Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Computerized tomographic (CT) scans and radiographs of 19 children with nonorbital rhabdomyosarcoma of the head and neck region presenting between 1975 and 1987 were reviewed retrospectively. The scans were assessed for the extent of soft tissue disease and for adverse prognostic features such as the presence of intracranial and parameningeal involvement and for bony destruction. Where applicable, the CT scans were compared with plain radiographs. Follow-up scans after treatment were also reviewed and, where available, histology was correlated with any residual masses. CT was valuable in the initial assessment of the extent of the primary tumor and, therefore, in treatment planning. It was useful in identifying poor prognostic features such as bony destruction and intracranial extension but was not found to be helpful in elucidating the nature of residual or recurrent masses.
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PMID:The role of computerized tomographic scanning in the management of rhabdomyosarcoma in nonorbital head and neck sites. 220 56

We describe a 13-year-old girl with a primary nasopharyngeal rhabdomyosarcoma presenting with an isolated breast metastasis and no other evidence of distant metastatic spread. Only seven cases have been previously reported, and in all these cases the primary tumor was located either on an extremity or the buttock, and in six the disease was widely disseminated at diagnosis.
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PMID:Nasopharyngeal rhabdomyosarcoma presenting as a breast mass. 220 67

A series of 15 consecutive children with head and neck nonorbital rhabdomyosarcoma (RMSA) with meningeal extension were prospectively treated with chemotherapy consisting of Adriamycin (doxorubicin; Adria Laboratory, Columbus, OH) (ADM), vincristine (VCR), cyclophosphamide (CPM), and dactinomycin (DACT) followed by radiotherapy (60 Gy) to the primary tumor volume, along with intrathecal methotrexate (IT MTX). Thirteen of 15 responded to preradiation chemotherapy. Four of 13 relapsed. Relapse occurred at the level of the primary tumor in three of four. The 3-year progression-free survival (PFS) was 59%, similar to that achieved in a previous series treated with a comparable therapeutic approach that also included whole-brain radiotherapy as a prophylaxis of possible occult meningeal seeding. It is concluded that CNS prophylaxis with radiotherapy is questionable in the management of childhood RMSA with meningeal extension.
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PMID:Questionable role of CNS radioprophylaxis in the therapeutic management of childhood rhabdomyosarcoma with meningeal extension. 223 Aug 72

Thirty-four patients with extremity rhabdomyosarcoma were treated at Memorial Sloan-Kettering Cancer Center during the years 1970 to 1987. The regional lymph node status at diagnosis was histologically confirmed in 27 patients. Of these 27 patients, there were 20 males and 7 females, ranging in age from 6 months to 22 years (median 10 years). The primary tumor was located in the upper and lower extremity in 11 and 16 patients, respectively. The histologic subtypes included embryonal in 13 patients and alveolar in 14 patients. Fourteen patients had no evidence of regional lymph node involvement (N0) and 13 patients had lymphatic metastases (N1). All patients were further staged according to the extent of surgical resection, the local invasiveness and size of the primary tumor, and the presence (M1) or absence (M0) of distant metastases. With a median follow-up time from diagnosis of 9.2 years (minimum 5.3 years), the overall survival was 48% (13/27): N0, 12/14 and N1, 1/13 (P = .0002). Excluding those patients with M1 disease, the survival rate was: N0, 11/12; N1, 1/10 (P = .0001). The first site of relapse in the M0 patients was distant metastases in 8/10 cases. Our results suggest that regional lymph node involvement at diagnosis is an important prognostic factor in extremity RMS. Its presence appears to be associated with a high incidence of relapse in metastatic sites and poor survival outcome.
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PMID:Prognostic significance of regional lymph node involvement in childhood extremity rhabdomyosarcoma. 223 17

