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)

Approximately two thirds of children with rhabdomyosarcoma will be cured of their tumor and become long-term survivors. Multidisciplinary care has brought about improvements in survival. Yet our therapies of today are suboptimal in many situations and demand intense effort toward improving cure rates and quality of life. Rhabdomyosarcoma is a complex childhood malignancy with differing anatomic sites of presentation and varying histologic subtypes, each presenting with unique patterns of growth, making it inappropriate to consider the disease a single entity. Avenues of future research involve cytogenetic and molecular genetic investigations that will aid in diagnosis and screening and, it is hoped, guide clinicians to more specific therapy.
Arch Surg 1989 Sep
PMID:Rhabdomyosarcoma: contemporary status and future directions. The Lucy Wortham James Clinical Research Award. 277 1

Survivors of hereditary retinoblastoma are at increased risk for the development of second primary tumors, most commonly osteosarcoma. Recent molecular genetic data demonstrate that a pleiotrophic effect of the retinoblastoma gene may be responsible for the development of these sarcomas. This report describes the incidence of second nonocular malignancies among 53 infants seen at Stanford University Medical Center who have been followed a median of 11.7 years. Of these, 42 initially had bilateral disease and eleven had unilateral disease. Of 53 infants, 50 received irradiation either as part of the initial therapy or as treatment for recurrent disease. The actuarial survival for the entire group is 67% at 30 year follow-up with a median survival of 79% at 11.7 years. Eight patients developed eleven second primary tumors. All occurred in the group having hereditary retinoblastoma. Eight were within the previously irradiated field and three were at distant sites. The second tumors included seven osteosarcomas, one angiosarcoma, one rhabdomyosarcoma, one malignant fibrous histiocytoma, and one unclassifiable round blue cell tumor. The actuarial incidence of the development of a second primary malignancy was 6% at 10 years, 19% at 20 years, and 38% at 30 years. The latent period from treatment of retinoblastoma to the diagnosis of malignancy ranged from 5.2 years to 36.2 years (median 16 years). An aggressive approach with combined modality therapy including radical resection, re-irradiation and/or chemotherapy was used to treat these second primary tumors in five of eight patients. In four of the five, there was no evidence of disease at 22-72 months following treatment. In the three patients who did not receive aggressive combined treatment, there were no survivors. These data confirm the previously reported risk of developing a second primary tumor among survivors with hereditary retinoblastoma. Careful long-term follow-up for this genetically susceptible group is essential for early detection and implementation of curative therapy.
Int J Radiat Oncol Biol Phys 1989 Sep
PMID:Aggressive management of second primary tumors in survivors of hereditary retinoblastoma. 277 44

To investigate a recent report suggesting extensive bone erosion (EBE) as an important prognostic factor in head and neck rhabdomyosarcoma, a retrospective review was performed of 32 patients with gross residual and/or metastatic non-orbital head and neck rhabdomyosarcoma treated between the years 1971-1987. Treatment consisted of surgery, radiation therapy (median primary dose, 5000 cGy) and combination chemotherapy according to the Memorial Sloan-Kettering Cancer Center (MSKCC) T2 and T6 protocols. The MSKCC staging system was used: Stage II, 23 patients; Stage III, 6 patients; and Stage IV, 3 patients. With a median follow-up of 48 months (range 17 months to 13 years), the overall survival and local control rates were 72% (23/32) and 75% (24/32), respectively. Local control was achieved in 11/11 patients without extensive bone erosion (Stages II, 9/9; III, 1/1; IV, 1/1) as compared to 13/21 patients (Stages II, 10/14; III, 2/5; IV, 1/2) with extensive bone erosion (p = 0.02). Our data appear to support the recently reported finding that extensive bone erosion is an important predictor of local failure.
Int J Radiat Oncol Biol Phys 1989 Sep
PMID:The influence of extensive bone erosion on local control in non-orbital rhabdomyosarcoma of the head and neck. 277 53

