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)

Fifteen children, 14 males and 1 female with a mean age of 4.9 years, were treated for rhabdomyosarcoma of the bladder and the prostate, between 1976 and 1985. In 14 patients, the disease was limited to the pelvis, while one had pulmonary metastases. The lesions were trigonal in 12 patients and involved the prostate in the other three. Eleven patients received vincristine-Adriamycin-cyclophosphamide (VAC) chemotherapy, followed by radiation therapy. Four of these 11 patients required cystoprostatectomy for residual or persistent disease. Of the remaining four patients, two underwent radical cystoprostatectomy, one partial cystectomy and the patient with pulmonary metastases received only chemotherapy. Six patients were alive at 5 years (40% 5-year survival). Six patients died of local relapse within 18 months, one patient died of an unknown cause, while two patients were lost to follow-up free of disease after 2 years.
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PMID:Rhabdomyosarcoma of the bladder and prostate in children. 194 14

Thirty-six previously untreated patients younger than 21 years of age with sarcoma arising in the perineal region were entered on the Intergroup Rhabdomyosarcoma Studies (IRS) I and II from 1972 through 1984. The tumor histologic subtype was alveolar rhabdomyosarcoma (RMS) in 20 patients (56%), embryonal RMS in 11 patients (30%), and other types of sarcoma in 5 patients (14%). Fifteen children had grossly complete surgical excision (Clinical Groups I and II), and 15 had localized gross residual tumor (Group III) after initial operative management. Six patients had distant metastases (Group IV) at diagnosis. Twelve patients without distant metastases underwent regional lymph node biopsy; tumor involvement was found in six. Subsequent treatment consisted of chemotherapy with vincristine (VCR) and dactinomycin (AMD) for all patients; patients in Groups III and IV also received cyclophosphamide (CYC) with or without Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH). Radiation therapy (RT) was administered to patients in Clinical Groups II, III, and IV. Overall, 28 (78%) patients achieved a complete clinical response. The 3-year disease-free survival rate for these patients was 42%, compared with 52% for all other patients in the combined IRS I and IRS II series (P = 0.44). The overall 3-year survival rate was 59%, compared with 64% for all other patients in IRS I and IRS II (P = 0.48). Aggressive treatment is needed in children with perineal sarcoma to improve their prognosis.
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PMID:Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1972 through 1984. 218 89

A series of 15 consecutive children with head and neck nonorbital rhabdomyosarcoma (RMSA) with meningeal extension were prospectively treated with chemotherapy consisting of Adriamycin (doxorubicin; Adria Laboratory, Columbus, OH) (ADM), vincristine (VCR), cyclophosphamide (CPM), and dactinomycin (DACT) followed by radiotherapy (60 Gy) to the primary tumor volume, along with intrathecal methotrexate (IT MTX). Thirteen of 15 responded to preradiation chemotherapy. Four of 13 relapsed. Relapse occurred at the level of the primary tumor in three of four. The 3-year progression-free survival (PFS) was 59%, similar to that achieved in a previous series treated with a comparable therapeutic approach that also included whole-brain radiotherapy as a prophylaxis of possible occult meningeal seeding. It is concluded that CNS prophylaxis with radiotherapy is questionable in the management of childhood RMSA with meningeal extension.
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PMID:Questionable role of CNS radioprophylaxis in the therapeutic management of childhood rhabdomyosarcoma with meningeal extension. 223 Aug 72

As part of two sequential protocols using intensive combined modality treatment in pediatric and adolescent sarcomas, 31 consecutive patients with primary chest wall tumors were treated between November 1977 and March 1986. This group included 13 patients with peripheral neuroepithelioma (Askin's tumor), 11 patients with Ewing's sarcoma, 3 patients with rhabdomyosarcoma, and 4 patients with undifferentiated sarcomas. Following complete work-up, 17 patients presented with localized disease and 14 patients presented with metastases. Patients received intensive combined modality treatment with combination chemotherapy (vincristine, cyclophosphamide, Adriamycin, +/- actinomycin-D and DTIC) and high-dose conventionally fractionated radiation therapy to the primary (55-60 Gy) and non-pulmonary metastases (45-50 Gy). Radiation techniques used for the primary chest wall tumor varied with the clinical presentation. Patients achieving a complete response received either low-dose fractionated TBI (1.5 Gy/0.15 Gy fx/5 weeks) or high-dose TBI (8 Gy/4 Gy fx/2 days) and an intensive cycle of chemotherapy followed by autologous bone marrow transplantation. Twenty-five of 31 patients were judged to have a complete response (including 1 patient with complete resection). With minimum follow-up of 6 months and median follow-up of 36 months from completion of treatment, 14 patients remain disease-free with 2 additional patients alive in second remission after relapse. Patients with localized disease at presentation have improved disease-free survival and overall survival compared to patients with metastases at presentation. All 17 localized patients achieved a CR and 11 are NED compared to 8 of 14 metastatic patients achieving a CR and only 3 are NED. There have been 5 loco-regional recurrences with 3 "in-field" failures and 2 failures in the regional pleura. There were no treatment-related deaths and no clinically significant cases of pneumonitis. To date, 2 patients have significant treatment related morbidity, including 1 patient with scoliosis requiring surgery and 1 patient with acute leukemia developing 42 months after the start of therapy (presently in remission). We conclude that this intensive combined modality therapy results in a high CR rate and good local control with acceptable morbidity. Patients with metastatic disease at presentation remain a therapeutic challenge.
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PMID:Treatment of sarcomas of the chest wall using intensive combined modality therapy. 264 97

