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At least 19 recordsLinked to original sources
PubMed1979

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.

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.

Adolescent↗
PubMed1978

Incidence of meningeal involvement by rhabdomyosarcoma of the head and neck in children: a report of the Intergroup Rhabdomyosarcoma Study (IRS).

141 patients with embryonal rhabdomyosarcoma (RMS) of the head and neck are reviewed. 57/141 had lesions of para-meningeal sites. 20/57 (35%) developed evidence of direct meningeal extension. 18/20 (90%) died of this complication. Radiation portals and doses were limited in 42% and 32%, respectively. All patients had chemotherapy for 6 weeks prior to radiation. The significance of the adequacy of radiation factors and the timing of chemotherapy are reviewed. Recommendations for managing these patients include earlier use of radiation and increased coverage of adjacent meninges by radiation including total craniospinal axis radiation when brain meningeal involvement exists.

Child↗
PubMed1979

Changing concepts in management of pelvic rhabdomyosarcoma in children.

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.

Child↗
PubMed1978

Rhabdomyosarcomas of the uterus.

The authors report the clinicopathologic findings in four cases of adult women with rhabdomyosarcomas that originated in the endometrium or cervix, or both. All tumors were pure sarcomas and not components of carcinosarcomas or malignant mixed mesodermal tumors. In two patients, the tumors were typical botryoid embryonal rhabdomyosarcomas. Both patients were alive and well following combined treatments with surgery and chemotherapy or irradiation 3.0 and 2.7 years after operation, respectively, although one had a solitary pulmonary metastasis resected. The other two women had pleomorphic rhabdomyosarcomas, and both died rapidly of widespread tumor. Combination therapy rather than surgery alone probably should be used initially for patients with rhabdomyosarcomas of the uterus.

Adult↗
PubMed1979

Primary rhabdomyosarcoma of the tentorium with peculiar angiographic findings.

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.

Adolescent↗
PubMed1978

Primary tumors of the external and middle ear. III. A clinicopathologic study of embryonal rhabdomyosarcoma.

Embryonal rhabdomyosarcoma of the middle ear-mastoid is a highly lethal neoplasm of childhood in most cases, which clinically simulates a chronic infection. Extensive hemorrhage and necrosis in the biopsy specimen may obscure and often delay the diagnosis. Two of the six embryonal rhabdomyosarcomas in this study showed histologic evidence of differentiation. These same two patients were the only long-term survivors. Similar examples of "differentiated" embryonal rhabdomyosarcomas of the middle ear-mastoid with prolonged survival have been recorded individually in the literature. Therapeutic implications of this finding have been discussed.

Child↗
PubMed1977

[Rhabdomyosarcoma of the skin].

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.

Aged↗
PubMed1979

[Change in the karyotypic structure of mouse and rat rhabdomyosarcomas on their transplantation into the anterior chamber of the eye].

A study has been made of 7 transplatable lines of mice rhabdomyosarcomas and one line of rat rhabdomyosarcoma during their transplantation into the eye anterior chamber subcutaneous tissue. In all, 10 subcutaneous transplants and 15 transplants into the eye anterior chamber (EAC) were examined. Etanol fixed print smears were subjected to the Feulgen reaction to measure the DNA content using a cytophotometer MCPhU-1; 100 cells being measured in each transplant. In the majority of the EAC transplants, a statistically significant decrease of the karyotypic variability was found in additionto the augmentation to the diploid cell ratio as compared to subcutaneously proliferating populations of the same tumour lines. In some cases EAC transplants displayed exclusively diploid (periploid) populations of tumour myoblasts. Shifts in the karyotypic structure of populations towards diploidy, revealed during the cultivation of transplantable rhabdomyosarcomas, may be regarded as a phenomenon of the "karyotypical normalization" of tumour cells. The disappearance or sharp decrease of tetraploid or hypertetraploid classes of cells in EAC transplants may be due to the increase of their selective value in condition of immunological privilege of diploid, karyotypically normal cells, and of reduction of the genome mutation frequency in a diploid fraction of tumor myoblast populations.

Animals↗
PubMed2026

DNA Methylation Profiling of Pediatric Ectomesenchymoma Supports Embryonal Rhabdomyosarcoma-Like Epigenetic Identity.

Ectomesenchymoma is a rare, biphenotypic pediatric tumor combining rhabdomyoblastic and neuroectodermal differentiation. We characterize two novel cases through integrated genomics and the first report of genome-wide DNA methylation profiling. Both tumors harbored RAS-pathway mutations (HRAS p.Gly13Arg; NRAS p.Gln61His). Methylation analysis, including microdissected components, consistently aligned ectomesenchymoma with the embryonal rhabdomyosarcoma superfamily, revealing a shared myogenic epigenetic program despite neural differentiation. Shared copy-number profiles across distinct histological regions supported a monoclonal origin. Overall, our data support a close biological relationship between ectomesenchymoma and embryonal rhabdomyosarcoma and indicate that RAS-pathway testing and methylation profiling can significantly refine diagnostic precision.

Humans↗
PubMed1978

Ultrastructural observations on the histogenesis of alveolar rhabdomyosarcoma.

Ultrastructural examination of an alveolar rhabdomyosarcoma revealed that the bulk of the tumor was composed of small polygonal cells containing polyribosomes, short strands of rough endoplasmic reticulum, and variable amounts of glycogen; the cells were joined by small desmosome-like structures. Similar cells, as well as a variety of larger forms which appeared to be derived from the small cells, were observed along the septa; the larger forms developed a copious cytoplasm and occasionally produced myofilaments. Some of these cells were separated from the septa by basement membranes. Giant cells were also present in the alveolar spaces; these formed neither basement membranes nor myofilaments. The septa themselves were composed largely of collagen and fibroblasts. It is suggested that the commonly proposed correspondence of alveolar rhabdomyosarcoma to the myotube stage of muscle differentiation is incorrect; if anything, the overall pattern of the tumor is epithelial-like, and may correspond to the organization of mesenchymal cells at the stage of somite differentiation. The resemblance of the masses of small tumor cells to the cells of Ewing's sarcoma is discussed.

