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)

Forty-three children with malignant soft tissue sarcomas (IRS Groups II-IV) were treated with rapid dose delivery chemotherapy protocol comprising six courses of vincristine, adriamycin and cyclophosphamide, given in most cases within 8 weeks (Rapid VAC). This was followed in 36 patients by high dose melphalan with autologous bone marrow rescue. Twenty-six patients also received irradiation to the site of primary tumour. The Rapid VAC regimen was well tolerated and largely administered as an out-patient. There was one toxic death which occurred 2 months after high dose melphalan due to a combination of infection and possible anthracycline cardiomyopathy. Stages were, (Intergroup Rhabdomyosarcoma Study (IRS) system) Group, Group II--four patients. Group III--27 patients and Group IV--12 patients; International Society of Paediatric Oncology (SIOP) staging, Stage I--11, Stage II--13, Stage III--7, Stage IV--12. Actuarial survival at 5 years for all stages is 57% and event free survival 44%. For patients with non-metastatic diseases, 62% and 53% respectively. This treatment strategy utilises the philosophy of rapid drug delivery with high dose consolidation and enables all chemotherapy to be finished within a 4 month period. In general, a conservative approach was applied to both radiation and surgery to minimise late sequelae related to these treatment modalities. Although the small number of high risk patients in this study limits conclusions regarding efficacy in these subgroups the overall results with this regimen appear to be comparable to that with other approaches.
 ...
PMID:Rapid VAC high dose melphalan regimen, a novel chemotherapy approach in childhood soft tissue sarcomas. 189 70

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.
 ...
PMID:Orthotopic heart transplantation eleven years after left pneumonectomy. 304 83

Since 1970, we have carried out cancer chemotherapy and immunotherapy in cooperation with Japanese scientists, particularly Prof. H. Umezawa, who has generously supplied bleomycin, peplomycin, acalcinomycin A (ACM), THP-adriamycin (THP), neothramycin and bestatin. Malignant tumors curable by pharmacotherapy are polycythemia vera (CR 100%), acute lymphoid leukemia (ALL) (CR 80%), Burkitt tumor (CR 80 or 50%), Hodgkin disease (CR 80%), chorioepithelioma (CR 80%), testicular cancer (CR 80%), ovary cancer of children (CR 80%), Wilms renal cancer (CR 60%), rhabdomyosarcoma (CR 75%), osteosarcoma (CR 60%), Ewing tumor (CR 60%), brain tumor of children (CR greater than 50%), testicular embryonal cancer of children (CR greater than 50%), acute myeloid leukemia (AML) (CR 50%), non-Hodgkin lymphoma (NHL) (CR 50%), ovary cancer of adults (CR 40%), small cell lung cancer (CR 20%) and breast cancer. Our experimental and/or clinical experience with ACM, THP, methoxy-9-ellipticine lactate, navelbine, 4-demethyl-epipodophyllotoxin-beta-d-ethyledene glucoside, bestatin and interferon is presented. ACM is effective against AML, ALL, NHL, Burkitt tumor, breast cancer. We have comparatively investigated cardiac and dermal toxicity of 12 kinds of anthracycline antibiotics and mitoxantrone, using golden hamsters. Of the drugs examined, ACM, THP, AD-32 and AD-143 cause much less cardiomyopathy and alopecia than the other agents. The results have been confirmed by electron microscopic studies. Bestatin is an immunorestorator, which recovers immunological functions decreased in aged animals. We hope that cancer chemotherapy and immunotherapy will progress in future and contribute to cure of neoplasms. Japanese scientists have been making a great contribution in the field of cancer pharmacotherapy, and we are eager to cooperate with Japanese scientists in cancer treatment studies.
 ...
PMID:[Japanese-French cooperation in tumor pharmacotherapy: 1970-1990]. 619 71

A 20-year-old man was found to have high plasma creatine kinase (EC 2.7.3.2) activity in association with a rhabdomyosarcoma of the prostate. There was a very high proportion of the MB isoenzyme. Clinical findings and changes in creatine kinase activity with time showed that this increase was not ascribable to myocardial disease. We believe that the high proportion of CK-MB, confirmed by a combination of electrophoretic and immunoinhibition studies, originated from the tumor.
 ...
PMID:High creatine kinase MB isoenzyme activity associated with a rhabdomyosarcoma. 687 18

The aim of this study was to identify patients treated in Great Britain for childhood cancer and subsequently referred for cardiopulmonary transplantation in order to assess diagnosis, cancer treatment, management and outcome. Computerised record linkage between the National Registry of Childhood Tumours and the national transplant database held and maintained by the United Kingdom Transplant Support Service Authority (UKTSSA) was used to identify patients. Verification and clinical details were then obtained from the oncology and transplant centres. 16 patients were identified from the 31992 cases of childhood malignancy diagnosed in Britain since 1970. These comprised 13 heart transplants, 2 heart/lung transplants and 1 patient who died while on the heart transplantation waiting list. All 14 potential heart transplant patients had cardiomyopathy presumed secondary to anthracycline therapy. The original diagnoses were acute myeloblastic leukaemia (3), Wilms' tumour (4), rhabdomyosarcoma (2) and one each of five different solid tumours. Median age at diagnosis was 44 months (range 4-165 months). Median anthracycline dose was 413 mg/m2 (range 240-680 mg/m2). 13 of the 14 potential cardiac transplantation patients were more than 2 years from end of their cancer treatment before requiring transplantation and the transplantation was performed 2-126 months after onset of cardiac failure at a median age of 163 months. Five year actuarial survival from transplantation was 74%. There was no recurrence of the original malignancy in any of these patients. Both heart/lung patients died, 3 and 11 months after the transplant. These heart transplantation data suggest that, in Britain, survival compares favourably with that of patients whose heart transplant was required for other causes of cardiomyopathy. This indicates that patients successfully treated for childhood cancer should not be excluded from transplant programmes.
 ...
PMID:Cardiac transplantation in childhood cancer survivors in Great Britain. 908 61

