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)

Twenty-two patients with rhabdomyosarcoma have presented in the years 1970--1977. Of 8 patients who had inadequate postoperative chemotherapy and radiotherapy, or none at all, only 2 patients with small completely resectable tumors are alive and well at 6 and 4 yr after surgery. Fourteen patients had intensive combination chemotherapy in addition to appropriate surgery and radiotherapy. Five have died and the remaining nine are well and free of disease at follow up ranging from 9 mo to over 5 yr. A continued place in the treatment regime is advocated for early surgery aimed at total excision of the primary tumor where this is feasible.
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PMID:Recent experience in the treatment of rhabdomyosarcoma. 42 61

The records of 27 patients with rhabdomyosarcoma involving the parameningeal area (nasopharynx, paranasal sinus, and middle ear) treated from 1961 to 1976 were reviewed. Due to the location of the primary tumor, radiation and chemotherapy were used but surgery was limited to simple biopsy. In the literature, spread of tumor from these primary sites to the meninges has been as high as 26-35%. In this series, meningeal disease developed in only 2 of the 27 patients (7%).
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PMID:Parameningeal rhabdomyosarcoma. 42 88

We have described an unusual case of polyostotic fibrous dysplasia in a 25-year-old white woman who had malignant transformation into a mesenchymal tumor with widespread metastases and features of rhabdomyosarcoma. Masculinization also occurred with high plasma testosterone levels. A mechanism for the development of masculinizing features is discussed.
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PMID:Malignant transformation of polyostotic fibrous dysplasia. 42 32

Clinical aspects of patients with soft tissue tumors encountered at the Department of Orthopaedic Surgery, Toho University over a 15 year period between 1961 and 1976 were analyzed. Accurate clinical records and histological findings were available in 91 patients, 80 of whom had benign tumors and 11 of whom had malignant ones. Recurrence was seen in 7 of the 80 patients with benign tumors. The prognosis was poor in those with malignant tumors as expected, especially in those with rhabdomyosarcoma.
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PMID:Clinicopathological study of soft tissue tumors. 42 18

An implantation model has been used to investigate the cellular progression of chemically induced subcutaneous neoplasia in the mouse. Implantation of 3,4-benzpyrene induced persistent changes in the normal process of connective tissue formation around the implant. Light-microscope and autoradiographic studies have shown a temporal progression from aberrant filter- or muscle-associated cells through proliferative foci to large invasive sarcoma. Electron microscopy revealed that presarcomatous cell foci consisted of one of two different cell types. These were either spindle cells with ultrastructural characteristics similar to foreign-body-induced sarcoma, or cells with the ultrastructural features of rhabdomyosarcoma. The subsequent appearance of two histological groups of sarcoma that were ultrastructurally similar to the cells of the early proliferative foci indicated that both elements may progress to form tumours. However, the constituent cells of both groups of tumours displayed a broad histological and ultrastructural spectrum and the marked similarity between the undifferentiated cells of each suggested that both may have arisen from diverse differentiation of a common pluripotential cell such as the pericyte.
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PMID:Cellular progression of neoplasia in the subcutis of mice after implantation of 3,4-benzpyrene. 44 13

Primary intracranial rhabdomyosarcoma is extremely rare, only nine cases have been reported in the world literature. To differentiate it from medullomyoblastoma, rhabdomyosarcoma should be devoid of neuroblastic elements. A case of rhabdomyosarcoma of the tentorial region in a 17-year-old boy is reported. Carotid angiograms demonstrated a peculiar neovascularity. Multiple cytologic preparations of CSF repeatedly contained rhabdomyosarcoma cells and CSF dissemination in the spinal subarachnoid space was documented at autopsy. The literature is reviewed.
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PMID:Primary rhabdomyosarcoma of the tentorium with peculiar angiographic findings. 45 44

Survival with embryonal rhabdomyosarcoma of all sites has improved dramatically in recent years with the increased use of long-term, cyclic, multidrug chemotherapy. Protocols have been established and are currently being evaluated by the Intergroup Rhabdomyosarcoma Study. The management of embryonal rhabdomyosarcoma of the pelvic viscera, though, remains troublesome. Limited surgical excision is rarely possible and high-dose radiotherapy to the bony pelvis may cause severe and disabling growth disorders. Yet, survival with these lesions is increasing as with rhabdomyosarcoma from all sites and is directly related to a well-planned and aggressive multidisciplinary program. We have seen 12 cases of pelvic rhabdomyosarcoma within the last seventeen years at this institution. These cases will be reviewed in regard to varying modes of therapy and survival. Our current therapeutic approach, based on national and local experience, will be presented.
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PMID:Changing concepts in management of pelvic rhabdomyosarcoma in children. 45 95

Pineal teratomas are relatively uncommon intracranial neoplasms. A rhabdomyosarcoma developed in a pineal teratoma in a 14-year-old boy and was rapidly fatal despite radiation therapy. This is the second reported case of a pineal teratoma giving rise to a rhabdomyosarcoma.
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PMID:Rhabdomyosarcoma arising in a pineal teratoma. 45 53

One boy, given radiotherapy and cytostatic drugs for a rhabdomyosarcoma died nine years later of acute leukaemia. A girl, who had received radiotherapy for an inoperable suprasellar tumour and also given cytostatic drugs, fell ill seven years later with a glioblastoma. Among a total of 750 children with malignant neoplasm observed by the authors, a permanent cure rate of about 50% is to be expected among about 300 children treated recently. If this rate applies to the entire Federal Republic of Germany, about 1,000 children are likely to be cured annually. In these circumstances it is likely that 80-160 children treated in any one year will develop a second neoplasm or leukaemia 20 years later, most commonly as a late sequela of the treatment.
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PMID:[Risk of a second malignant neoplasm after successful treatment of a malignant tumour in children (author's transl)]. 45 71

The clinical characteristics, surgical treatment, clinical grouping, chemotherapy, radiotherapy, complications and survival of paratesticular rhabdomyosarcoma are discussed. When the combined approach of radical inguinal orchiectomy, retroperitoneal node dissection, chemotherapy and radiotherapy when indicated is used a survivorship in excess of 80 per cent can be anticipated for all stages of disease. A combined multidisciplinary approach to this lesion is stressed to achieve improved survivorship in this previously lethal lesion.
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PMID:Paratesticular rhabdomyosarcoma in children. 45 95


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