Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Intergroup Rhabdomyosarcoma Study, initiated in 1972, has admitted more than 700 patients with childhood rhabdomyosarcoma in a 6-year period. Although it was designed primarily to answer specific questions about various aspects of radiotherapy and chemotherapy, some lessons have been learned concerning the surgical approach to this disease. Operative resection, which is not always feasible, is determined chiefly by the clinical stage and anatomic site. Thus far, data for some anatomic sites indicated that total gross resection at some point in the treatment schedule achieved better results than did incomplete resection. A possible exception to this occurred when the orbit is the primary site. The incidence of lymphatic metastases from lesions in some anatomic sites (e.g., paratesticular, pelvic genito-urinary, and extremity) was higher than was previously appreciated. This finding will influence decisions concerning surgical biopsy or dissection of regional lymph nodes for many of these lesions. Differences in histology, particularly those relating to the alveolar type, indicated the need for a more aggressive approach to selected lesions, but we need more data to confirm this determination. Lastly, patients who initially received radiotherapy or chemotherapy to pelvic sites have had striking benefits, and this finding should encourage the development of schedules that specify operative resection of these lesions later in the program rather than as the initial treatment.
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PMID:Surgical lessons from the Intergroup Rhabdomyosarcoma Study. 702 87

Between 1972 and 1978, 780 patients were entered on the Intergroup Rhabdomyosarcoma Study. The results indicated that postoperative irradiation of the tumor bed was unnecessary when vincristine, dactinomycin, and cyclophosphamide (VAC) were given in combination for 2 years after excision of a localized tumor (group I). This drug combination was no better than vincristine combined with dactinomycin given to patients with grossly resected tumors (group II) who also received postoperative irradiation. Intensive chemotherapy regimens, "pulse" VAC alone or VAC combined with adriamycin followed by irradiation, were equally effective in advanced stages and induced tumor regression in 85% of the patients who had gross residual disease after surgery (group III) and in 68--74% of the patients with metastatic disease at diagnosis (group IV). Two-year relapse-free survival rates were projected to be 83% for group I, 72% for group II, 65% for group III, and 28% for group IV; the overall survival rates were higher for each group. Patients with local and distant recurrences had equally poor prognoses.
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PMID:The Intergroup Rhabdomyosarcoma Study: update, november 1978. 702 97

In 202 patients with rhabdomyosarcoma of the head and neck who registered in the first Intergroup Rhabdomyosarcoma Study, the primary lesions arose about the eye and orbit in 26%, in parameningeal sites in 46%, and in other head and neck areas in 28%. Histopathologically, 78% were embryonal-botryoid, 9% alveolar, 10% undifferentiated, and 3% extraosseous Ewing's types. Actual three-year relapse-free survival rates were calculated from data on 103 of these patients who were free of distant metastases at diagnosis and in whom follow-up had been completed for a three-year period. The actual relapse-free survival rates were 91% (21/23) for those with eye/orbit primaries, 46% (20/44) for those with parameningeal primaries, and 75% (27/36) for those with other head and neck sites affected. Among those with no clinical evidence of tumor activity at two years, 8% (6/75) had subsequent relapses.
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PMID:Three-year relapse-free survival rates in childhood rhabdomyosarcoma of the head and neck: report from the Intergroup Rhabdomyosarcoma Study. 707 38

Rhabdomyosarcomas (RMS) arising in the head and neck region, retroperitoneum, and perineum were considered together here because the usual surgical approach is incisional biopsy. Thus successful treatment of these neoplasms depends on effective radiation therapy and chemotherapy. From November 1972 through December 1976 the Intergroup Rhabdomyosarcoma Study accrued 127 patients with primary tumors in the head and neck, 34 with orbital tumors, 24 with sarcomas arising in the retroperitoneum-pelvis (no genitourinary), and 11 with perineal lesions. Results of treatment varied among these primary sites. Patients with orbital RMS had the best prognoses; 77% of them were free of disease, compared with a 51% disease-free rate in patients with nonorbital head and neck RMS. In this latter group, disease recurrence was evenly divided among local failure, distant metastases, and direct meningeal extension. Prognoses were similar for retroperitoneal tumors; 46% of such patients are currently free of disease. That the perineum was a rare site for RMS was fortunate because only 3 of 11 such patients (27%) are free of detectable disease now (January 1979). We concluded that the site of the primary tumor is an important prognostic variable in children with RMS.
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PMID:Special considerations related to primary site in rhabdomyosarcoma: experience of the Intergroup Rhabdomyosarcoma Study, 1972--76. 730 Aug 96

During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhabdomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, less than 4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.
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PMID:Rhabdomyosarcoma of the female urogenital tract. 733 62

