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 20-year-old white male was initially suspected clinically and pathologically of having an acute lymphoblastic leukemic process because of fatigue, severe anemia, thrombocytopenia, a leuko-erythroblastic peripheral blood picture, and a diffusely infiltrated bone marrow. Subsequent review of the bone marrow material indicated cytologic features consistent with either an embryonal, undifferentiated small cell mesenchymal malignancy or reticulo-endothelial malignancy. Ultimately, the electron microscopic (EM) study of the tumor proved to be diagnostic of rhabdomyosarcoma. An extensive search for a primary site of rhabdomyosarcoma did not show any lesion, although the genitourinary region was clinically suspected. The clinical course was a rapidly downhill one with extensive bone and CNS involvement. The patient died 5 months later. An autopsy permit was not obtained. This case emphasizes the occasional tendency of rhabdomyosarcoma to masquerade as a hematopoietic malignancy at the time of presentation and the usefulness of EM study in confirming a diagnosis.
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PMID:Systemic rhabdomyosarcoma presenting as leukemia: case report with ultrastructural study and reviews. 330 41

Rhabdomyosarcoma and other small round-cell neoplasms of infancy and childhood frequently involve the bone marrow. An unusual clinical presentation of diffuse bone marrow involvement as the sole manifestation of the disease represents a difficult diagnostic challenge. We report the case of an 18-year-old boy presenting with lower back pain, sternum tenderness, anemia, thrombocytopenia, leukoerythroblastic blood film, hypercalcemia, and renal failure. No solid tumor was found. A diagnosis of rhabdomyoblastic bone marrow metastatic involvement was initially suggested by the morphological picture, and later confirmed by the ultrastructural findings. Hypercalcemia and renal failure were controlled, initially, with furosemide and calcitonin. The patient was treated with intensive chemotherapy, including vincristine, actinomycin D, cyclophosphamide, and doxorubicin. A remission was achieved, lasting 5 months until relapse.
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PMID:Rhabdomyosarcoma presenting with diffuse bone marrow involvement, hypercalcemia and renal failure. 335 35

Etoposide (VP-16), 150 mg/M2, given intravenously daily for 3 days every 3 weeks resulted in 3 complete responses and 6 partial responses in 154 patients with a spectrum of recurrent malignant solid tumors. There was evidence of disease control in an additional 37 patients (27 mixed responses and 10 stable disease). These responses occurred primarily in patients with Ewing's sarcoma, Hodgkin's disease, neuroblastoma and rhabdomyosarcoma. Most of the patients had every extensive prior therapy; however prior therapy with teniposide (VM-26), the congener of VP-16, did not seem to preclude responses to the latter drug. Myelosuppression was the principal form of toxicity. Neutropenia characterized by absolute neutrophil counts of 0.5 to 0.9 x 10(9)/L occurred in one-half of the patients, and thrombopenia with platelet counts of less than 25 to 49 x 10(9)/L in one-fourth. These results demonstrate a favorable therapeutic index for VP-16 in several recurrent childhood solid tumors, supporting its use as a component of primary therapy for these diseases.
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PMID:Clinical trial of etoposide (VP-16) in children with recurrent malignant solid tumors. A phase II study from the Pediatric Oncology Group. 341 Jun 65

