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 children, 9 male and 13 female, with a non-orbital rhabdomyosarcoma of the head and neck, treated between 1970 and 1988, have been reviewed. Since 1972, treatment has consisted of combination chemotherapy, and where necessary radiotherapy and/or surgery. Complete clinical remission after initial chemotherapy was observed in 21 children. Six children were cured after primary treatment but 15 developed recurrent disease. Thirteen children had a parameningeal localized tumour, with eventual meningeal involvement in 5. Survival in this group was worse than in the non-meningeal group. Lymph node metastasis at first presentation (5 patients) had no influence on prognosis, whereas development of lymph node metastases during follow-up resulted in 100% mortality. All patients were retrospectively classified according to both the IRS-classification and TNM-descriptive system. No correlation with either system could be established. Fourteen of 15 children with recurrent disease were treated, 4 of whom were cured. Thus, 10 out of 22 (45%) children were long-term survivors.
 ...
PMID:Rhabdomyosarcoma of the head and neck in children. 138 2

In order to examine surgical factors predictive of fatal outcome in patients presenting with histologically verified rhabdomyosarcoma of the urinary bladder, we performed a retrospective analysis of cases presenting between the years 1970 and 1985 and treated by protocol. Twenty-five patients were identified and data were complete for univariate and multivariate analysis on all. Staging was done according to the criteria of the International Union Against Cancer (TNM). Median age at presentation was 14.7 years and 10 patients were younger than 10 years. Median follow-up was 4.8 years overall and 8.4 years in survivors. Four patients presented with involvement of regional lymph nodes and three with distant metastases. Complete surgical resection, defined as negative microscopic margins, was accomplished by total cystectomy in 14 patients, and partial cystectomy in two. In this group cystectomy was performed prior to chemotherapy and radiation in five and after in 10 (persistent disease). Three salvage cystectomies were performed in patients who recurred after initial complete responses to chemotherapy and radiation therapy. Thirteen patients received a median of 3,000 cGy (range, 1,800 to 5,000 cGy) of external beam pelvic irradiation, and two received brachytherapy. All patients received multiple agent chemotherapy according to either the T2 or T6 protocol. There are 11 disease-free survivors (44%) and 10 of these have been followed for more than 6 years. One patient is alive with disease 6.5 years after diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:Prognostic factors in bladder and bladder-prostate rhabdomyosarcoma. 226 60

In order to examine factors predictive of fatal outcome in children presenting with histologically confirmed extremity rhabdomyosarcoma, we performed a retrospective analysis of our institutional experience from 1970 to 1985. Thirty-five patients were identified and staged according to international criteria (TNM). Variables evaluated for their predictive effect on fatal outcome included (1) tumor invasiveness, (2) tumor size, (3) anatomic location of the primary, (4) regional lymph node involvement, (5) distant metastases at presentation, (6) complete surgical resection, (7) use of amputation, and (8) alveolar histologic subtype. Significant predictors of mortality included (1) tumor invasiveness (P less than or equal to .0001), (2) regional node involvement (P less than or equal to .0002), (3) distant metastases at the time of presentation (P less than or equal to .001), (4) alveolar histology (P less than or equal to .001), (5) size of primary (P less than or equal to .007), and (6) completeness of surgical resection (P less than or equal to .05). In multivariate analysis, local tumor invasiveness was the most important predictor of fatal outcome with an associated relative risk of 18. We conclude that local tumor invasiveness is the most important determinant of clinical stage.
 ...
PMID:Factors predictive of mortality in pediatric extremity rhabdomyosarcoma. 230 93

