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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Prestudy patient characteristics and specific therapy of all eligible patients with rhabdomyosarcoma entered into Intergroup Rhabdomyosarcoma (RMS) Studies I (IRS-I) (1972 to 1978, n = 686) or II (IRS-II) (1978 to 1984, n = 1,002) were examined for their relationship to survival within each of the four clinical groups using univariate and multivariate analyses. The estimated survival at 5 years from the start of treatment was 56% in IRS-I and 62% in IRS-II (P = .006). The largest survival difference between studies was in patients with group III tumors (52% v 65%). The clinical group was the most important patient characteristic related to survival in both studies. Survival progressively decreased for patients from clinical group I (localized disease, completely resected) to group IV (metastatic disease at the onset). In clinical group I, the only patient characteristic consistently related to survival was histology. Patients with alveolar tumors had the poorest survival, while those with botryoid/embryonal lesions had the best survival. In clinical group II, no characteristic was consistently related to survival. In clinical group III, an orbital primary site was associated with a favorable survival. In clinical group IV, patients with genitourinary tumors had a significant survival advantage. Use of disease-free survival as an end point gave very similar results. This information, from the largest available data base on prognostic indicators in childhood RMS in the context of aggressive multimodal therapies, is being used to plan therapy in the forthcoming study (IRS-IV).
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PMID:Prognosis in children with rhabdomyosarcoma: a report of the intergroup rhabdomyosarcoma studies I and II. Intergroup Rhabdomyosarcoma Committee. 221 11

Rhabdomyosarcoma, the most common soft tissue sarcoma of childhood, involves the temporal bone in approximately 7% of reported cases. Until recently, the outcome of this disease was always fatal. The recent Intergroup Rhabdomyosarcoma Study Group (IRS-I) reported on the efficacy of multimodality therapy consisting of multiagent chemotherapy, radiation, and surgical resection when indicated. Twelve patients with rhabdomyosarcoma involving the temporal bone were treated between 1966 and 1988. Three patients were treated according to the IRS-I protocols and the remaining nine patients received various combinations of treatment modalities. Ten patients succumbed to their disease, most with distant metastases or intracranial extension. Two patients are alive; one at 5 1/2 years and one at 19 years. It is apparent that, although survival for rhabdomyosarcoma in general has improved with the use of IRS-I protocols, prognosis remains poor for disease involving the temporal bone and other parameningeal sites.
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PMID:Rhabdomyosarcoma of the ear and temporal bone. 281 61

The patterns of disease extent, response to treatment, and survival rates were examined in 129 children registered as metastatic (Group IV) soft-tissue sarcoma patients on the Intergroup Rhabdomyosarcoma Study (IRS)-I protocol from November 1972 through October 1978. The patients' ages at diagnosis ranged from 3 months to 21 years (median, 10.5 years); 72 were male patients and 57 were female patients (male-to-female ratio, 1.3:1). The tumor arose in an extremity in 34 patients, the head and neck (exclusive of the orbit) in 27, the genitourinary tract in 25, the retroperitoneum or pelvis in 14, the trunk in 12, the intrathoracic region in nine, the hepatobiliary system in four, the perineum in two, and the orbit in two patients. The histologic types of soft-tissue sarcoma confirmed by the pathology review committee were embryonal rhabdomyosarcoma in 68 patients, alveolar rhabdomyosarcoma in 41, undifferentiated sarcoma in 14, embryonal-botryoid in two, pleomorphic in one patient, and other sarcoma in three patients. Of 105 patients who were confirmed in Group IV and had complete information concerning sites of metastatic deposits, 53 patients presented with metastases restricted to only one anatomic region or tissue; the other 52 had diffuse metastases. The most common sites of distant tumor deposits were the lungs, bone marrow, and other soft tissues. After treatment with vincristine, dactinomycin, and cyclophosphamide with or without Adriamycin (Adria Laboratories, Inc., Columbus, OH) and radiation therapy, 65 (50%) of the 129 eligible patients achieved complete disappearance of all detectable tumor. The likelihood of achieving and maintaining a complete response for 2 years was highest among patients whose tumors originated in the genitourinary tract, retroperitoneum-pelvis, or the hepatobiliary or perineum regions. Thirteen of these 45 children (29%) are alive and disease-free at a minimum of 2 years after initiation of treatment. Only 10 of the other 84 (8%) are surviving disease-free at 2 years (P = 0.02). The presence of restricted rather than diffuse metastases at diagnosis (P = 0.02) was the only other characteristic significantly related to long-term complete remissions. One hundred and two patients (79%) died at a median of 47 weeks after diagnosis, 92 from sarcoma and ten from other causes.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I. 284 74

