Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Records of 374 patients referred to the National Cancer Institute with a diagnosis of sarcoma over a 24-year period were reviewed to study the incidence of lymph node involvement in this disease. One hundred and thirteen patients had operations involving the draining nodal area and evaluation of these nodes for tumor. Only three patients (2.6%) had evidence of sarcoma metastatic to draining lymph nodes. Prophylactic removal or radiotherapy of draining lymph node areas in most adults with sarcomas does not appear to be worthwhile. A comprehensive analysis of the literature is presented.
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PMID:Early lymphatic spread of osteogenic and soft-tissue sarcomas. 27 29

Of 47 children with an initial diagnosis of lymphosarcoma, reticulosarcoma or Non-Hodgkin's lymphoma (NHL), 13 had to be excluded at the histologic reevaluation: in 10 an undifferentiated sarcoma, in 2 Hodgkin lymphoma was found; in one patient no definite classification of the tumor was possible. Of the remaining 34 patients there were 26 boys and 8 girls. One patient had a nodular, 33 a diffuse NHL. Of the latter 16 had a Burkitt-type (LB-), 3 a lymphoblastic, convoluted (LC-), 8 a lymphoblastic, "other" (LO-) and 6 a histiocytoid (H-) NHL. Primary localization: abdomen: 13/34; "peripheral" lymph nodes: 9/34; mediastinum: 5/34; nasopharynx: 4/34; subcutis: 2/34; skeleton: 1/34. Twelve of 17 NHL with primary localization in the abdomen or nasopharynx were LB-NHL, 8/14 NHL with primary localization in "peripheral" nodes or mediastinum were LC- or LO-NHL. Only 2/17 NHL with abdominal or nasopharyngeal primary, but 9/14 NHL with "peripheral" nodal or mediastinal primary developed leukemic extension and/or CNS involvement. 6 of 34 patients are living without evidence of disease for 1 1/2+ to 13+ years; 5/34 died but lived for 85, 57, 37, 22 and 22 months; 9/34 lived 6--12 months; 14/34 died within less than 6 months. Patients with abdominal primary either died within 5 months or survived (for 165+, 63+ and 25+ months). Aggressive local therapy (surgery and radiotherapy with approximately 4000 R) may be adequate for strictly localized (stage I) disease, particularly if the primary localization is abdominal. In all other diffuse NHL of childhood an early, aggressive chemotherapy, later combined with radiotherapy to bulk disease and prophylactic CNS-treatment is essential for inducing long-term remissions and, possibly, cures. For prognosis the primary localization appeared to be more important than histology and stage. The most decisive factor, however, is therapy.
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PMID:Malignant non-Hodgkin's lymphoma (NHL) in childhood. Retrospective analysis of 34 cases. 61 79

A total of 299 patients with Stage IA-Stage IIB nodal lymphoma treated by irradiation were evaluated for initial reactivation. The abdomen was the major site of reactivation for patients with supradiaphragmatic Hodgkin's disease. The non-Hodgkin's lymphomas reactivated in a variable fashion. Reactivation was prompt for patients with reticulum-cell sarcoma and longer for patients with other lymphomas. Initial extranodal manifestations were present in 24%, 51%, and 63% of patients with Hodgkin's disease, lymphosarcoma, and reticulum-cell sarcoma, respectively.
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PMID:Lymphomas: initial reactivation. 110 30

Nonrhabdomyosarcoma soft tissue sarcomas in infants and children are rare malignancies with most of the clinical data gained by retrospective analysis. In 1986, a prospective multicentered study was initiated by the Pediatric Oncology Group (POG) with a total of 75 cases now entered for epidemiologic examination. Median age of presentation was 12 years (range, newborn to 20 years). The male to female ratio was 2.3 to 1. The most common soft tissue tumor was synovial cell sarcoma (32/75, 42%), followed by fibrosarcoma (10/75, 13%), malignant fibrous histiocytoma (9/75, 12%), and malignant neurogenic tumors (8/75, 10%). Sixty-five percent of all tumors presented on the extremities (44% lower extremity, 21% upper extremity). Tumors of the trunk accounted for 28% (abdomen 15%, thorax 13%), whereas head and neck tumors were 7%. By TNMG classification, 16% presented as stage I, 21% as stage II, 33% as stage III, and 30% as stage IV. Age at presentation did not affect clinical site or stage. All upper extremity tumors presented with localized disease, whereas lower extremity tumors presented with regional nodal disease in 7% and metastatic disease (pulmonary) in 23% of the cases. Seventy-eight percent of the abdominal tumors were metastatic at diagnosis; the other 22% had extensive regional disease. In the thorax 78% had localized lesions with 22% having extensive regional disease. Ninety percent of synovial cell sarcomas were on the extremities: 84% localized disease and 12% metastatic spread at presentation. Sixty percent of the fibrosarcomas presented on the extremities with 80% having localized disease and 20% metastatic spread. Only 25% of the neurogenic tumors presented with localized disease, whereas 50% had metastases at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:A prospective study of nonrhabdomyosarcoma soft tissue sarcomas in the pediatric age group. 131 9