In the 1981 cooperative soft-tissue sarcoma (CWS-81) study, a clear correlation between the degree of response to initial chemotherapy comprising vincristine, actinomycin D, cyclophosphamide, and Adriamycin (VACA) and the survival of patients with rhabdomyosarcoma was found. In the subsequent CWS-86 study, cyclophosphamide was replaced by ifosfamide (VAIA) in the expectation that the combination VAIA might be more effective than VACA. In both studies, the initial cytostatic response for primary unresectable tumors was evaluated after the first cycle of chemotherapy at weeks 7-9. The reduction in tumor volume was measured by computerized axial tomographic (CAT) scan or sonography, and the patients were categorized as complete responders, patients with a tumor regression of greater than 2/3 albeit incomplete, patients with a tumor regression of less than 2/3 but greater than 1/3, and nonresponders, who underwent either a tumor regression of less than 1/3 or tumor progression. We compared the response rate obtained with VACA chemotherapy and that resulting from VAIA chemotherapy. The preliminary data from this comparison show a tendency for a higher rate of good responders (complete and less than 2/3 tumor regression) to be induced by VAIA therapy (71%) than that obtained using the VACA combination (55%). From the response-prognosis relationship, we confidently expect that the final outcome for patients in the present study will be better than that in the previous study.
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PMID:Comparison of the rates of response to ifosfamide and cyclophosphamide in primary unresectable rhabdomyosarcomas. 266 90

A total of 488 tumors entered in the Eastern Cooperative Oncology Group (ECOG) Study EST 3377 were evaluated histologically by a panel of pathologists from member institutions for quality control purposes. The overall agreement rate between the eligible submitting diagnosis and the pathology review panel's diagnosis was 74% (312/424). In 10% (44/424), the case was excluded because it was deemed to be nonsarcoma. In the other 16%, the disagreement concerned the type of sarcoma. The histologic type with the lowest agreement rate was rhabdomyosarcoma (17%), followed by sarcoma not otherwise specified (NOS) (27%), angiosarcoma (33%), and fibrosarcoma (48%). These figures reflect the significant degree of difficulty in the diagnosis of these tumor types. The treatment response rate of soft tissue sarcomas in the randomized study of Adriamycin (Adria Laboratories, Columbus, OH) regimens was slightly higher for those with lower grade sarcomas, i.e., 25% (four of 16) response rate for Grade 1 lesions; 22% (17/77) for Grade 2, and 21% (35/170) for Grade 3. When adjusted for type of sarcoma, there was no noticeable difference between Grade 1-2 versus 3 in response rate. A statistically significant difference in the percentage of complete responders was noted between Group A tumors (synovial sarcoma, hemangiopericytoma, sarcoma NOS, and Ewing's; 12.2%) versus Group B tumors (all other types--mostly spindle cell sarcomas; 3.5%) (P = 0.02).
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PMID:Pathologic analysis of advanced adult soft tissue sarcomas, bone sarcomas, and mesotheliomas. The Eastern Cooperative Oncology Group (ECOG) experience. 273 94