The results of treatment of 686, previously untreated patients younger than 21 years with rhabdomyosarcoma or undifferentiated sarcoma, who were entered on Intergroup Rhabdomyosarcoma Study-I (IRS-I) were analyzed after a minimum potential follow-up time of 7 years. Patients in Clinical Group I (localized disease, completely resected) were randomized to receive either vincristine, dactinomycin, and cyclophosphamide (VAC) or VAC + radiation. At 5 years, approximately 80% of patients given either treatment were still disease-free and there was no significant difference between treatments in the overall percentages of patients surviving of 93% and 81%, respectively (P = 0.67). Patients in Clinical Group II (regional disease, grossly resected) were randomized to receive either vincristine and dactinomycin (VA) + radiation or VAC + radiation. At 5 years, 72% and 65% of the patients, respectively, were disease-free and there was no evidence of a difference between treatments (P = 0.46). The overall survival percentage at 5 years was approximately 72% for both treatments. Patients in Clinical Groups III (gross residual disease after surgery) and IV (metastatic disease) were randomized to receive either "pulse" VAC + radiation or "pulse" VAC + Adriamycin (doxorubicin) + radiation. The complete remission (CR) rate was 69% in Clinical Group III and 50% in IV, with no statistically significant difference in CR rates between treatments in either group. Those who achieved a CR had a nearly 60% chance of staying in remission for 5 years in Clinical Group III compared with approximately 30% in Clinical Group IV. The overall survival percentage at 5 years was 52% in Clinical Group III compared to 20% in Clinical Group IV (P less than 0.0001). The 5-year survival percentage for the entire cohort of 686 patients was 55%. Survival after relapse was poor, being 32% at 1 year and 17% at 2 years. The risk of distant metastasis was much greater than the risk of local recurrence within each clinical group, and there was no evidence of differing types of relapses between treatments. Primary tumors of the orbit and genitourinary tract carried the best prognosis, whereas tumors of the retroperitoneum had the worst prognosis. The authors conclude that for the therapeutic regimens evaluated there was no therapeutic advantage to including radiation in the treatment of Clinical Group I disease, or cyclophosphamide given as a daily low-dose oral regimen in the treatment of Clinical Group II disease or Adriamycin in the treatment of Clinical Groups III and IV diseases.
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PMID:The Intergroup Rhabdomyosarcoma Study-I. A final report. 327 86

A review of 1415 patients without distant metastasis from the Intergroup Rhabdomyosarcoma Study (IRS) I and II revealed an overall 10% incidence of identified lymphatic spread at diagnosis, whereas 81 of 592 children with localized rhabdomyosarcoma who had grossly complete resection (and therefore with more complete pathologic data) had histologically proven lymphatic spread (14%). The percentage of patients in this latter group with nodal metastases was highest for the prostate (41%), paratesticular sites (26%), and genitourinary sites overall (24%). Sites with a small percentage of proven lymphatic involvement were the orbit (0%), nonorbital head and neck sites (7%), and truncal sites (3%), whereas the percentage of patients with nodal metastases from extremity lesions was 12%. The primary tumor mean diameter was significantly larger in the group with nodal metastases, but there was no evidence of a relationship between lymphatic spread and age, sex, or histologic subtype. Patients with lymph node metastases who had resection had a poorer survival rate (logrank P value = 0.001), with a 3-year survival estimate of 54%, compared with 78% for patients without lymphatic metastases. Patients with extremity lesions and positive lymph nodes also did poorly when compared with patients with normal nodes (P = 0.006), and a similar observation was made for patients with paratesticular sarcoma (P = 0.06).
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PMID:Lymphatic metastases with childhood rhabdomyosarcoma. A report from the Intergroup Rhabdomyosarcoma Study. 329 2