The current management of pediatric vaginal and vulvar rhabdomyosarcoma (RMS) uses cytoreductive chemotherapy before surgical intervention. During the Intergroup Rhabdomyosarcoma Study (IRS)-III (1984 to 1988), 27 evaluable patients were entered on a preoperative chemotherapy protocol. Among 24 patients with vaginal primaries, 20 had an initial biopsy with gross residual (group III), 3 had resection with positive margins (group IIA) and 1 had metastatic disease (group IV). At subsequent surgery, 7 patients underwent partial or complete vaginectomy and 6 of them had no viable tumor identified in the specimen. Only 1 of these 7 patients underwent a cystectomy, whereas 5 underwent hysterectomy. Seventeen patients in this group have no evidence of disease 66 to 108 months after diagnosis; 2 died of chemotoxicity and 1 of unknown causes after achieving a complete response. Ten of these 17 patients were treated with biopsy and chemotherapy only. Four of these 10 had radiotherapy as well. All group IIA patients have no evidence of disease. The 1 group IV patient had rapidly progressive disease with early death. This primary chemotherapy protocol resulted in less need for operative intervention or irradiation compared with previous experience. There was no local recurrence, and 20 of 24 patients remain continuously relapse-free with no evidence of disease. All 3 patients with vulvar primaries were treated by wide local excision and chemotherapy and have no evidence of disease. Conservative surgical intervention for vaginal RMS with primary chemotherapy and adjunctive radiation when necessary appears to result in excellent disease-free survival.
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PMID:Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III. 747 27

Previous studies have suggested that women with uterine rhabdomyosarcomas (RMS) represent a distinct group of patients who present at an older age, are less responsive to treatment, and have a poorer prognosis than patients with vaginal RMS. During the Intergroup Rhabdomyosarcoma Study (IRS) III and the IRS IV pilot study, 14 patients were registered with uterine primary RMS. Three patients presented with cervical tumors that were completely removed (group 1). Eight patients had initial biopsies with gross residual disease (group 3), and 3 had metastatic disease at presentation (group 4). Of the 5 patients treated with primary chemotherapy or chemotherapy and radiation, 2 had delayed hysterectomy and vaginectomy, 1 had no further surgery, and 2 had exploratory laparotomy with no evidence of disease. There were no relapses or deaths in this group. One patient underwent initial resection of a broad ligament mass, experienced an early (3-week) recurrence of the mass while on chemotherapy, and progressed to developing distant metastases and death. Four patients died of chemotherapy toxicity or sepsis, one after achieving a complete response from chemotherapy and hysterectomy. This primary chemotherapy or chemotherapy and radiotherapy regimen resulted in 8 of 9 (89%) patients (not including those who died of chemotoxicity) surviving between 1.5 and 6 years without evidence of disease. Of the surviving patients, 2 had hysterectomy and vaginectomy, but pathological specimens showed only localized microscopic residual tumor. This report suggests that less vigorous operative resection may be possible in combination with primary chemotherapy when treating uterine rhabdomyosarcomas.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Conservative management of uterine pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III and IV pilot. 747 49

The administration of external beam radiation therapy (EBRT) has been an integral part of the successful treatment of childhood sarcomas. However, EBRT has severe late morbidity in the developing child. In an attempt to deliver adequate tumoricidal radiation while preserving bone growth and organ function, 13 children with diverse sarcomas were treated with high dose rate brachytherapy (HDR). Seven patients had rhabdomyosarcoma and six patients had other soft tissue sarcoma variants. All patients were treated with disease-appropriate chemotherapy, usually according to the intergroup Rhabdomyosarcoma Study. Eleven patients received fractionated 36 Gy HDR alone at a mean of 3.5 months from diagnosis. Two patients received 10-12.5 Gy intraoperative HDR brachytherapy and additional 27 Gy EBRT. Nine of 11 patients in first remission have had no recurrences. One died of recurrent pulmonary metastases. The other patient that did recur is disease-free 21 months post-recurrence. Two additional patients were treated with HDR after tumor recurrence. One patient with recurrent Ewing's sarcoma, relapsed and died. The second is disease free 3 months after autologous bone marrow transplant. Grade 1 morbidity occurred in 46%, Grade 2 in 15%, and Grade 3 in 8% of the children, while relatively good bone and organ growth was maintained. The combination of conservative surgery, chemotherapy, and HDR offers the potential for disease control in young children while preserving bone growth and organ function.
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PMID:High-dose-rate brachytherapy in childhood sarcomas: a local control strategy preserving bone growth and function. 756 9

Radioiodinated meta-iodobenzylguanidine (131I-MIBG) has been widely used for the diagnosis of neuroblastomas, pheochromocytomas, paragangliomas and medullary carcinomas of thyroid. We have developed a procedure for preparation of 131I-MIBG and studied its utility in diagnosis of primary and metastatic neural crest tumours. Studies were carried out in 54 patients. Of them 39 cases were of neuroblastomas, 1 pheochromocytoma; 6 operated medullary carcinomas; 5 paragangliomas; 2 Ewing's sarcoma and 1 Rhabdomyosarcoma; The sensitivity for the detection of primary tumours of neuroblastomas was 94% and for the detection of metastasis was 83%; while in the case of paragangliomas and medullary carcinoma, the sensitivity was 75% and 70% respectively. Our experience in the present study shows that 131I-MIBG scintigraphy is a sensitive and specific diagnostic tool to localise primary and metastatic disease of neural crest tumours.
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PMID:131I-MIBG scintigraphy in neural crest tumours. 792 41

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.
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PMID:Principles of staging of soft-tissue sarcomas. 847 13


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