One hundred medical records of children with rhabdomyosarcoma were reviewed independently by ten European and American specialists primarily concerned with pediatric cancer patients. Each specialist assessed the stage of disease using a pre-treatment staging system (International Society of Pediatric Oncology-Union Internationale Contre le Cancer TNM [SIOP-UICC TNM] and a post-surgical staging system (Inter-group Rhabdomyosarcoma [IRS] grouping system). These two prognostic systems are based on different criteria, and there is no simple correspondence between them. The reliability of the two systems, based on patients' characteristics extracted from medical records, was similar and satisfactory (Kappa index: 0.78 and 0.82 with the TNM system and with the IRS system, respectively). Nevertheless, the IRS grouping system, which considers a primary method of treatment: Surgery, is dependent upon the surgeon, the institution, and the current concept of treatment. We believe that the TNM system provides the best description of extent of disease and should be used for determining therapeutic options and comparing therapeutic results.
 ...
PMID:An attempt to use a common staging system in rhabdomyosarcoma: a report of an international workshop initiated by the International Society of Pediatric Oncology (SIOP). 274 93

From 1970 to 1987, 34 patients younger than 22 years of age with extremity rhabdomyosarcoma were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC). All patients were treated according to protocols consisting of surgery, radiotherapy, and multiple drug chemotherapy. Sixteen of 34 patients survived, and 14 continue to be disease-free; the 5-year survival rate was 44%. A retrospective univariate analysis of data according to the TNM staging system was undertaken. Tumor invasion, regional nodal involvement, distant metastases, and alveolar histologic condition each had a significant impact on survival. However, in multivariate analysis, the stage of disease at diagnosis was the most important predictor of survival outcome.
 ...
PMID:Prognosis in childhood rhabdomyosarcoma of the extremity. 280 12

Records of 323 patients with TNM Stage M0 sarcoma of soft tissue treated by the Radiation Medicine Service of the Massachusetts General Hospital over a 14-year period were reviewed to study the incidence and the implication of regional lymph node involvement. Nineteen patients (5.9%) had evidence of sarcoma metastatic to draining lymph nodes, zero of 63 (0%) were Grade 1 sarcomas, two of 118 (2%) were Grade 2, and 17 of 142 (12%) were Grade 3 sarcomas. Among patients with Grade 3 sarcomas, rhabdomyosarcoma (five of 14), vascular sarcoma (two of five), and epithelioid sarcoma (four of five) were associated with a higher incidence of lymph node involvement than synovial sarcomas (zero of four), fibrosarcomas (zero of 16), malignant fibrohistiocytomas (one of 29), neurofibrosarcomas (one of eight), liposarcomas (one of 14), and leiomyosarcomas (one of ten). From the 19 patients who had evidence of metastatic nodes, six (32%) were alive more than 58 months after the treatment of the nodes; four of six patients were without further tumor. The data of this study are compared with those cited in a review of the literature.
 ...
PMID:Lymph nodes as sites of metastases from sarcomas of soft tissue. 330 55

From 1964 to 1978, 313 patients, 192 males and 121 females, with soft tissue sarcoma underwent surgery in our hospital. All patients have been followed up for over 5 years. One hundred ninety patients (60.7%) had recurrence of the tumor after previous surgery. The tumor was located in the head in 55 cases, in the trunk in 136, and in the extremities in 122 cases. Regional lymph node metastases occurred in 23 patients (7.4%). Fibrosarcoma, neurofibrosarcoma, and rhabdomyosarcoma were the types most commonly encountered in this series. The 5-year survival rate, according to the extent of surgical resection for 117 patients with local resection, was 47.9%; for 75 patients with wide local resection, 62.7%; for 43 patients with amputation, 18.6%; and for 78 patients with local resection combined with irradiation, 48.7%. For the whole series, the 5- and 10-year survival rates were 47.6% and 35.8%, respectively. The 5-year survival rates of stages I, II, III, and IV (TNM classification) were 60%, 46.9%, 40%, and 22.2%, respectively.
 ...
PMID:Surgical management of soft tissue sarcomas, with an analysis of 313 cases. 335 24

