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

VP-16-213, a semisynthetic podophyliotoxin, was tested for antitumor and clinical toxicity in 126 children. The drug was administered iv daily x 5 days every 2 weeks at a starting dose of 75 mg/m2/day. The dose was increased by 25 mg/m2/day/course until clinical response or significant toxicity occurred. The only major toxicity was hematologic, with neutropenia as the most predominant feature. There was one local allergic reaction at the site of injection. No systemic allergic responses were reported. The drug demonstrated significant activity in acute myelomonocytic leukemia with four responses among 19 patients, less activity in acute myelocytic leukemia with two responses among 44 patients, and little activity in acute lymphocytic leukemia with only one partial response among 12 patients. Objective partial responses occurred in ten of 48 patients with solid tumors: two each with Wilms' tumor, lymphoma, and histiocytosis X, and one each with rhabdomyosarcoma, neuroblastoma, Ewing's sarcoma, and undifferentiated carcinoma. The inclusion of VP-16-213 in combination chemotherapy for childhood acute myelomonocytic leukemia and acute myelocytic leukemia appears indicated in patients relapsing after initial therapy. For solid tumors this is an interim report, with further patient accrual required before specific comments can be made.
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PMID:Phase II study of VP-16-213 in childhood malignant disease: a Children's Cancer Study Group Report. 29 6

Eleven cases of Pneumocystis carinii pneumonia were diagnosed during a 3 1/2-year period at a pediatric hospital where this infection had never been identified previously despite appropriate studies. The incidence of infection was 3.0, 7.4, and 4.2 cases per 1,000 patient months in children being treated for acute leukemia, neuroblastoma, and rhabdomyosarcoma, respectively. The outbreak coincided with increased intensity of chemotherapy for these malignancies. Ten of the patients had received four or more chemotherapeutic agents within three months of the onset of infection. Because no exogenous source of the epidemic was found, latent endogenous infection activated by immunosuppression was presumed to be the ultimate cause of the outbreak. Increased intensity of chemotherapy may result in P carinii outbreaks and may be an indication for anti-Pneumocystis prophylaxis with trimethoprim/sulfamethoxazole in patients at risk.
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PMID:An outbreak of Pneumocystis carinii pneumonia at a pediatric hospital. 31 May 39

Rhabdomyosarcomas are malignant tumors, and especially occur in childhood. In adults they are extremely rare. A pleomorphe rhabdomyosarcoma of the skin, localized on the nose of an 85-year old female patient, is reported. After surgical removal the defect was reconstructed with a full skin graft. The four histologic types of rhabdomyosarcoma (pleomorphic, alveolair, embryonal, botryoid) are discussed.
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PMID:[Rhabdomyosarcoma of the skin]. 34 Apr 23

The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.
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PMID:Paratesticular rhabdomyosarcoma in childhood. 35 76

Seventeen children with rhabdomyosarcoma stage I to III diagnosed since 1973 are presented. The coordinated management utilizing surgical excision, irradiation (5000 rad) and systemic adjuvant multiagent chemotherapy is described. The favorable sites were head and neck sites and genitourinary region. The mean survival is at present 19 months. 14 children are alive and well. 13 children had no tumor recidivation, 1 child had 8 months after beginning of the therapy lung metastases which after irradiation with 2000 rad disappeared. 3 children (stage III) died 9, 14 and 20 months after diagnosis during therapy by metastases. Acute and late effects on normal tissues from radiation and chemotherapy were noted in 12 cases. A review of the literature and therapeutic alternatives in the future are indicated.
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PMID:[Juvenile rhabdomyosarcoma. Diagnosis and new therapeutic possibilities]. 36 96

A newborn with a pleomorphic rhabdomyosarcoma of the eyelid was treated by chemotherapy, surgery, and radiation therapy and experienced a good response. The nine previous cases in the literature are reviewed and this patient's therapy discussed.
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PMID:Orbital rhabdomyosarcoma in a neonate. 38 75