Since 1978, six boys with prostatic rhabdomyosarcoma have been treated at our institution. Three had localized disease and were managed by initial biopsy, vincristine, actinomycin-D, and cyclophosphamide (VAC) chemotherapy, and bladder-sparing surgery with or without irradiation. Further combination chemotherapy ("pulse" VAC, Adriamycin, VP-16, cisplatin, and ifosfamide) was continued for 20 to 22 months following the induction course. Two boys had microscopic residual disease undetected by frozen section and unresponsive to radiotherapy. Subsequent total cystectomy 4 and 7 months later resulted in eradication of disease. In one patient, preservation of the bladder was achieved at the age of 3 months for 8 years. Artificial sphincter inserted to cure his urinary incontinence failed because of ischemia secondary to cuff compression and scar tissue. He is alive today with a modified Koch pouch urinary diversion. Of the 50% who had metastatic disease at presentation, two were dead within 12 months despite aggressive chemotherapy and irradiation. The third is currently on treatment. Although chemotherapy has markedly improved the prognosis, surgery is still necessary in most cases for cure. Bladder salvage is a desirable goal; however, residual microscopic disease, difficulty with frozen-section disease detection, and poor tissue vascularization for subsequent sphincter replacement remain significant obstacles.
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PMID:Rhabdomyosarcoma of the prostate in childhood: current challenges. 280 70

The patterns of disease extent, response to treatment, and survival rates were examined in 129 children registered as metastatic (Group IV) soft-tissue sarcoma patients on the Intergroup Rhabdomyosarcoma Study (IRS)-I protocol from November 1972 through October 1978. The patients' ages at diagnosis ranged from 3 months to 21 years (median, 10.5 years); 72 were male patients and 57 were female patients (male-to-female ratio, 1.3:1). The tumor arose in an extremity in 34 patients, the head and neck (exclusive of the orbit) in 27, the genitourinary tract in 25, the retroperitoneum or pelvis in 14, the trunk in 12, the intrathoracic region in nine, the hepatobiliary system in four, the perineum in two, and the orbit in two patients. The histologic types of soft-tissue sarcoma confirmed by the pathology review committee were embryonal rhabdomyosarcoma in 68 patients, alveolar rhabdomyosarcoma in 41, undifferentiated sarcoma in 14, embryonal-botryoid in two, pleomorphic in one patient, and other sarcoma in three patients. Of 105 patients who were confirmed in Group IV and had complete information concerning sites of metastatic deposits, 53 patients presented with metastases restricted to only one anatomic region or tissue; the other 52 had diffuse metastases. The most common sites of distant tumor deposits were the lungs, bone marrow, and other soft tissues. After treatment with vincristine, dactinomycin, and cyclophosphamide with or without Adriamycin (Adria Laboratories, Inc., Columbus, OH) and radiation therapy, 65 (50%) of the 129 eligible patients achieved complete disappearance of all detectable tumor. The likelihood of achieving and maintaining a complete response for 2 years was highest among patients whose tumors originated in the genitourinary tract, retroperitoneum-pelvis, or the hepatobiliary or perineum regions. Thirteen of these 45 children (29%) are alive and disease-free at a minimum of 2 years after initiation of treatment. Only 10 of the other 84 (8%) are surviving disease-free at 2 years (P = 0.02). The presence of restricted rather than diffuse metastases at diagnosis (P = 0.02) was the only other characteristic significantly related to long-term complete remissions. One hundred and two patients (79%) died at a median of 47 weeks after diagnosis, 92 from sarcoma and ten from other causes.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I. 284 74

The clinical and pathologic findings of two patients with malignant ectomesenchymomas of soft tissue are reported. Malignant ectomesenchymomas are a composite of ganglion cells or neuroblasts and one or more malignant mesenchymal elements, usually rhabdomyosarcoma. Our first case was composed of ganglioneurosarcoma plus rhabdomyosarcoma, the second composed of neuroblastoma plus rhabdomyosarcoma. The name is derived from the suggestion that they arise from pluripotential ectomesenchyme. An English language literature search revealed 11 other cases that arose in soft tissue and had adequate clinicopathologic data. Of the 13 cases, 10 occurred in infants, three occurred in adults, and nine were males. Six patients were alive and free of disease at last follow-up (range, 0.6-12 years; mean, 3.4 years), four patients had died of tumor (within 0.5-3.3 years; mean, 1.3 years), one case had died of Adriamycin (doxorubicin) toxicity, and two cases had been lost to follow-up. Complete surgical resection is the mainstay of treatment and chemotherapy appears to be important.
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PMID:Malignant ectomesenchymoma of soft tissue. Report of two cases and review of the literature. 295 Sep 92

Orthotopic heart transplantation was performed in a 21-year-old medical student 11 years after left pneumonectomy for a rhabdomyosarcoma. The cardiomyopathy was the result of the administration of doxorubicin (Adriamycin). The surgical procedure was largely facilitated as a result of an in-hospital donor and the absence of major adhesions. The early postoperative course was mainly uneventful. The patient is doing well 9 months after operation, without any episode of rejection or infection.
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PMID:Orthotopic heart transplantation eleven years after left pneumonectomy. 304 83


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