We found that rhabdomyosarcoma (RMS) subcellular membranes contain sialyltransferase activities for LcOse4Cer and GgOse4Cer acceptors. Chromatographic analyses and neuraminidase lability of the sialyltransferase products indicated that the principal site of sialylation was the non-reducing terminal galactosyl moiety. In order to control for the effects of cell density in culture, metastatic S4T18 RMS cells and nonmetastatic F9-4/21 RMS cells were harvested at 2 X 10(4) to 6 X 10(4) per cm2 prior to analyses. Irrespective of metastatic potential, we found that sialyltransferase-specific activities were influenced by cell densities. F9-4/21 cells, for example, at a density of 6 X 10(4), produced membranes with sialyltransferase-specific activities to LcOse4Cer 1.9-fold higher than cells at 2.1 X 10(4)/cm2. Metastatic potential (predetermined in vivo) appeared to be correlated with an accelerated effect of cell density on the sialyltransferase activity to LcOse4Cer. Metastatic S4T18 cells at 6.3 X 10(4)/cm2 yielded membranes with sialyltransferase-specific activities 5.4-fold higher than membranes from cells at 1.9 X 10(4)/cm2. Conversely, fucosyltransferase activities in the presence of LcOse4Cer were highest in non-metastatic F9-4/21 cells at low cell densities. Quantitative analyses of monosialoganglioside fractions of RMS cells were in agreement with the sialyl-transferase studies. HPLC and HPTLC analyses demonstrated the presence of glucosamine-containing monosialoganglioside with Rf identical with the radioactive products of LcOse4Cer sialylation, which increased 4.5-fold on a per mg protein basis as cell densities increased in S4T18 cells in culture from 1.9 X 10(4)/cm2 to 6.3 X 10(4)/cm2. Plasma membrane marker Na+, K+, ATPase-specific activity also increased in RMS metastatic cells in a manner comparable to that described for the sialyl-transferase activity to LcOse4Cer. Our results suggest that metastatic potential is expressed in the rate of sialylation at specific membrane sites of RMS intercellular contact. We propose a process of selection for metastasis whereby specific cell surface non-reducing galactosyl termini are recognized by intercellular transferases and lectins in the primary tumor, and the corresponding labile sialylated sites (on disseminated cells) are recognized by host neuraminidases.
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PMID:Monosialoganglioside biosynthesis by subcellular membranes of rhabdomyosarcoma cell lines differing in metastatic potential. 233

In an attempt to evaluate the radiocurability of microscopic disease in childhood rhabdomyosarcoma (RMS) with total tumor doses of less than 4,000 cGy, we performed a retrospective analysis of all patients with microscopic residual RMS who were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC) during the years 1970 to 1987. There were 32 patients ranging in age from 3 months to 22 years (median, 6 years) with microscopic residual of either (1) a localized primary tumor (MSKCC, stage IB; Intergroup Rhabdomyosarcoma Study [IRS] group IIA), 19 patients; or (2) an involved lymph node region with the primary tumor completely resected (MSKCC stage III; IRS group IIC), 13 patients. Twenty-nine of the 32 patients presented with embryonal histology. All patients were treated with combination chemotherapy (CT) and megavoltage external beam radiotherapy (RT). The RT was delivered in either conventional fractionation of 180 to 200 cGy daily (30 patients) or hyperfractionation of 150 cGy twice daily (two patients). Fifteen patients received RT doses of less than 4,000 cGy with a range of 3,000 to 3,600 cGy and a median value of 3,100 cGy; 17 patients received 4,000 cGy or more with a range of 4,000 to 6,000 cGy and a median value of 4,600 cGy. With a median follow-up of 11 years, the relapse-free survival was 25 of 32 patients (less than 4,000 cGy, 12 of 15; greater than or equal to 4,000 cGy, 13 of 17). The RT local control rate was 30 of 32 (less than 4,000 cGy, 14 of 15; greater than or equal to 4,000 cGy, 16 of 17 [P = .94]). Our results suggest that radiation doses of below 4,000 cGy, when combined with effective multiagent CT, may be sufficient for local control of microscopic disease in childhood embryonal RMS.
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PMID:Radiocurability of microscopic disease in childhood rhabdomyosarcoma with radiation doses less than 4,000 cGy. 239 58

The effects of carcinogenic nickel [(Ni) CAS: 7440-02-0] and Ni compounds on the natural killer (NK) cell activity of rat peripheral blood mononuclear cells (PBMCs) were studied. Rhabdomyosarcomas were locally induced by one im injection of Ni or Ni subsulfide [(Ni3S2) CAS: 12035-72-2] dust in the hind leg of WAG rats. A weakly tumorigenic dose of 5 mg Ni3S2 (tumor incidence, 2%) induced a transient decrease of PBMC NK activity against YAC-1 cells in vitro (from the 17th to the 23d wk after Ni3S2 inoculation), which could be restored by in vivo injections of partially purified rat fibroblastic interferon (IFN). Injection of 20 mg Ni (tumor incidence, 47.5%) produced a long-lasting depression of NK cell activity (from the 8th to the 23d wk). In vivo chronic IFN treatment of the Ni-injected rats neither restored NK cell activity nor affected the tumor incidence. However, NK cells of Ni-treated animals responded normally to IFN in vitro. Prospective analysis of individual NK cell responses showed that a persistent depression of basal NK cell activity was restricted to rats that subsequently developed a tumor. In these animals the time between carcinogen treatment and clinical detection of the primary tumor was positively correlated with the mean level of NK cell activity (3-4 determinations/rat). Admixture of manganese to Ni inhibited the development of tumors and also prevented the depression of NK cell activity produced by Ni alone. Noncarcinogenic Ni oxide stimulated NK cell activity. These results point out the possible involvement of NK cells in resistance to Ni-induced carcinogenesis.
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PMID:Inhibition of rat natural killer cell function by carcinogenic nickel compounds: preventive action of manganese. 243 44