Basement Membrane↗
PubMed1978

Paratesticular rhabdomyosarcoma in childhood.

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.

Adolescent↗
PubMed1979

Rhabdomyosarcoma arising in a pineal teratoma.

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.

Adolescent↗
PubMed1978

Genetics of susceptibility in the platyfish/swordtail tumor system to develop fibrosarcoma and rhabdomyosarcoma following treatment with N-methyl-N-nitrosourea (MNU).

About 7000 animals of 65 different genotypes of the xiphophorine fish were treated with the direct acting chemical carcinogen N-methyl-N-nitrosourea (MNU; 10(-3)M; four times for 1 hour in two week intervals), in order to find out whether the susceptibility for development of fibrosarcomas and rhabdomyosarcomas is directly related to the genotype. A genotype specific susceptibility was found, ranging from zero to about nine percent. The highest susceptibles were found in certain backcross hybrids involving P.variatus/X.helleri-hybrids and X.helleri, as the recurrent parent. These genotypes were further analysed. Both P.variatus and X.helleri, as werr as their F1 proved to be insusceptible; while from the three backcrosses, which were tested, namely the BC1, BC4 and BC15, both the BC1, and the BC4, were susceptible, but the BC15 was insusceptible. The results are interpreted on the basis of the assumption that the differential susceptibility is a function of the type of control of a tumor gene (Tu-Fi-Rh) endogenous to P.variatus and involved in development of fibrosarcomas and rhabdomyosarcomas. Accordingly, in P.variatus and in the F1 the Tu-Fi-Rh is controlled by repressing genes (R-genes) linked as well as non-linked to Tu-Fi-Rh; because simultaneous mutation of both R-genes following treatment with MNU is an extremely unlikely event, these genotypes have an extremely low susceptibility. By contrast, in the BC1 and the BC4 the non-linked R-genes become eliminated and only the linked R-gene remains for repression of Tu-Fi-Rh; this condition confers a high degree of susceptibility, because one single mutation may lead to impairment of the R-gene and to Tu-Fi-Rh-mediated formulation of fibrosarcomas and rhabdomysarcomas. In the BC15, furthermore, also the Tu-Fi-Rh has become eliminated, resulting in a loss of the susceptibility. The results suggest that in the xiphophophorine fish the susceptibility for responding to MNU-treatment with the development of fibrosarcomas and rhabdomysarcomas is related directly to the genotype.

Age Factors↗
PubMed1979

Intrascrotal rhabdomyosarcoma.

Rhabdomyosarcoma is the most frequent spermatic cord tumor of infants, children, and young adults, but also occurs as a primary tumor in the testis, epididumis, and testicular tunics. In the last fifteen years, 7 patients with intrascrotal rhabdomyosarcoma were treated at our institution, and an additional 155 cases were found on review of the English literature. On the basis of these 162 cases, incidence and survival statistics were calculated with particular attention to employed forms of therapy. An over-all survival of greater than 73 per cent should be obtainable with proper utilization of surgery, radiation therapy, and combination chemotherapy.

Adolescent↗
PubMed1979

A therapeutic approach to childhood pelvic rhabdomyosarcoma without pelvic exenteration.

A therapeutic approach using chemotherapy rather than radical surgery as the initial modality of treatment was employed in 13 children with pelvic rhabdomyosarcoma. One patient failed to respond to chemotherapy and required anterior pelvic exenteration. Eight patients are alive and free of disease 24 to 96 months after diagnosis. Limited surgery for minimal residual disease was used in two patients. Radiation therapy at lower than conventional doses was used in seven of eight patients. This experience supports a therapeutic approach to childhood pelvic rhabdomyosarcoma with an initial chemotherapy regimen, resulting in the use of surgical procedures less detrimental to pelvic organs without compromising survival.

Adolescent↗
PubMed1979

The treatment of orbital rhabdomyosarcoma with irradiation and chemotherapy.

Fifty-eight patients with orbital rhabdomyosarcoma were treated with irradiation alone (25) or irradiation and chemotherapy (33) with follow-ups of 6 months to 14 years (mean 5.2 yr). At present, 74% are alive and 26% have died. Local control of the tumor was accomplished in 91% of cases. When local sinuses were invaded the survival rate was 55%. Chemotherapy appears to be of greatest value when disease is limited to the orbit. Irradiation or irradiation and chemotherapy should now be the treatment of choice for orbital rhabdomyosarcoma.

Adolescent↗
PubMed1978

Rhabdomyosarcoma arising within a cystosarcoma phyllodes. Case report and review of the literature.

Rhabdomyosarcoma of the breast is a rare tumor that is characterized by its rapid growth, large size, and poor prognosis. It most often presents as a pure neoplasm and infrequently as the stromal component of a cystosarcoma phyllodes. The clinical and pathological features of a cystosarcoma with a rhabdomyosarcomatous stroma occurring in a 45-year-old woman are presented, and the results are discussed in reference to the 24 published cases of mammary rhabdomyosarcoma. The patient was treated by radical mastectomy and died 2.5 years later with pulmonary and cerebral metastases. Though two long-term survivors have been reported, cures are infrequent and no form of therapy has been uniformly successful.

Adolescent↗
PubMed1979

Rhabdomyosarcoma of infancy and childhood. Problems of morphologic classification.

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.

Adolescent↗