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)

Five of the 12 cardiac sarcomas reviewed in this study were recruited from 7,200 autopsy studies, including 2,980 performed on patients dying from malignant disease (overall incidence of 0.07 p. 100; 0.16 p. 100 in the group with malignant disease). These tumours are fifty times less common than cardiac metastases which were observed in 12.5 p. 100 of patients in the group with malignant disease. The other 7 cases of cardiac sarcoma were recovered from our surgical pathology archives over a 16 years period. The sarcomas were of the following histological types: angiosarcoma (6 cases), rhabdomyosarcoma (3 cases), fibrosarcoma (2 cases) and mesothelioma (1 case). The clinical presentation was very variable. The diagnosis was often made only at surgery or autopsy. The prognosis was very poor, the majority of patients dying of cardiac constriction or visceral metastases, within one year of diagnosis.
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PMID:[Primary malignant tumors of the heart. Anatomo-clinical study of 12 cases]. 404 Nov 51

We will report the result obtained from sensitivity tests on various anti-cancer agents for malignant bone and soft-tissue tumors based on SDI (Succinic Dehydrogenase Inhibition Test) method with the use of enzymic activities as marker since 1976. Our study comprised 27 cases altogether 15 cases of osteosarcoma, one case each of Ewing's sarcoma, malignant fibrous histiocytoma and malignant lymphoma, 3 cases of metastatic bone tumor and one case each of angiosarcoma, fibrosarcoma, rhabdomyosarcoma, liposarcoma, 2 cases of metastatic lung tumor among soft-tissue sarcomas. In all cases, sensitivity tests were done on the tumor tissues according to SDI method at the same time as biopsy for the determination of the appropriate medications. Four to six weeks of pre-operative intra-arterial infusion was done followed by radical operation. The results obtained are as follows. Observing the long-term results between subjects that applied anti-cancer agents decided by sensitivity test and those without sensitivity test. The 5 years cumulative survival rate jumped from 30.5% to 50.5%, showing a clear improvement.
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PMID:[Clinical evaluation on the sensitivity test for anti-cancer agents in malignant bone and soft-tissue tumors]. 608 33

Bone marrow biopsy was performed as part of the initial assessment in 74 patients with soft tissue sarcoma. Infiltration of the marrow by tumour was present in four cases, all from the group of 56 patients who had other evidence of metastatic disease, giving an overall incidence of 7%. The histological subtypes were pleomorphic rhabdomyosarcoma, angiosarcoma, synovial sarcoma and myxoid liposarcoma, of which the first three were high-grade tumours. Although it was not possible to determine whether response to chemotherapy was influenced by marrow involvement, haematological toxicity seemed excessive.
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PMID:Bone marrow involvement in adult soft tissue sarcomas. 629 16

A retrospective review of 112 cases treated by one author (J.C.) over the 21 year period 1953-1973 is performed. In 62% of the cases squamous cell carcinoma was the histologic diagnosis, with adenoid cystic carcinoma accounting for a further 16%. The remaining histologic types were few in number and included fibrosarcoma, muco-epidermoid carcinoma, melanoma, chondrosarcoma, adenocarcinoma, malignant Schwannoma, lymphosarcoma, malignant mixed tumor, rhabdomyosarcoma, angiosarcoma, and osteogenic sarcoma. Three years following diagnosis more than 1/3 of the squamous cell carcinomas were alive but by 10 years the survival rate had fallen to 16%. Orbital involvement at the time of presentation or noted at surgery had a poor prognosis with a 17% five year cure rate and only 2% alive at 10 years. Erosion of the pterygoids or pterygopalatine fossa invasion as diagnosed radiologically or intra-operatively revealed all patients dead of disease within five years despite radical surgery and irradiation. Cervical nodal metastases at primary presentation was a particularly ominous finding with all patients dead of disease within three years. Metastases developing later had a better prognosis with 39% surviving five years when treated aggressively.
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PMID:Carcinoma of the maxillary sinus. A correlation of the clinical course with orbital involvement, pterygoid erosion or pterygopalatine invasion and cervical metastases. 630 78