We have evaluated the activity of ifosfamide in 75 patients with recurrent sarcomas and pediatric solid tumors. All patients had previously received cyclophosphamide in combination with other chemotherapeutic agents. Ifosfamide was administered as a continuous 5 day infusion at a dose of 1800 mg per M2, except in the last 14 patients who received the drug as a daily one hour infusion at the same dose level. Partial response was observed in 9 of 20 patients with Ewing's sarcoma, 2 of 9 patients with rhabdomyosarcoma, 3 of 17 patients with osteogenic sarcoma and 4 of 29 patients with various other neoplasms. A further 6 patients had stable disease, defined as the absence of progression for at least 6 cycles of therapy. Thus overall response rate was 24%, with the highest response rate of 45% being observed in Ewing's sarcoma. Toxicity was acceptable, although there was quite marked leucopenia (median nadir 700) with less profound thrombocytopenia (median nadir 87,000). Sepsis occurred in 3 patients but no patient died as a result of infection. Hematuria occurred in 43% of patients who did not receive mesna, and in 26% of patients who did, although prior pelvic irradiation was found to be a significant risk factor for hematuria. Only 1 of 14 patients without prior pelvic irradiation or hematuria developed hemorrhagic cystitis when treated with ifosfamide and mesna. Confusional states developed in 6 patients. We conclude that ifosfamide is an active agent in patients with relapsed sarcomas and childhood solid tumors, even when such patients have been previously treated with cyclophosphamide.
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PMID:A phase II study of ifosfamide in the treatment of recurrent sarcomas in young people. 381 17

The combination of dacarbazine and doxorubicin was given to 26 children with untreated rhabdomyosarcoma to determine its efficacy as front-line chemotherapy. A treatment course consisted of 250 mg/m2 of dacarbazine given iv on Days 1-5 and 60 mg/m2 of doxorubicin given iv on Day 1. After three courses of therapy, 17 patients (65%) achieved partial response and nine failed to respond. The side effects of treatment consisted of nausea, vomiting, flu-like symptoms, neutropenia associated with fever, mucositis, and thrombocytopenia (rarely). Although the response rate is comparable to other drug combinations, the lack of complete responses to the combination indicates that it is less effective as front-line therapy.
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PMID:Combination of dacarbazine and doxorubicin in the treatment of childhood rhabdomyosarcoma. 402 41

We have reviewed our experience using single-dose dactinomycin (Act D). Twenty-nine patients with Ewing's sarcoma or rhabdomyosarcoma received 114 courses of Act D (2 mg/m2) in combination with vincristine, cyclophosphamide, or DTIC with or without radiotherapy. Side effects included nausea and vomiting (100% of the courses), severe thrombocytopenia (16%), granulocytopenia (18%), and mucositis (25%). In addition, skin reactions were observed in 74% of the courses following concomitant radiotherapy. These results were compared with those following 152 courses of combination chemotherapy which contained doxorubicin in place of Act D and which were administered to these same patients. It is concluded that single-bolus Act D is no more toxic, is at least as effective, and is more conveniently administered than the traditional divided daily dose of Act D.
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PMID:Tolerance to single-dose dactinomycin in combination chemotherapy for solid tumors. 722 64

This study aimed to assess the response to a novel combination chemotherapy containing carboplatin plus epirubicin in previously untreated children with metastatic rhabdomyosarcoma. 81 children (< or = 18 years) were treated between 1989 and 1994 with a combination of carboplatin, epirubicin and vincristine, given as initial therapy as part of a multicentre European trial (SIOP Intergroup Study of Stage IV Malignant Mesenchymal Tumours in Children). The chemotherapy regimen (CEV) was: carboplatin 500 mg/m2 plus epirubicin 150 mg/m2 administered on day 1, and vincristine 1.5 mg/m2 administered on days 1 and 7. Response was evaluated at day 21. 2 patients achieved complete remission and 41 patients partial remission, with an overall response rate of 53% (95% confidence interval 45-76%). Three patients showed progressive disease (4%). Toxicity was mainly haematological, with 52% experiencing grade IV neutropenia and 34% grade IV thrombocytopenia. Mucositis and infections were not severe. There were no toxic deaths. The combination of carboplatin, epirubicin and vincristine is effective and well tolerated in patients with metastatic rhabdomyosarcoma.
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PMID:Response of previously untreated metastatic rhabdomyosarcoma to combination chemotherapy with carboplatin, epirubicin and vincristine. 908 60