This article reports on the diagnostic features, radiographic findings, staging, operative management, treatments, and treatment results for 95 children (mean age, 10 years) with paratesticular sarcoma; they were treated on the Intergroup Rhabdomyosarcoma Study (IRS) I and II protocols. Embryonal rhabdomyosarcoma (RMS) was the most common histologic subtype (97% of cases). Patients were randomly assigned to receive various therapeutic regimens according to IRS protocols. Among 81 patients (85%) with localized disease, most (57) were in Clinical Group I (localized, completely excised tumors), and 20 were in Group II (gross excision with tumor-involved, regional retroperitoneal lymph nodes or microscopic residual). Only four were in Group III (gross residual disease in the primary site or retroperitoneal lymph nodes). Three of them achieved a complete response (CR) after induction therapy and two then relapsed. The relapse-free survival (RFS) estimates at 3 years from diagnosis were 93% in Group I and 90% in Group II. Distant metastases were present at diagnosis in only 14 patients. Twelve of them achieved CR, but four subsequently relapsed; their 3-year RFS estimate was 67%. Overall, survival rates among the 95 children were excellent (89% at 3 years) compared to the entire IRS series (63%). Treatment with radical orchidectomy and chemotherapy was sufficient for Group I patients. Orchidectomy, chemotherapy, and radiation therapy were highly effective in Group II patients and in a large proportion of those with more advanced tumors. Recommendations for the diagnostic examinations and management of future patients with paratesticular sarcoma are given.
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PMID:Paratesticular sarcoma in childhood and adolescence. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1973-1983. 344 Feb 40

The need for a pretreatment staging system to accurately assess programs of therapy for the various sites and stages of childhood rhabdomyosarcoma has become apparent as detailed analyses of many factors affecting prognosis and treatment choices have been accomplished through the national cooperative trial, the Intergroup Rhabdomyosarcoma Study (IRS). Initiated in 1972, the IRS has thus far used a clinicopathologic grouping system that is based heavily on therapeutic decisions, particularly on whether or not excision is accomplished and the extent of such an operation. The major problem with this and several other staging classifications now in use is that they depend on pathologic data obtained after surgical treatment has been initiated or rejected. In addition, they do not consider histologic variations of this neoplasm which may be important in estimating prognosis. The large body of clinical data now accumulated in the IRS has provided an excellent opportunity for developing a database for evaluating the International Union Against Cancer (UICC) pretreatment staging system and also the potential for using histologic categories in the staging process. The records of 505 eligible patients entered into the IRS between 1978 and 1982 were used to determine the prognostic impact of a number of pretreatment factors. These included local invasiveness of the primary neoplasm on clinical examination, tumor size, clinical status of regional nodes, clinical or radiologic evidence of distant metastases, and favorable or unfavorable histologic categories. A retrospective assessment of the relationship of these pretreatment observations to survival experience has been carried out. This retrospective study indicates definite prognostic significance for all of the individual factors used on the UICC system except clinical status of regional nodes. Also, these data serve as a basis for considering the possibility of including favorable v unfavorable histology in the pretreatment staging system now being tested prospectively in the ongoing IRS protocols.
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PMID:Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study (IRS-II). 354 38