Amputation has traditionally been advised for extremity epithelioid sarcoma because of its pattern of innocuous presentation and relentless soft-tissue and nodal metastasis. To assess the role of amputation in extremity epithelioid sarcoma, we reviewed our experience with 42 patients treated between 1961 and 1986. On presentation with localized primary tumor (n = 18), nine of 11 patients who underwent wide local excision and four of six patients who underwent excisional biopsy were free of disease, and one patient who underwent amputation died. After presentation with localized recurrence (n = 12), four of six patients who underwent wide local excision and two patients who underwent excisional biopsy were free of disease; three other patients who underwent wide local excision had margins that tested positive on pathologic examination, of whom one was free of disease; one patient who underwent amputation died of disease. On presentation with regional metastasis (n = 12), only one of five patients who underwent wide local excision and one of seven patients who underwent amputation were free of disease. Primary amputation offered no apparent overall survival benefit to patients presenting with regional metastasis. The favorable outcome after local resections for localized disease indicates that wide local excision with margins that test negative on pathologic examination is preferable to radical amputation in these patients.
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PMID:Extremity epithelioid sarcoma. Amputation vs local resection. 184 77

The clinicopathologic, immunohistochemical, and flow cytometric characteristics of 34 cases of mammary carcinoma with metaplasia were compared with those of 20 cases of pure sarcoma of the breast. All 20 of the latter tumors showed the pattern of malignant fibrous histiocytoma. There were 20 cases of carcinoma with mesenchymal metaplasia, 7 cases of carcinoma with mixed epithelial (squamous) and mesenchymal metaplasia, and 7 cases of carcinoma with epithelial metaplasia (four mixed ductal/squamous and three pure squamous cell carcinomas). No patient with pure sarcoma had lymph node metastases develop; all nodal metastases were found in patients who had carcinoma with metaplasia, although in one case the carcinomatous component was seen only within a lymph node metastasis. Epithelial antigens were found not only within the epithelial elements of all cases of carcinoma, but also within the apparent mesenchymal elements of 44% of the carcinomas showing divergent differentiation. Flow cytometric analysis of eight cases of carcinoma with mesenchymal metaplasia showed aneuploidy/tetraploidy in six neoplasms. For patient management purposes, the distinction of pure sarcoma from carcinoma with metaplasia is important, but additional subclassification of carcinoma with metaplasia is of greater biologic than clinical interest.
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PMID:Carcinomas with metaplasia and sarcomas of the breast. 174 99

A retrospective morphologic survey (1973-1983) of 146 cases of malignant lymphoma among the Hawaii-Japanese (migrant Japanese and their offspring) was conducted to determine whether differences in the incidence and cytologic types of malignant lymphoma exist when compared to those of native Japanese (lifetime residents of Japan). The age-adjusted incidence rates for malignant lymphoma among the Hawaii-Japanese were similar to rates for U.S. whites. However, higher rates for follicular centre cell (FCC) lymphoma with a follicular pattern were observed in the Hawaii-Japanese population when compared with rates for native Japanese. On the basis of the cytologic types of the Lukes-Collins classification, non-Hodgkin's lymphomas among the Hawaii-Japanese resembled those of Western countries, rather than those of Japan. B-cell lymphomas predominated (72 per cent), while T-cell types comprised 23 per cent of cases. Follicular centre cell types were encountered most often (59 per cent), and the small cleaved FCC subtype was the most common (30 per cent). The high degree of follicularity (29 per cent) was at variance with the consistently low rates reported in Japan. This may be explained, in part, by higher rates of nodal lymphomas among the Hawaii-Japanese. Of the T-cell lymphomas, diffuse large cell types (T-cell immunoblastic sarcoma, T-IBS), often with cytologic pleomorphism, were relatively frequently encountered (16 per cent), and comprised 15 per cent of non-Hodgkin's lymphomas; this observation necessitates special clinical and epidemiologic consideration in view of the large Japanese migration to Hawaii from HTLV-I endemic regions of southern Japan. No registered cases of non-Hodgkin's lymphoma or of Hodgkin's disease were documented in Hawaii-Japanese subjects under the age of 15 years. The age-adjusted incidence rates for Hodgkin's disease among the Hawaii-Japanese were similar with those of native Japanese. Nodular sclerosis was the most frequent histologic subtype. The difficulty in distinguishing between Hodgkin's disease and non-Hodgkin's lymphoma, particularly when immunologic cell surface markers are not available, is addressed. Low rates for chronic lymphocytic leukemia among the Hawaii-Japanese were confirmed. Not one well-documented case was identified in the 11-year period surveyed.
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PMID:Malignant lymphoma in Hawaii-Japanese: a retrospective morphologic survey. 270 45