Several human rhabdomyosarcoma cell lines, cultured primary tumor explants, and biopsies of tumor and normal skeletal muscle tissue expressed a 2.0-kilobase transcript that hybridized to the mouse muscle determination gene MyoD1. This transcript was found in tumor cell lines and primary explants that developed multinucleated myotubes but was absent in Wilms' tumors or cell lines and primary explants that developed multinucleated myotubes but was absent in Wilms' tumors or cell lines derived from other mesenchymal tumor cell types. Expression of the human homolog of MyoD1 therefore can define a tumor as a rhabdomyosarcoma. Transfection of the mouse MyoD1 gene into the human rhabdomyosarcoma cell line RD increased the ability of the tumor cells to differentiate into multinucleated myotubes and enhanced myosin heavy-chain gene expression but did not decrease tumorigenicity in nude mice.
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PMID:Expression of the MyoD1 muscle determination gene defines differentiation capability but not tumorigenicity of human rhabdomyosarcomas. 260 95

We have retrospectively reviewed the radiographic and clinical features of 56 cases of pelvic rhabdomyosarcoma seen in three Pediatric Hospitals between 1960 and 1986. There were 35 boys and 21 girls. The study aimed at better defining the role played by the various imaging techniques in the investigation of these tumors. The role of diagnostic radiology is the detection and delineation of the primary tumor, its local spread and distant metastases at the time of diagnosis and on follow-ups. Intravenous urography (IVU) and to a lesser extent barium enema (BE) and cystography (VCUG) were used even after the availability of ultrasonography (US) and computed tomography (CT). These two modalities have only partly replaced the traditional radiographic techniques. All patients seen or followed after 1977 (28 patients) had both US and CT examinations. We have focused our discussion on these relatively new imaging modalities namely US and CT. Magnetic resonance was not utilized in any patient in this series. US was by far the best imaging technique available for lesions of the urinary bladder and those invading the bladder wall in children as it was possible to visualize, measure and follow these tumors. Scrotal US was used to confirm the clinical diagnosis of a paratesticular mass. CT showed to best advantage the tumour and its relationship to pelvic organs, musculature and bones.
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PMID:CT and ultrasound imaging of pelvic rhabdomyosarcoma in children. A review of 56 patients. 267 47

Among 404 patients with primary tumors of extremity-trunk sites entered in the Intergroup Rhabdomyosarcoma Study (IRS) (1972 to 1984), 154 were placed in clinical group IIa, ie, with negative nodes but with "microscopic residual" (MR) disease, following the initial excisional (not biopsy) procedure. An elective reexcision of the area of the primary tumor (PRE) was performed in 41 of these patients within 35 days (mean interval, 14 days; SE, 0.9) with no intervening therapy. These procedures consisted of wider excision of the tumor "bed," resulting in a technical transfer of these patients from group IIa to group I, ie, complete excision. This reduced intensity of nonsurgical therapy (irradiation and chemotherapy). Among the 41 patients who underwent PRE, the 3-year survival estimate (Kaplan-Meier) was 91% (SE, 4%). This may be compared with the results in 113 patients who remained in group IIa, in which the 3-year survival estimate was 74% (SE, 4%). A second group for comparison consisted of the 73 patients with trunk/extremity tumors who were placed in group I after a single excisional procedure, ie, no PRE, in whom the 3-year survival estimate was 74% (SE, 5%). Recognized prognostic factors influencing survival in these groups were comparable, with the exception of tumor size, ie, the largest tumors (greater than or equal to 10 cm in diameter) were concentrated in groups I and IIa. When patients with tumors greater than or equal to 10 cm in diameter (9.7% of the total) were removed from all three study groups, patients undergoing PRE had longer survival duration estimates than patients in the control groups.
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PMID:Primary reexcision for patients with 'microscopic residual' tumor following initial excision of sarcomas of trunk and extremity sites. 272 95


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