube-like giant cells. The exposure to retinoic acid in vitro resulted in time- and dose-dependent changes of both cell differentiation and cell growth. The mononuclear cells revealed bundles of newly formed thick and thin myofilaments, never observed in untreated cultures, and exhibited signs of contact inhibition. In addition, there was a statistically significant increase (P less than 0.001) in the number of terminally differentiated postmitotic myotube-like giant cells and in the creatine kinase activity (P less than 0.05) which was used as a biochemical differentiation marker. At the same time cell growth was significantly inhibited (P less than 0.001) in vitro and a decrease in plating efficiency, as well as in saturation density, was observed. These data demonstrate that retinoic acid can suppress cell growth and simultaneously initiate differentiation in a malignant mesenchymal tumor cell line. However, despite the clonal nature of BA-HAN-1C, the complete status of terminal differentiation was not achieved by all tumor cells. The reason why not all tumor cells responded to retinoic acid is unknown at the present time and will have to be the subject of further studies.
Cancer Res 1988 Sep 15
PMID:Terminal differentiation and growth inhibition of a rat rhabdomyosarcoma cell line (BA-HAN-1C) in vitro after exposure to retinoic acid. 340 49

Two hundred and ninety patients with soft tissue sarcomas (STS) in Osaka, Japan, were reviewed. The patients' ages ranged from one month to 84 years (mean 51 years) with a male to female ratio of 1.23:1. The tumors were located in the extremities (120 cases), the trunk (76 cases), the abdominal cavity (40 cases), and the head and neck (43 cases). Histologically the tumors were classified as malignant fibrous histiocytoma (101 cases, 34.8%), liposarcoma (28 cases, 9.7%), synovial sarcoma (24 cases, 8.3%), rhabdomyosarcoma (23 cases, 7.9%), neurogenic sarcoma (20 cases, 6.9%), fibrosarcoma (16 cases, 5.5%), leiomyosarcoma (16 cases, 5.5%) and others. From the present study, it appears that there are no significant differences between STS in Japan and Western countries with regard to the distributions of histological type and primary site.
Jpn J Clin Oncol 1988 Sep
PMID:Soft tissue sarcomas in Osaka, Japan (1962-1985): review of 290 cases. 341 87

DNA was extracted from formalin-fixed and paraffin-embedded tissues of 85 patients with pediatric malignant solid tumors which had been resected at surgery or obtained at autopsy during a 24-year period. The tumors examined included 25 rhabdomyosarcomas, 12 Wilms' tumors, 10 hepatoblastomas and 37 neuroblastoma group tumors. Neuroblastoma group tumors were subclassified into 25 neuroblastomas and 12 ganglioneuroblastomas among which 6 composite ganglioneuroblastomas were included. Sample blocks were selected from both tumors and normal tissues in the majority of cases. We were able to reliably detect N- and c-myc gene amplification in tumor DNA by dot blot-hybridization. The N-myc gene showed approximately from 3- to 500-fold amplification in 19 of 33 cases of stage IV neuroblastoma group tumor. All of these 33 patients had been intensively treated with chemotherapy and/or radiotherapy. The c-myc was amplified 8-fold in 1 case of rhabdomyosarcoma, but neither N-myc nor c-myc was amplified in any cases of Wilms' tumor or hepatoblastoma. We retrospectively examined the association among N-myc gene amplification, prognosis, and histologic subtype in 33 patients with stage IV neuroblastoma group tumors. The survival of the patients with N-myc gene amplification was shorter than that of the patients without amplification of N-myc (p less than 0.05). There was no significant difference in prognosis between the 2 histologic subtypes; neuroblastoma and ganglioneuroblastoma, and the cases of tumors with amplified N-myc showed shorter survivals for each subtype (p less than 0.05). In every case of neuroblastoma group tumor, the copy number of the N-myc gene was the same among primary site and multiple metastatic tumors, even when the lesions showed differences in histologic subtype like neuroblastoma and ganglioneuroblastoma.
Lab Invest 1988 Sep
PMID:Retrospective study on amplification of N-myc and c-myc genes in pediatric solid tumors and its association with prognosis and tumor differentiation. 341 33

We performed immunoperoxidase studies in 29 cases of rhabdomyosarcoma from the Intergroup Rhabdomyosarcoma Study I using antisera against actin, myosin, myoglobin, alpha-actinin, and tropomyosin. Although each of these antisera reacted with some of the tumors, none reacted with all of the tumors, and some tumors showed no reactivity. Antimyosin reacted with more tumors than any of the others, while antiactin and antimyoglobin were about equally sensitive. Antitropomyosin and anti-alpha-actinin reacted with few of the tumors. The better-differentiated tumors were more likely to react compared with the poorly differentiated tumors.
Arch Pathol Lab Med 1986 Sep
PMID:Immunohistochemical studies of rhabdomyosarcoma. 353 Jan 87

Twenty seven cases of rhabdomyosarcoma from pathologic files of the Hokkaido University Hospital from 1975 to 1985 were reviewed and the presence of myoglobin and S-100 protein was investigated immunohistochemically. Their average age at diagnosis was 30.7 years (ranging from two months to 77 years) and there was a predilection for males in a ratio of 1.7:1. The common locations were the head and neck. Nine cases were positively stained for myoglobin, mainly in the cytoplasm of rhabdomyoblasts and 19 cases were positive for S-100 protein and alpha-subunit of S-100 protein in the cytoplasm of immature tumor cells as well as of rhabdomyoblasts. In seven cases, both of myoglobin and S-100 protein were demonstrated. Although S-100 protein is not strictly specific, however, these results suggest that the immunohistochemical demonstration of S-100 protein can be a useful diagnostic tool of rhabdomyosarcoma.
Hokkaido Igaku Zasshi 1986 Sep
PMID:[An immunohistochemical study of myoglobin and S-100 protein in rhabdomyosarcoma]. 353 1

The use of fasciocutaneous flaps in the treatment of soft tissue defects has been widely accepted. This study presents a case in which a huge defect after surgical resection of an advanced rhabdomyosarcoma was reconstructed by an extraordinarily large vertical fasciocutaneous flap from the back.
Br J Plast Surg 1987 Sep
PMID:One stage reconstruction of a massive back defect with a large fasciocutaneous flap. 367 81


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