Twenty-eight patients with a uterine stromal sarcoma or mixed mesodermal tumor were treated with cisplatin 100 mg/m2 and Adriamycin 45-60 mg/m2, given with intravenous hydration every 3 to 4 weeks. Group I consists of 11 patients with measurable disease following initial surgery or with a recurrence. Eight of the eleven evaluable patients with measurable disease had a response (73%), and three of these patients have had a negative second-look procedure, and two are alive and disease free more than 24 months after initiation of treatment. Group II consists of 17 patients treated with adjuvant chemotherapy after primary surgery. The patients were selected for adjuvant therapy based on previous established poor prognostic features. Of the 17 patients in group II, 14 had invasion of the outer one-third of the myometrium and the other three had invasion to the middle one-third. Seven had documented positive pelvic and/or periaortic lymph nodes and five had positive peritoneal washings. With a median follow-up of 34 months, there have been only four recurrences in group II. Two of the recurrences occurred in patients who discontinued therapy after only two cycles of chemotherapy. There is a projected 5-year survival of 75% in these high-risk patients. Of the seven patients with documented nodal involvement, one patient died with a recurrence at 23 months, one patient died from a perforated diverticulum, and the other five are alive and disease free with a median follow-up of 36 months (34-90 months). Two patients with multiple positive nodes are disease free at more than 5 years. Combination chemotherapy with cisplatin and Adriamycin has a high response rate with advanced measurable disease and improves survival in high-risk patients who receive it as adjuvant therapy.
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PMID:Cisplatin and adriamycin combination chemotherapy for uterine stromal sarcomas and mixed mesodermal tumors. 276 23

The autopsy findings of 73 patients with uterine sarcoma were studied to determine the sites and possible modes of metastasis. Homologous mixed mesodermal tumors were the most frequent (41%) followed by leiomyosarcoma (26%), heterologous mixed mesodermal tumor (18.3%), stromal sarcoma (12%), and endolymphatic stromal myosis (3%). The peritoneal cavity and omentum were the most frequently involved sites (59%), followed by the lung (52%), pelvic lymph nodes (41%), paraaortic lymph nodes (38%), and liver parenchyma (34%). The presence of lung metastasis was not associated with pelvic or paraaortic node metastasis or intraperitoneal disease. Metastasis to other distant sites including the brain, heart, kidney, and bone were independent of pelvic and paraaortic nodal metastasis or intraperitoneal disease. Metastatic sites were not different among various histologic types. Distant metastatic sites were statistically associated with lung metastasis. Hematogenous metastasis best explains this metastatic pattern and adjuvant systemic therapy seems indicated.
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PMID:Patterns of metastasis in uterine sarcoma. An autopsy study. 291 99

A retrospective cooperative study was undertaken to analyze the fate of 300 clinical Stage I and II breast cancer patients who were alive and apparently cured with both breasts preserved, 10 years following primary limited surgery with irradiation. All patients had been treated by tumor excision, with or without axillary dissection, followed by megavoltage radiation therapy. Follow-up ranged from 10.5 to 26 years, median 14.5 years. The overall actuarial survival (Kaplan-Meier) of the 300 "cured" patients was 86% at 15 years and 78% at 20 years, with 38.5% of deaths attributable to breast cancer. The actuarial probability of remaining free of metastatic disease was 91% at both 15 and 20 years, independent of age or clinical stage. Sixteen patients (5.3%) developed recurrent cancer in the treated breast beyond the tenth year, the actuarial probability of remaining free of breast recurrence being 94% and 90% at 15 and 20 years, respectively. Contralateral breast cancers developed during the second decade in 5 patients, with a cumulative risk of 6.5% at 20 years. Significant treatment-related problems appeared during the second decade in 5 patients, including one chest wall sarcoma; all of these patients had received at least 60 Gy to breast and regional nodal areas. A comparison of these results with those in the literature allowed the following conclusions to be drawn: (a) the risk of death, as well as breast cancer mortality during the second decade, are similar for both conservatively and radically treated patients with Stage I and II breast cancer; (b) the risk of contralateral breast cancer is not greater than that observed following primary radical surgery without radiation therapy; (c) ipsilateral breast "recurrences" continue to occur at about 1% per year during the second decade. Such late recurrences are highly operable and have a favorable prognosis; (d) late progression of treatment-related sequelae is uncommon. This analysis supports the continued use of breast-conserving surgery with radiation therapy in the treatment of Stage I and II breast cancer.
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PMID:The second ten years: long-term risks of breast conservation in early breast cancer. 311 88


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