From 1964 to 1978, 313 patients with soft tissue sarcoma were treated in our hospital by resection and followed over 5 years. 192 patients were males and 121 were females. The sex ratio was 1.5:1. In the 190 cases who developed postoperative recurrence, 55 were in the head and neck, 136 in the trunk and 122 in the limbs and 23 (7.4%) as metastasis in the lymph nodes. By pathology, the common types were fibrosarcoma (86 cases), neurofibrosarcoma (66 cases) and rhabdomyosarcoma (32 cases). All the 313 patients were treated by resection, the overall 5 and 10 year survival rates were 47.6% and 35.8%. The 5 year survival rate was 47.9% in the 117 patients treated by local resection, 62.7% in the 75 by extensive resection, 18.6% in the 43 by amputation and 48.7% in the 78 by combination of resection and irradiation. According to the UICC TNM staging system, the 5 year survival was 60% in stage I, 46.9% in stage II, 40% in stage III and 22.2% in stage IV. In this series, discussion of the treatment modalities and prognosis factors in emphasized.
 ...
PMID:[Surgical management of soft tissue sarcoma]. 358 18

Seventy-four patients with rhabdomyosarcoma were initially staged according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping classification and then retrospectively using a TNM staging system based on the initial clinical extent of disease. The TNM system includes T1, tumor confined to site or organ of origin; T2, regional extension beyond the site of origin; N0, normal lymph nodes; N1, lymph nodes containing tumor; M0, no evidence of metastases; and M1, distant metastases. All patients received combination chemotherapy, and more than 90% received radiation therapy as part of their initial treatment program with curative intent. Fifty-three of 74 patients (72%) were group III according to the IRS system, indicating unresectable or gross residual tumor. A more uniform distribution was achieved using the TNM system. Freedom from relapse (FFR) was 43% and the actuarial survival rate was 47% for the entire study group at 10 years. All but one relapse occurred within 3 years of initial diagnosis, and only three of 38 relapsed patients were salvaged. All TNM stage I patients are surviving disease free. Among patients having stages II, III, and IV disease by the TNM system, FFR was 53%, 26%, and 11%, and the survival rates were 47%, 36%, and 33%, respectively. Thirty-two of 74 patients (43%) had evidence of lymph node involvement at presentation, and 28 (88%) of these had primary lesions that extended beyond the site of origin (T2 primary). Histologic subtype and primary site had little impact on outcome in a multivariate analysis, and T stage was identified as the single most significant covariate correlated with survival; a model composed of both T stage and M stage was the best one for predicting relapse. The presented data support a study using a prospectively assigned TNM staging system based on the initial clinical extent of disease for use in future therapeutic trials.
 ...
PMID:Rhabdomyosarcoma: the Stanford experience using a TNM staging system. 395 Jun 76

The TNM staging system is a modus for diagnosis and treatment in which T is the extent of the tumor involvement, N is lymph node involvement, and M is the metastases; this system is supplemented with a histologic malignancy grade. Staging systems identify specific prognostic factors with which to predict clinical outcome. Staging systems are useful for assigning treatment priorities, determining the role of adjuvant therapies, and evaluating clinical investigations. Unfortunately, no universally accepted staging system for soft-tissue sarcomas exists. This is related to the relatively low incidence of sarcomas, the unique and unpredictable behavior of sarcomas, significant disagreement regarding histogenesis and grading, and lack of consensus regarding the value of various prognostic factors. In adults, the two most commonly used staging systems are those developed by the American Joint Committee on Cancer and by Enneking. In children, the Intergroup Rhabdomyosarcoma Study and the International Union Against Cancer have described the systems most commonly used. These systems for soft-tissue sarcomas rely on an ability to accurately determine both the local and distant extent of disease. Advances in the field of computed tomography and magnetic resonance imaging have made this possible. It is likely that a staging system based upon a more sophisticated understanding of the basic biology of sarcomas will become available.
 ...
PMID:Principles of staging of soft-tissue sarcomas. 847 13


1 2 Next >>