Malignant peripheral nerve sheath tumors are uncommon sarcomas of Schwann cell or fibroblastic derivation which occasionally show histologic evidence of focal divergent differentiation to rhabdomyosarcoma, osteosarcoma, chondrosarcoma, angiosarcoma, epithelial elements, or a combination thereof. The finding of these heterotopic elements in nerve sheath sarcomas is believed to illustrate the differentiating capacity of neuroectodermal tissue. The authors present the clinicopathologic features of 17 such tumors, which represent 14.7% of the malignant peripheral nerve sheath tumors in the authors' institutional experience. Most of the lesions were associated with von Recklinghausen's disease. These tumors do not seem to differ from ordinary malignant peripheral nerve sheath tumors in presentation, operative, gross, and microscopic features, response to therapy, or prognosis.
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PMID:Malignant peripheral nerve sheath tumors with divergent differentiation. 643 4

Seventy-eight cases of adjuvant chemotherapy for primary soft tissue sarcoma including 51 cases of intra-arterial infusion chemotherapy were studied. The patients ranged in age from 1 to 92 years with a median age of 34 years. Thirty-nine patients were male and 39 were female. The seventy-eight cases were comprized of 17 rhabdomyosarcoma, 12 liposarcoma, 12 neurogenic sarcoma, 10 malignant fibrous histiocytoma, 8 leiomyosarcoma, 7 angiosarcoma, 8 others and 4 unclassified sarcomas. Fifty-one patients with soft tissue sarcoma of the extremities were treated by intra-arterial infusion chemotherapy with either VCQ (Vincristine and Carbazilquinone) or VCQ, A (Vincristine, Carbazilquinone and Adriamycin). Out of 42 patients with measurable lesions, 2 CR, 4 PR, 33 NC and 3 PD were obtained. Histological examinations demonstrated histological effect of GI 19, G IIa 11 and G IIb 7 by Ohboshi and Shimosato's criteria. Remarkable effects of treatment were noted in most rhabdomyosarcoma patients. After intra-arterial infusion chemotherapy, a variety of surgical procedures ranging from marginal resection and wide resection to radical amputation were employed in 44 patients. Local recurrence was 27% and distant metastasis developed in 47% of cases.
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PMID:[Adjuvant chemotherapy in the treatment of primary soft tissue sarcomas, with special reference to intra-arterial infusion chemotherapy]. 647 41

One hundred and forty adult patients with advanced sarcomas (125 soft tissue and 15 bone) were treated with a combination chemotherapy regimen consisting of cyclophosphamide, vincristine, Adriamycin, and DTIC (CYVADIC). There were 21 (15%) complete and 45 (32%) partial responders, with an overall response rate of 47%. The response rate was 50% (17% complete) for patients with soft tissue sarcomas compared with 20% (none complete) for patients with bone sarcomas. The median duration of response was 9.5 months (range, 1-40+ months) for complete responders and 7 months (range, 1-31 months) for partial responders. The median time to achieve response was 9 weeks and the median number of courses of therapy to response was three. The median survival time was 16 months for responders compared with 7 months for nonresponders (P = 0.001). The most responsive tumor types were neurofibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, fibrosarcoma, and angiosarcoma. Pulmonary and soft tissue metastases were more responsive than bone and liver metastases. CYVADIC toxicity was predominantly limited to myelosuppression, vomiting, fever of unknown origin, and neuropathy. CYVADIC is an effective combination chemotherapy regimen in the treatment of advanced sarcomas.
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PMID:Cyclophosphamide, vincristine, adriamycin, and DTIC (CYVADIC) combination chemotherapy for the treatment of advanced sarcomas. 737 60

We studied the clinical features, radiographic and pathological findings, treatment, and results for twenty-three patients who had been managed for a soft-tissue sarcoma of the hand between 1982 and 1990. The ages of the patients ranged from sixteen to seventy-six years (median age, thirty-one years). The most common clinical finding was a small, painless soft-tissue mass. Twenty of the tumors were high-grade, and eighteen were less than five centimeters in diameter. The most common diagnosis was synovial sarcoma, which was identified in eight patients. Leiomyosarcoma, rhabdomyosarcoma, and malignant fibrous histiocytoma developed in three patients each; epithelioid sarcoma, in two patients; and angiosarcoma, liposarcoma, neuroectodermal tumor, and clear-cell sarcoma, in one patient each. Curative wide excision or amputation was attempted in twenty-two patients; the margins were positive for tumor cells in eight, and local recurrence was seen in nine. Of the twenty-three patients, fourteen had survived, without evidence of disease, after a median duration of follow-up of forty-nine months, and nine had died of disease. The median rate of survival did not differ significantly on the basis of the size or grade of the tumor or the use of adjuvant treatment. However, the rate of survival of the patients who had a soft-tissue sarcoma of the hand that was less than five centimeters in diameter was significantly lower (p = 0.0008) than that of 152 patients who had a similar tumor at another site in an extremity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Treatment of soft-tissue sarcomas of the hand. 771 73