Between 1972 and 1986, 37 patients with lower genital tract malignancies were treated with intracavitary or interstitial brachytherapy. Thirteen patients presented with clear cell adenocarcinoma, 14 patients with embryonal rhabdomyosarcoma, 6 patients with endodermal sinus tumor, 3 patients with sarcoma, and 1 patient with an undifferentiated tumor. FIGO classification was: Stage I, 16%; Stage II, 47%; and Stage III, 37%. Treatment policy included initial exploratory laparotomy with lymph node biopsy and ovarian transposition, chemotherapy (except in clear cell adenocarcinoma) and/or external radiotherapy prior to interstitial brachytherapy. Chemotherapy consisted of a combination of VAC-Ad (V = vincristine, A = D actinomycin, C = cyclophosphamide, Ad = adriamycin) in rhabdomyosarcoma and sarcomas, and MAC-Ad (M = methotrexate) in endodermal sinus tumor. External radiotherapy was used in seven patients: in one to reduce a bulky clear cell adenocarcinoma (20 Gy) and in six for pelvic nodal involvement (45 Gy). Brachytherapy techniques depended on tumor site and extent, and on the anatomy of the patients. Vulvar tumors were implanted with iridium-192 wires by an afterloading plastic tube technique. Cervical and vaginal tumors were treated with individually tailored moulded vaginal applicators loaded with either cesium-137 or iridium-192, with or without interstitial implants by plastic tube or guide gutter technique. Computerized dosimetry allowed calculation of treatment volumes and doses delivered on the tumor and adjacent critical organs. The prescribed dose (including external radiotherapy) was 60-75 Gy with 1-3 brachytherapy applications of a low dose rate (0.2 Gy/hr). Six patients are dead: one from chemotherapy complication, three of metastases (two sarcomas, one endodermal sinus tumor) and two of pelvic failures and metastases (two clear cell adenocarcinoma). The overall disease free 5-year survival is 72%. Actuarial 5-year local control is 84%, but including salvage is 94%: three (two rhabdomyosarcoma, one clear cell adenocarcinoma) of the five local failures were salvaged by surgery, chemotherapy and/or brachytherapy. Metastases occurred in six patients, one (sarcoma) salvaged by chemotherapy and external radiotherapy. Complications requiring surgery occurred in five patients: two hydronephroses, one urethral stricture, one ileo-cecal obstruction, and one vesicovaginal fistula. Twelve of the 17 patients (71%) over 12 years of age are normally menstruating. Two patients have produced three normal children. This multidisciplinary management of lower gynecological tract tumors including brachytherapy is both conservative and effective.
Int J Radiat Oncol Biol Phys 1989 Sep
PMID:Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experience. 277 54

The A673 human rhabdomyosarcoma cell line constitutively produces an acid-soluble, potent immunosuppressive factor (ISF), which inhibits T-cell proliferation. We have partially purified this factor from the culture supernatant of A673 cells by a sequence of acid extraction, gel filtration, cation exchange chromatography, and reverse-phase HPLC. Characterization studies indicate that ISF is similar or identical to transforming growth factor beta (TGF beta). ISF exhibits a molecular weight of 25 kDa in sodium dodecyl sulfate-polyacrylamide gels. ISF, like TGF beta, is a very basic protein (pI = 9.5) that is sensitive to reduction. Anti-TGF beta 1 antibodies completely block ISF activity in the thymocyte assay. Furthermore, ISF, like TGF beta, stimulated the anchorage-independent growth of normal rat kidney fibroblasts in soft agar.
Cell Immunol 1989 Sep
PMID:Partial purification of an immunosuppressive protein from a human tumor cell line and analysis of its relationship to transforming growth factor beta. 278 19

Twenty-nine children (median age: 41 months) with advanced solid tumors received, as consolidation therapy, two consecutive courses of high-dose chemotherapy (HDC) followed by mafosfamide-purged autologous marrow transplantation (ABMT) with a 3- to 4-month interval between each course. The malignancies were neuroblastoma (n = 22), Ewing's sarcoma (n = 5) and rhabdomyosarcoma (n = 2). Patients received a preparatory regimen consisting of combined high-dose melphalan before each ABMT, with the exception of five patients who received busulfan and cyclophosphamide before the second ABMT. Prior to HDC, bone marrow sufficient for two transplantations was harvested in remission, treated with mafosfamide (50 micrograms/ml) and cryopreserved. Following incubation with the drug, a consistent inhibition (greater than 99%) of granulocyte and macrophage colony-forming units was observed. Despite the elimination of measurable hematopoietic progenitors, all patients underwent engraftment within a similar period of time after the first and the second ABMT. However, peripheral leukocyte and granulocyte recovery was delayed (median 26 and 28 days, respectively, after the first graft; median 27 and 28 days after the second graft). No difference was observed in the bacterial infections following the first and second ABMT. One patient died after the second transplant with diffuse aspergillosis. Recovery to 50 x 10(9) platelets/l occurred after a median 42 days (range 19-71) after the first ABMT and 43 days (range 14-110) after the second. Two patients died of recurrent disease before attaining a normal platelet level. One patient remained thrombocytopenic and died from visceral failure at day 200. These results demonstrate the feasibility of repeated ABMT with mafosfamide-treated marrow.(ABSTRACT TRUNCATED AT 250 WORDS)
Bone Marrow Transplant 1989 Sep
PMID:Hemopoietic reconstitution after repeated autologous transplantation with mafosfamide-purged marrow. 279 Mar 32

This paper discusses the case of a pregnant woman with polychemotherapy during the second trimester of pregnancy for rhabdomyosarcoma of the face and cessation of fetal growth with disappearance of amniotic fluid after the first course of therapy.
Prenat Diagn 1989 Sep
PMID:Anhydramnios and cessation of fetal growth in a pregnant mother with polychemotherapy during the second trimester. 279 54