The Costello syndrome is characterized by prenatally increased growth, postnatal growth retardation, coarse face, loose skin resembling cutis laxa, nonprogressive cardiomyopathy, developmental delay, and a outgoing, friendly behavior. Patients can develop papillomata, especially around the mouth, and have a predisposition for malignancies (mainly abdominal and pelvic rhabdomyosarcoma in childhood). Costello syndrome is likely to be an autosomal dominant disorder. The pathogenesis is unclear, but there are many clues for a disturbed elastogenesis, possibly through a disturbed elastin-binding protein reuse by chondroitin sulfate-bearing proteoglycans accumulation. A review of the findings in the 73 patients that have been described in sufficient detail is provided.
 ...
PMID:Costello syndrome: an overview. 1256 Oct 57

Macroglossia, prenatal or postnatal overgrowth, and abdominal wall defects (omphalocele, umbilical hernia, or diastasis recti) permit early recognition of Beckwith-Wiedemann syndrome. Complications include neonatal hypoglycemia and an increased risk for Wilms tumor, adrenal cortical carcinoma, hepatoblastoma, rhabdomyosarcoma, and neuroblastoma, among others. Perinatal mortality can result from complications of prematurity, pronounced macroglossia, and rarely cardiomyopathy. The molecular basis of Beckwith-Wiedemann syndrome is complex, involving deregulation of imprinted genes found in 2 domains within the 11p15 region: telomeric Domain 1 (IGF2 and H19) and centromeric Domain 2 (KCNQ1, KCNQ1OT1, and CDKN1C). Topics discussed in this article are organized as a series of perspectives: general, historical, epidemiologic, clinical, pathologic, genetic/molecular, diagnostic, and differential diagnostic.
 ...
PMID:Beckwith-Wiedemann syndrome: historical, clinicopathological, and etiopathogenetic perspectives. 1601 Apr 95

An increased risk of different types of malignancy has been reported in patients with Noonan syndrome (NS). We describe a patient with short stature, dysmorphic features, developmental delay, and congenital cardiomyopathy. At 5 years old, he presented with abdominal pain, constipation, and evaluation with ultrasound and computed tomography scan demonstrated the presence of an abdominal mass. Total resection of the mass and consequent histology revealed an embryonal rhabdomyosarcoma. Rhabdomyosarcoma is a rare tumor in NS patients and to the best of our knowledge only 2 cases have been reported so far. The presentation underlines the importance of frequent follow-up of patients with NS, since the incidence of malignancy is low but existing.
 ...
PMID:Rhabdomyosarcoma in a patient with Noonan syndrome phenotype and review of the literature. 1748 16

Hypothyroidism may cause decreased cardiac output and heart failure-and when severe, bradycardia and pericardial effusions may develop. Chemotherapies, particularly doxorubicin, are known and often irreversible causes of cardiomyopathy. As such, when cardiomyopathy develops in patients who have been exposed to anthracycline chemotherapy, the importance of ruling out other reversible causes such as hypothyroidism cannot be overstated. We present a case of acute systolic heart failure in a patient post-doxorubicin chemotherapy and radiation therapy for alveolar rhabdomyosarcoma, found to have severe hypothyroidism as a reversible cause of cardiomyopathy.
...
PMID:Hypothyroid cardiomyopathy in a patient post-doxorubicin chemotherapy. 2705 39

Rhabdomyosarcomas in the parapharyngeal space are quite rare. We report herein on the case of a 14-month-old boy who was hospitalized with a 2-month history of stridor. On admission, right peritonsillar swelling was noted. CT demonstrated the presence of a large tumor in the right parapharyngeal space. MRI findings showed a right parapharyngeal tumor, 6-cm in diameter. Histopathologic evaluation of the tumor revealed embryonal rhabdomyosarcoma. The clinical staging of the Intergroup Rhabdomyosarcoma Study Group (IRSG) was classified as group III. According to regimen 35 of the IRSG III treatment protocol, radiochemotherapy was started, comprising combination therapy with vincristine, actinomycinD, cyclophosphamide, adriamycin, and carboplatin. The patient tolerated the therapy, but with severe pancytopenia and fever. His sedation during irradiation was difficult. After he received a total dose of 10 Gy and had undergone 9 cycles of chemotherapy, an operation for the rhabdomyosarcoma was performed, resulting in successful removal of the tumor. Doses of 20 Gy were intraoperatively administered. After surgery, residual chemotherapy and irradiation was resumed. The evaluation of the response to therapy was complete remission. After 4 years from the start of therapy, all chemotherapy was halted. At present there is no evidence of recurrence or metastasis, and the patient is a well-developed college student. His sequela following therapy consisted of facial asymmetry, dental abnormalities, and pigmentation of the neck. No adriamycin cardiomyopathy was found.
 ...
PMID:A Case of Infantile Large Parapharyngeal Rhabdomyosarcoma Followed Up for More Than 20 Years. 3005 85


1