We report a multicentre phase II study of orally administered prolonged schedule etoposide in children with refractory or relapsed malignancy. 83 children were entered into the study. The largest diagnostic groups were neuroblastoma (n = 20), rhabdomyosarcoma/soft tissue sarcoma (n = 16) and brain tumours (n = 16). Etoposide was administered twice daily at a dose of 50 mg/m2/day for 21 days using the intravenous preparation given orally. Disease reassessment was performed after the second course. Etoposide plasma concentrations were measured by HPLC, 2 and 6 h after administration of therapy on days 7 and 14 in 15 patients. 61 patients completed two courses and were evaluable for response. There was 1 complete response (CR), 5 partial responses (PR) 22 stable disease (SD) and 33 progressive disease (PD). Of the 6 with responses, 3 had a diagnosis of medulloblastoma/cerebral primitive neuroectodermal tumour. 24 of 26 patients with SD/PR/CR received further courses with excellent palliative effect. The main toxicity observed was myelosuppression, with 8% and 7% of evaluable courses complicated by grade III-IV neutropenia and thrombocytopenia, respectively. Severe infection (grade III-IV) was rare, complicating only 2/94 evaluable courses. Plasma etoposide median concentrations at 2 h after administration on day 7 of course 1 were 1.5 (range 0.6-2.4) micrograms/ml. Total course 1 area under the etoposide plasma concentration versus time curve (AUC) values were estimated using a limited sampling model. Grade > or = 2 leucopenia was only observed in patients with a day 72 h etoposide concentration of > 2 micrograms/ml or a course 1 AUC of > 35 mg/ml.min. It is concluded that given at a dose of 50 mg/m2/day in two doses for 21 day courses, oral etoposide is well tolerated in children. A correlation between drug concentrations and toxicity was observed. Overall, a low response rate was seen (approximately 10%), but disease stabilisation appears to occur, and useful palliative effect was frequently noted. The response in brain tumours was more encouraging (3/14 PR) and this group requires further evaluation.
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PMID:Phase II study of 21 day schedule oral etoposide in children. New Agents Group of the United Kingdom Children's Cancer Study Group (UKCCSG). 947 Aug 39

We report on a 17-year-old young man with rhabdomyosarcoma in the right parotid area. Relapse therapy was performed with high dose chemotherapy and consecutive autologous stem cell rescue. During this therapy heparin-induced thrombocytopenia was diagnosed. Because of its proven antithrombotic activity Danaparoid-Sodium, a natural low molecular glycosaminoglycan preparation, was used for further antithrombotic prophylaxis. We discharged our patient from the laminar air flow unit six months ago. The alternative antithrombotic therapy was tolerated without any problems. No bleeding events occurred, thrombotic complications and veno-occlusive disease of the liver were avoided.
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PMID:[Heparin-induced type II thrombocytopenia within the scope of high dose chemotherapy with subsequent stem cell rescue]. 962 42

A case of a 25-year-old man who presented with low back, knee, and hip pain with bruising, leukocytosis, and thrombocytopenia is reported. Histopathologic and flow cytometric studies revealed a malignant neoplasm, which was thought to be consistent with an unusual type of acute leukemia. Subsequent immunohistochemical studies revealed diffuse bone marrow involvement by rhabdomyosarcoma, demonstrated by positivity for desmin and muscle-specific actin. In addition, the diagnosis of metastatic rhabdomyosarcoma was confirmed with cytogenetic and ultrastructural studies. The incidence of rhabdomyosarcoma, a disease usually seen in childhood, presenting with extensive bone marrow involvement and mimicking acute leukemia in an adult is extremely rare. This case highlights the need to consider rhabdomyosarcoma in the differential diagnosis in patients who present with a leukemic picture and atypical blasts that lack the usual hematopoietic markers.
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PMID:Rhabdomyosarcoma mimicking acute leukemia in an adult: report of a case with histologic, flow cytometric, cytogenetic, immunohistochemical, and ultrastructural studies. 987 Aug 49


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