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)

A retrospective study was conducted on 126 patients with rhabdomyosarcoma enrolled in the Japanese Pediatric Tumor Registry between 1971 and 1980. The age of the patients ranged from less than 1 year to 15 years, and the male to female ratio was 1.3:1.0. Primary sites included the pelvis (37.3 per cent), abdomen (23.8 per cent), head and neck (21.4 per cent), thorax (9.5 per cent), extremities (6.4 per cent) and unknown (1.6 per cent). According to the staging system of the Japanese Society of Pediatric Surgeons, the extent of disease was classified into stages Ia (26.2 per cent of the total); Ib (14.6 per cent); II (12.6 per cent), III (29.1 per cent) and IV (17.5 per cent). The clinical stage was significantly correlated with survival outcome in this series (p less than 0.05). Age, sex, histology and primary site per se had no independent prognostic influence on tumor-free survival. With regard to treatment modalities, surgery was performed in 94.0 per cent of the patients, and radiotherapy at a mean dose of 37 Gy, and/or multi-agent chemotherapy in 41.7 per cent and 80.0 per cent, respectively. The patients who underwent total excision had a better survival outcome than those who did not (p less than 0.05). Combination chemotherapy such as VAC was more commonly administered in the latter study period. The overall 2-year tumor-free survival rate (2YTFSR) significantly improved from 24.0 per cent in the former period between 1971 and 1975 to 48.7 per cent in the latter period between 1976 and 1980 (p less than 0.05).
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PMID:The clinical features and prognosis of rhabdomyosarcoma: follow-up studies on pediatric tumors from the Japanese Pediatric Tumor Registry 1971-1980. Part II. Committee of Malignant Tumors, Japanese Society of Pediatric Surgeons. 224 42

158 cases of the Cooperative Ewing's Sarcoma Trials (CESS 81/86), which have been documented at the Pediatric Tumor Registry, Kiel, were studied by conventional light microscopy and immunohistochemistry. There were 77 cases of typical Ewing's sarcoma with 70 cases being located in the skeleton and 7 in soft tissues. Of the 14 cases of atypical Ewing's sarcoma 7 cases each were localized in bone and in soft tissue, respectively. In contrast to typical Ewing's sarcoma, cells of atypical Ewing's sarcoma were larger and displayed more heterochromatin. Both, typical and atypical Ewing's sarcoma reacted positively for vimentin. Other stains were negative, notably the neuron specific enolase (NSE). In 55 cases a diagnosis of malignant peripheral neuroectodermal tumor (MPNT) was made. Histologically most of these tumors resembled atypical Ewing's sarcoma. By immunohistochemistry positive reactions were found for NSE, vimentin, protein S-100, neurofilaments and glial fibrillary acidic protein. In 3 cases a diagnosis of small cell osteosarcoma was made. There were 2 cases of undifferentiated sarcoma of bone, 2 cases of soft tissue sarcoma of undetermined histogenesis and 2 cases of rhabdomyosarcoma. Of the 4 tumors which could be investigated for response to polychemotherapy, 1 each corresponded to grade II and III, respectively, and 2 to grade IV according to the classification of histologic grade of regression established by Salzer-Kuntschik et al. (1983).
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PMID:[Cooperative Ewing's Sarcoma Studies 81/86: pathologico-anatomic and immunohistochemical findings and differential diagnosis of Ewing sarcoma]. 306 61

544 malignant soft tissue tumors have been collected at the Pediatric Tumor Registry in Kiel including 300 cases of rhabdomyosarcoma (55%). In 237 of the 300 cases the diagnosis is certain. Liposarcoma and malignant fibrous histiocytoma which are typical tumors of adult age are rare in our material. Among rhabdomyosarcomas embryonal rhabdomyosarcoma (eRMS) clearly predominates accounting for almost 72% of all rhabdomyosarcomas. Differentiation of tumor cells in eRMS may vary considerably. Therefore, three groups of eRMS were distinguished and analyzed for clinico-pathologic features: 1. Primitive eRMS with less than 10 rhabdomyoblasts. 2. Intermediate eRMS with 10-50% rhabdomyoblasts. 3. Well differentiated eRMS with greater than 50% rhabdomyoblasts. By immunohistochemistry, vimentin positive cells were found in all three groups. The number of desmin positive cells depended upon the grade of differentiation. Thus, there were more desmin positive cells in well differentiated eRMS. Primitive and well differentiated eRMS were predominantly located in the head and neck area, intermediate eRMS in the abdomen. Primitive eRMS were noted in higher stages than tumors of the other two groups. Response to chemotherapy as evaluated in the 7th week of treatment was better in well differentiated eRMS. Moreover, patients of this group achieved more often complete remission. It is concluded from the present study that differentiation in eRMS may have an influence on the clinical presentation and clinical course of the disease. Therefore, this question should be investigated in more detail in a larger prospective study.
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PMID:[Rhabdomyosarcoma: morphology and cellular differentiation]. 352 25