A serious problem for every pathologist is how to avoid making errors in histopathological diagnosis. Five cases which I misdiagnosed or nearly did were reviewed, and the ways to avoid making errors were discussed. The first case was clinically a gastric carcinoma. In the biopsy of gastric mucosa, I took a lymphoepithelial lesion for poorly differentiated adenocarcinoma. In the surgical specimen, the histological diagnosis was malignant lymphoma. The second case was a tumor of cecum. Clinically cecal cancer was suspected and operated due to the complication of invagination. Histological examination of cecal tumor revealed marked lymphocytic infiltration with plasma cells. The immunohistochemistry showed a mixed pattern of B- and T-lymphocytes with monoclonal proliferation of plasma cells (IgA, Kappa). Because an inflammatory polyp could not be ruled out, it was offered to consultation. The diagnosis was malignant lymphoma, lymphoplasmacytoid type. The third case was a neck tumor. Although histological diagnosis at first was undifferentiated carcinoma, immunohistochemically tumor cells were unexpectedly negative for epithelial membrane antigen and positive for creatine kinase-mm. Finally it was diagnosed as rhabdomyosarcoma. The fourth case was a struma nodosa. In the frozen section, it was diagnosed as follicular adenoma. However, in the permanent section, the diagnosis was corrected to papillary carcinoma, follicular type, because many intranuclear inclusions were clearly observed. The last case was a finger tumor. Since hemangiosarcoma could not be histologically ruled out, it was offered to consultation. The diagnosis was intravascular papillary endothelial hyperplasia. In conclusion, I emphasize the importance of consultation in order to avoid making errors in histopathological diagnosis.
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PMID:[Review of the misdiagnosed cases focusing on the quality control of histopathological diagnosis]. 796 14

In a review of 8724 de novo malignancies that occurred in 8191 organ allograft recipients sarcomas were 7.4% of cancers. Kaposi's sarcoma (KS) made up 5.7%, and other sarcomas (OS) 1.7% a much higher proportion than in the general population. KS was most common in Arab, black, Italian, Jewish, or Greek patients. In 60% of patients with KS the lesions were confined to the skin and/or oropharynx while 40% involved internal organs and/or lymph nodes. Complete remissions following various treatments occurred in 53% of the former group and 27% of the latter. In both groups 32% and 60% of remissions, respectively, occurred when the only treatment was reduction or cessation of immunosuppressive therapy. However, this treatment caused impaired function or allograft loss from rejection in 22 of 34 kidney recipients. Recurrent KS occurred in 5% of patients in remission when immunosuppressive therapy was resumed. Nine of 114 patients (8%) tested for human immunodeficiency virus were positive. Most OS arose in internal organs or soft tissues. The major types were fibrous histiocytoma (20 patients), leiomyosarcoma (15), fibrosarcoma (12), rhabdomyosarcoma (9), hemangiosarcoma (8), undifferentiated sarcoma (7) and mesothelioma (6). Several unusual features were noted. Remarkably, 10 of 105 (10%) sarcomas occurred adjacent to or in a renal (6) or hepatic (4) allograft. Leiomyosarcomas are rare in children, yet 5 of 15 (33%) occurred in pediatric patients. Three hemangiosarcomas occurred in forearms at sites of arteriovenous fistulas used for pretransplant hemodialysis access. One leiomyosarcoma and one fibrosarcoma occurred in previously irradiated areas. One patient with mesothelioma had a history of asbestos exposure and two others had possible exposure.
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PMID:Sarcomas in organ allograft recipients. 854 79


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