Nine Down's syndrome (DS) children, four with acute leukemia, one with acute leukemia as well as rhabdomyosarcoma, and four with other hematologic disorders, were analyzed for constitutional and acquired chromosomal aberrations. Acquired clonal chromosomal aberrations were identified only in the acute leukemia cases, and four of the five acute leukemia demonstrated numerical and/or structural aberrations involving chromosomes #8, #19, and #21. Of the 11 aneuploid stem cell lines identified in the five acute leukemia cases, trisomy 21, trisomy 8, trisomy 19, and tetrasomy or pentasomy 21 was found in 11, seven, four, and two lines, respectively. The frequent appearance of multiple stem cell lines with common and/or overlapping chromosomal aberrations in acute leukemia cases demonstrates the existence of genomic instability and heterogeneity of the neoplastic cell population, which results from clonal chromosomal evolution. Furthermore, trisomy 19 was identified only with the concurrent presence of trisomy 8, suggesting that the nondisjunction of chromosome #19 probably occurred after that of #8. Trisomy 21 was observed in every aneuploid stem cell line and, in one case, trisomy 21 was maintained in the bone marrow leukemic cells but not in the orbital rhabdomyosarcoma cells, indicating that this constitutional chromosomal aberration is probably crucial for and predisposed to the development of acute leukemia in DS patients. The association of acquired clonal chromosomal aberrations, especially those involving chromosomes #8, #19, and #21, with DS acute leukemia strongly suggests the clinical implication of cytogenetic analysis in the diagnosis of acute leukemia development in DS patients.
Cancer Genet Cytogenet 1987 Sep
PMID:Nonrandom chromosomal aberrations and clonal chromosomal evolution in acute leukemia associated with Down's syndrome. 295 85

A total of 22 adult nude mice (7 approximately 17 weeks old) of BALB/cA origin were inoculated subcutaneously (SC) with human tumor cell lines derived from rhabdomyosarcoma and lung carcinomas, including squamous cell carcinoma, bronchiolo-alveolar carcinoma, small cell carcinoma, giant cell carcinoma and 2 types of adenocarcinoma. 12 neoplasms (54.5%) was confirmed at the initial transplantation and 7 serially transplantable tumors were established in 201 nude mice. Metastatic behavior of 7 human tumor cell lines grown in nude mice was judged histologically after sacrifice and SC transplanted tumors could either be highly metastatic (giant cell carcinoma), moderately metastatic (adenocarcinoma PAb), poorly metastatic (squamous cell carcinoma and adenocarcinoma PAa) or non-metastatic (small cell carcinoma and bronchiolo-alveolar carcinoma). The tumor from SC inoculated giant cell carcinoma (PG) could produce regional and/or distant lymph node metastases (12/12) and lung metastases (9/12) in BALB/cA nude mice maintained under semi-barrier system conditions. Six NIH nude mice developed metastatic tumor nodules both in the lymph node and lung (6/6) under the same condition. The highly metastasizing property of PG transplanted tumors remained after passage for as many as 18 times. The limitations of nude mice as an in vivo model for the study of tumor biology are discussed, eg. malignant neoplasms rarely metastasize when transplanted into nude mice. The use of such model (PG) could lead to a better understanding of the biology of metastasis, thus contributing to the development of new approaches to the therapy of metastasis.
Zhonghua Zhong Liu Za Zhi 1985 Sep
PMID:[Establishment of highly metastatic human tumor cell line in nude mice]. 300 53

Malignant fibrous histiocytomas (MFH) belong to the most frequent soft tissue tumours in adults and have to be discriminated from other tumours with similar morphology. Various tumour markers aid the differential diagnosis. Twenty cases of MFH were studied immunohistochemically using antibodies to vimentin, TPA, desmin, lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin, S-100 protein, neurone-specific enolase (NSE), laminin, fibronectin and ferritin. Vimentin and lysozyme were found in the tumour cells of all, alpha 1-antitrypsin of 18, alpha 1-antichymotrypsin of 19, fibronectin of 16 and ferritin of 12 cases. Antibodies of TPA, desmin, S-100 protein, NSE and laminin did not reveal positive immunoreactivity. Exclusion of spindle-cell carcinoma can be made by positive vimentin and negative TPA reactivity, of melanoma by negative S-100 reactivity, and of leio- and rhabdomyosarcoma by lack of desmin immunoreactivity. Schwannomas contain S-100 protein, but lack lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and fibronectin. Pleomorphic liposarcomas cannot be distinguished from MFH on the basis of immunohistochemical staining. Vimentin, alpha 1-antitrypsin, alpha 1-antichymotrypsin and fibronectin can, therefore, be regarded as useful markers in the differential diagnosis of MFH.
Wien Klin Wochenschr 1986 Sep 26
PMID:[Immunohistochemical studies in the differential diagnosis of malignant fibrous histiocytoma]. 302 16


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