This study examines differences between cancer centers (CC) and noncancer centers (NCC) in terms of management procedures and outcomes for three pediatric solid tumors: Wilms' tumor (N = 147), rhabdomyosarcoma (N = 87), and medulloblastoma (N = 76). Data were derived for the period 1970-1979 from the population-based Greater Delaware Valley Pediatric Tumor Registry maintained at the Children's Cancer Research Center, which routinely collects data on all childhood neoplasms that occur in a 31-county region. Management measures reviewed included the degree to which important pretreatment evaluations were performed, types of therapy used, and extent of follow-up examinations conducted. Outcome variables were three-year disease-free survival and frequency of deaths related to complications of therapy. Differences in three-year disease-free survival between CC and NCC were noted for medulloblastoma (52% v 24%) and rhabdomyosarcoma (48% v 10%), but not for Wilms' tumor (79% v 68%). Among medulloblastoma patients, differences were detected in the frequency of pretreatment evaluations and in the therapy used. The principal management contrast found in rhabdomyosarcoma was that multiagent chemotherapy was used less often in NCC. Wilms' tumor patients were evaluated and treated similarly in the community versus the CC, except for some contrasts in the surgical approach and the frequency of follow-up for the detection of late complications.
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PMID:Influence of place of treatment on diagnosis, treatment, and survival in three pediatric solid tumors. 608 50

Incidence rates of cancer among children aged 0-14 for the period 1970-79 have been generated with the use of data from the Greater Delaware Valley (GDV) Pediatric Tumor Registry. This population-based registry covers a 31-county area and has a pediatric base population of 2 million. During the period, approximately 2,300 cases of childhood cancer were diagnosed in the region. Incidence rates for all histologic types combined are similar to rates from other large surveys conducted in the United States and Western Europe. However, certain histology-specific rates in the GDV vary by race. In the GDV nonwhites relative to whites have higher rates of Wilms' tumor, soft tissue sarcomas other than rhabdomyosarcoma, and retinoblastoma. These contrasts are supported by surveys in African populations showing relatively higher rates of these tumors among African black children. GDV whites exceed nonwhites in incidence of acute leukemia, neuroblastoma, and Ewing's sarcoma. African black children also experience low rates of these tumors. The frequency of central nervous system tumors is similar for GDV whites and nonwhites, despite reports of a rarity of these neoplasms in African blacks. Variations in incidence rates reveal population subgroups with particular tumor susceptibilities and may provide clues as to the relative influence of heredity and environment on patterns observed.
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PMID:Incidence of childhood cancer: experience of a decade in a population-based registry. 657 21

Rhabdomyosarcomas (RMS) and extraosseous Ewing's sarcomas (EOE) including malignant peripheral neuroectodermal tumors (MPNT) are the most frequent soft tissue malignancies of childhood. They account for 60.2% of 2.350 cases collected in the files of the Kiel Pediatric Tumor Registry. RMS: It is absolutely necessary to distinguish between embryonal (e) and alveolar (a) RMS, since these are two distinct tumor entities with significant differences in clinical presentation, morphology, molecular biology, cytogenetics and prognosis (Botryoid and spindle cell RMS are special variants of eRMS). The overall proportion of eRMS: aRMS is 2.4:1. Most cases of eRMS develop in the first 10 years of life (77.3%) while the age distribution of aRMS is almost constant in childhood and adolescence. Embryonal RMS exhibit a significantly higher proportion of male patients than aRMS (m:f = 1.72:1 vs. 1.06:1). A higher percentage of aRMS cases (25%) shows metastatic disease at the time of diagnosis than eRMS (8%), and the overall survival rates of aRMS are significantly lower even in localised disease (stage I-III) than in eRMS (59% vs. 76%; p < 0.002) (Data from the Cooperative Soft Tissue Sarcoma Study CWS). EOE: Despite proven histogenetic relationship (identical chromosomal rearrangements and fusion genes) the members of the Ewing's sarcoma (ES) family, classic ES and MPNT, whether osseous or extraosseous display significant differences in location, morphology and prognosis. Morphologically, MPNT and classic ES can be considered to be the extremes of a spectrum with overt neurodifferentiation in the former and lack of neural differentiation in the latter. Matched-pairs analysis of CWS EOE and MPNT cases show dramatically more unfavorable overall survival rates in MPNT than in EOE (45% vs. 67%).
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PMID:[Rhabdomyosarcoma and extraosseous Ewing's sarcoma]. 1009 21

Soft tissue malignancies in childhood and adolescence encompass a wide variety of histologically and genetically different tumor entities. In the files of the Kiel Pediatric Tumor Registry, 4,272 soft tissue malignancies were collected since 1977. Rhabdomyosarcomas are by far the most frequent sarcomas (44.6 % of the cases), followed in decreasing order of frequency by the family of Ewing tumors (peripheral primitive neuroectodermal tumors and extraosseous Ewing's sarcomas; altogether 22.3 %), malignant peripheral nerve sheath tumors (8.1 %), synovial sarcomas (5.0 %), leiomyosarcomas (3.2 %), fibrosarcomas (2.4 %), extrarenal malignant rhabdoid tumors (2.0 %), and alveolar soft tissue sarcomas (1.1 %). A further group (11.3 %) includes rare tumors, intermediate fibrohistiocytic tumors, and unclassified sarcomas. Embryonal rhabdomyosarcomas are 2.5 times more frequent than the alveolar rhabdomyosarcomas, which are prognostically unfavorable and located predominantly in the extremities and the trunk. With regard to clinical findings, histology, molecular biology and prognosis, embryonal and alveolar rhabdomyosarcomas have to be considered as two different tumor types. The family of Ewing tumors includes extraosseous Ewing's sarcoma and peripheral primitive neuroectodermal tumors (synonym: malignant peripheral neuroectodermal tumors), the former tumors without and the latter with neural differentiation. Many cases of infantile malignant peripheral nerve sheath tumors and infantile fibrosarcomas are low-grade malignancies and prognostically more favorable than their "adult" counterparts.
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PMID:[Soft tissue malignancies in childhood and adolescence. Pathology and clinical relevance based on data from the kiel pediatric tumor registry]. 1550 56