Twenty-seven previously untreated children with gross residual (20) or metastatic (seven) rhabdomyosarcoma were treated with pulse-VAC (vincristine weekly for 12 doses plus dactinomycin and cyclophosphamide simultaneously given daily for 5 days) and radiotherapy. Toxicity during the 12-week induction period included 23 of 27 (85%) with an absolute neutrophil count (ANC) under 500/mm3; 16/27 (59%) were given intravenous (I.V.) antibodies. Three patients developed Gram-negative sepsis and two of them died. In the first 12 weeks, eight children had a complete response (CR) and another 10 a good partial response (PR), a total of 18 of 27 favorable responses (67%). At 12 weeks, 20 patients received either intermittent pulse-VAC (Regimen H) or a pulse of adriamycin plus vincristine and cyclophosphamide alternating with pulse-VAC (Regimen I) every 4--6 weeks. After this first "maintenance," only seven patients (35%) developed an ANC under 500/mm3 and only three (15%) were given I.V. antibiotics. Severe toxicity disappeared with drug dose reduction in subsequent courses. The overall CR rate was 59% with a PR rate of 15%, a total of 74% favorable responses. This rate is not significantly better than that obtained by previous IRS chemotherapy and radiotherapy schedules for patients with gross residual and metastatic rhabdomyosarcoma. Future studies in these patients will concentrate on diminishing myelosuppression while shortening the rest period between pulses, in order to deliver more drug per unit time.
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PMID:Evaluation of intensified chemotherapy in children with advanced rhabdomyosarcoma (clinical groups III and IV). The Intergroup Rhabdomyosarcoma Study (IRS) Committee of the Cancer and Leukemia Group B Children's Cancer Study Group, Southwest Oncology Group. 39 31

The classification of the histologic types of rhabdomyosarcoma is based on poorly defined criteria. This has resulted in marked disparities in studies reported from different institutions, as well as difficulties in assessment of the clinical behavior of the different histologic types. A retrospective morphologic analysis of 36 consecutive cases of rhabdomyosarcoma of childhood was undertaken according to predefined and strict guidelines for diagnosis. Undeflecting adherence to such criteria identified the embryonal type as the most common form, and the alveolar variant as a distinct clinicopathologic entity with a much more aggressive course; it also resulted in a large proportion (approximately one-fourth) of sarcomas of undertermined histogenesis. In spite of either prolonged follow-up observation with repeated biopsies, autopsy study, or electron-microscopic study of tumor tissue, no evidence could be obtained to substantiate the rhabdomyogenic derivation of the latter group of neoplasms. Precise systematization of the morphology of these cases may be contingent upon careful inventory of their fine structural features; current classifications appear to have disregarded the morphologic heterogeneity of this group of tumors.
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PMID:Rhabdomyosarcoma of infancy and childhood. Problems of morphologic classification. 39 6

Preliminary clinical trials using cryopreserved autologous bone marrow reinfusion have now been carried out at our institution in 5 children and 2 adults with advanced stages of neuroblastoma, rhabdomyosarcoma, non-Hodgkin's lymphoma and small cell carcinoma of the lung. Normal numbers of in vitro colony forming cells (CFU-C) were obtained from these patients despite prior courses of combination chemotherapy. The dose of marrow cells cryopreserved ranged from 1-6 X 10(8) cells/kg and recovery of CFU-C after thawing averaged 50%. Partial or complete hematologic reconstitution was achieved in all patients. The time for recovery ranged from 10-43 days for leukocytes (greater than 1000 cells/mm3) and 23-45 days for platelets (greater than 50,000/mm3). Two patients have died of interstitial pneumonitis due to cytomegalovirus. Three patients have died of recurrent tumor at 40, 48 and 156 days post-transplant. Two patients have had significant therapeutic benefit. One of these had a stable partial response permitting the use of further post-transplant therapy and is alive and well 16+ months post-transplant. The other patient had a complete response and remains tumor-free 25+ months following therapy. We conclude: 1) Autologous bone marrow reinfusion permits hematologic reconstitution following marrow-ablative therapy. 2) A quantity of marrow sufficient for this purpose remains viable following cryopreservation even when obtained from patients previously exposed to chemotherapy. 3) Autologous bone marrow reinfusion now allows the exploration of more intensive cytoreductive therapy in selected malignancies.
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PMID:Autologous bone marrow transplantation in the treatment of selected human malignancies: The Johns Hopkins Oncology Center Program. 40 Jun 94

The basic informations concerning pathology and clinical stagging of malignant tumours localized in urinary bladder and prostatic gland are introduction to proper presentation of own material collected and treated in the years 1962-1977. The material consists of 23 cases of rhabdomyosarcoma localized in minor basin. Formerly in the years 1962-1975, the basic form of treatment was surgery with/or radiotherapy. Chemotherapy that was given accidently in special indications as single dose or for a short time. Since 1975, we have started with systematic multidrug chemotherapy (VAC) used as an important part of complex therapy. Finally the authors discuss the place of chemotherapy in complementary treatment on the base of the literature and their own experience.
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PMID:[Chemotherapy of bladder and prostatic neoplasms in children]. 40 Jul 71


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