One hundred one eligible children with soft-tissue sarcomas arising within the retroperitoneal space have been registered on Intergroup Rhabdomyosarcoma Study Committee (IRS) studies I and II and followed for at least 2 years or until death. The most common presenting symptoms and signs were pain in the abdomen or lower extremities, and/or an abdominal mass, usually noted by a parent or a physician seeing the child for other complaints. The median age at diagnosis was 6.5 years and the sex ratio (M/F) 1.7:1. Histologic types were embryonal or botryoid rhabdomyosarcoma (RMS) in 58 patients, alveolar RMS in 8, pleomorphic RMS in 2, undifferentiated sarcoma in 20, extraosseous Ewing's sarcoma in 4 and unspecified sarcoma in 9. Median tumor size was 10 cm, significantly larger than the 7.5 cm noted in the IRS studies as a whole (P less than 0.05). One patient had complete tumor removal (Group I); 12 had grossly complete removal with microscopic residual tumor (Group II). Fifty-one patients had residual local tumor after biopsy or partial resection (Group III) and 37 patients had distant metastases at diagnosis (Group IV). Treatment included surgery, radiation therapy (RT) and combination chemotherapy with vincristine and actinomycin D with or without cyclophosphamide and Adriamycin (doxorubicin) according to protocol. Thirty-nine of 99 patients (39%) had major difficulties in the delivery of specified RT. Seventy patients received sufficient therapy to be evaluable for treatment response. Forty-one (58%) achieved a complete remission and 16 (23%) achieved partial remission. Twenty-four of 41 children (59%) achieving a CR have relapsed. The proportion of children who remained relapse-free at 2 and 3 years of follow-up was 44% or 42%, respectively. Overall, 40 children have developed recurrent sarcoma and the median disease-free interval and overall survival times were 54 and 88 weeks, respectively. Most children experienced severe myelosuppression; there were three early deaths from infection which occurred during granulocytopenic periods. One third of the patients experienced cystitis. Children with soft-tissue sarcomas arising in the retroperitoneal space have a poor prognosis associated with large tumors which, because of location and/or extent (Groups III or IV), are unresectable and difficult to treat.
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PMID:Soft tissue sarcomas arising in the retroperitoneal space in children. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. 389 67

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.
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PMID:Rhabdomyosarcoma: the Stanford experience using a TNM staging system. 395 Jun 76

Four hundred and twenty-three children with newly-diagnosed soft-tissue sarcoma were treated on the Intergroup Rhabdomyosarcoma Study I from November 1972 through December 1976. After institution of therapy, 341/423 (80.6%) achieved a complete response (no detectable tumor), but 115/341 (33.7%) subsequently relapsed. The types and efficacy of management after relapse were ascertained by questionnaire. Information sufficient for critical analysis was obtained for 98/115 patients (85%). Thirteen of the 98 patients had an isolated local recurrence; the other 85 developed metastases in regional, distant, or multiple sites simultaneously. Patients with distant metastases at diagnosis were most likely to develop recurrence, as were those with sarcoma arising in the perineum-anus, retroperitoneum-pelvis, gastrointestinal tract, or extremity. Those with tumor primary in the orbit or genitourinary tract had the lowest recurrence rate, as did those whose tumor histology was nonalveolar. Individualized treatment was given to 90 of the 98 patients after relapse, and a second complete response was achieved in 33 of them (37%). However, only five of the 90 patients (5.5%) are currently tumor-free. The outlook is poor when recurrence develops in patients still receiving chemotherapy; only two of 74 such patients survive, compared to three of 16 who have relapsed after cessation of treatment. Relapse after initial complete response to therapy has a poor prognosis, regardless of site of recurrence. Although effective retrieval therapy can prolong survival, only 2 of 12 patients (17%) with local recurrence and three of 78 patients (4%) with metastatic recurrence remain free of disease.
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PMID:Prognosis of children with soft tissue sarcoma who relapse after achieving a complete response. A report from the Intergroup Rhabdomyosarcoma Study I. 685 May 44


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