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)

Primary intracranial rhabdomyosarcoma is extremely rare, only nine cases have been reported in the world literature. To differentiate it from medullomyoblastoma, rhabdomyosarcoma should be devoid of neuroblastic elements. A case of rhabdomyosarcoma of the tentorial region in a 17-year-old boy is reported. Carotid angiograms demonstrated a peculiar neovascularity. Multiple cytologic preparations of CSF repeatedly contained rhabdomyosarcoma cells and CSF dissemination in the spinal subarachnoid space was documented at autopsy. The literature is reviewed.
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PMID:Primary rhabdomyosarcoma of the tentorium with peculiar angiographic findings. 45 44

Echovirus 30 was the cause of abacterial meningitis and pharyngitis in 22 cases in the Ludwigshafen on Rhine area in summer 1976 and in a further 26 cases in various regions of the German Federal Republic. In 11 children echotype-30-virus could be isolated from the CSF. In 47 children the diagnosis was established by a rise of neutralising antibodies. Positive virus isolation from the CSF was only possible during the first two days of the illness using rhabdomyosarcoma cell tissue cultures. These were particularly sensitive to the echovirus 30 subtype found.
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PMID:[Echovirus 30 epidemic (author's transl)]. 66 44

The consultants agreed that the differential diagnosis should focus on congenital masses, including an encephalocele, glioma, dermoid, hamartoma, hemangioma, rhabdomyosarcoma, neurofibroma, and nasolacrimal duct cyst. There was some disagreement as to which is the best way to evaluate the mass, ranging from an MRI (Dr. Reilly), to CT scan (Dr. Cotton), to both MRI and CT (Dr. Koopman). Blood tests to evaluate pituitary function could be indicated if there was a sphenoid defect (Dr. Reilly). None of the experts would biopsy this lesion. All would proceed with a definitive resection. One surgeon would defer surgery for several months and then perform the resection via a biocoronal craniotomy (Dr. Reilly). A combined anterior craniotomy and external ethmoidectomy would be planned by another (Dr. Koopman). The third consultant would combine an anterior craniotomy with a mid-face degloving, external rhinoplasty, or lateral rhinotomy approach (Dr. Cotton). Routine perioperative antibiotics would only be used by two of the surgeons (Drs. Reilly and Koopman). If a CSF leak were encountered there are several options. A small lesion could be allowed to close on its own (Dr. Reilly). If the leak occurred while the bicoronal incision was still open or if the leak were large, it could be repaired from above (Drs. Reilly and Koopman). One surgeon would proceed with a repair from above even if the leak were encountered during the intranasal approach (Dr. Cotton). Only one surgeon would restrict postoperative activity with intubation and sedation or paralysis (Dr. Koopman). Regarding follow-up, no one was concerned about the final pathology report.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nasal mass in a pediatric patient. 139 78

The levels of peripheral progenitor cells was measured serially after cancer chemotherapy in 4 patients with non-Hodgkin's lymphoma and one patient with rhabdomyosarcoma who received recombinant human granulocyte colony-stimulating factor (rG-CSF). This study was composed of two independent phases: in the first phase, patients received a course of cytotoxic chemotherapy only, and in the second phase, they received the same chemotherapy followed by subcutaneous injection of rG-CSF (2 micrograms/kg/day) for 10-14 days. In the control phase, the levels of granulocyte-macrophage colony-forming units (CFU-GM) and erythroid burst-forming units (BFU-E) per milliliter increased during the early recovery phase, but rG-CSF treatment increased the number of CFU-GM 3 to 18-folds, and the number of BFU-E increased 1.3 to 4.6-folds. An overshoot in the blood progenitor levels occurred at the day 8-10 of rG-CSF administration. And then, the peak of neutrophil count followed 3-5 days later. After the discontinuation of rG-CSF, the number of blood CFU-GM and BFU-E fell rapidly. This results suggest that in vivo expansion of circulating hemopoietic progenitors can be achieved by the administration of rG-CSF, and this approach might be clinically applicable to cancer patients who are a candidate of peripheral blood stem cell autotransplantation.
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PMID:[Expansion of peripheral blood progenitors by recombinant human granulocyte colony-stimulating factor]. 170

The pharmacokinetics of alkylating activity were studied in 17 children treated i.v. with ifosfamide (IF) at 3 g/m2 as a 1-h infusion for 2 consecutive days every 3 weeks, with mesna as a uroprotector. Two patients were treated for a newly diagnosed rhabdomyosarcoma according to the current SIOP (International Society of Pediatric Oncology) protocol. The other 15 patients were treated in a phase II study and presented with one of the following malignancies in relapse: neuroblastoma (7), osteosarcoma (3), soft tissue sarcoma (2), Wilms' tumor (1), non-Hodgkin's lymphoma (1), and acute lymphoblastic leukemia (1). Plasma alkylating activity levels determined by using 4(4'-nitro-benzyl)-pyridine showed considerable inter-individual and intercyclic variations and decreased biphasically, with mean alpha and beta half-lives of 60 min and 6-7 h, respectively. Probably as a result of liver mixed-function oxidase induction, on the 2nd day of treatment the terminal half-lives were shorter, the plasma exposures were lower, and the mean plasma clearances were higher. Renal excretion was almost complete after 24 h, accounting for a mean of 19% of the injected dose. The CSF alkylating activity levels, obtained in four children, were always lower than the plasma levels and ranged from 8 to 51 micrograms/ml, with a mean CSF/plasma ratio of 0.53 +/- 0.23 during the first 12 h. We conclude that IF alkylating activity was biphasically cleared from the plasma, with significant interindividual and intercyclic variability, that the renal contribution to the clearance was low, and that high levels of CSF alkylating activity could possibly contribute to the CNS toxic side effects observed in pediatric patients treated with high-dose IF/mesna.
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PMID:Alkylating activity in serum, urine, and CSF following high-dose ifosfamide in children. 250 56

Primary intracranial rhabdomyosarcoma (RMS) is a rare tumor in infancy and childhood that is found in various locations in the central nervous system. The clinical course worsens rapidly, and the final outcome is poor, with a median survival time of 8-10 months. Invasion of the meninges, spontaneous intratumoral bleeding, spinal leptomeningeal CSF spreading of tumor cells, and early recurrence of the mass are the distinctive features of RMS. Diagnosis of RMS may be missed: immunohistochemical staining using specific markers (myoglobin, myosin, desmin, vimentin, enolase), along with ultrastructural studies, provide the basis for making the final diagnosis. Treatment of RMS includes surgical excision, craniospinal radiation therapy, and chemotherapy. We report two cases of primary RMS in the CNS located in the posterior fossa and frontotemporal area. Both children underwent total surgical removal of the mass. Early recurrence of the tumor mass was noticed in both patients 2 months after surgery. Both children died shortly thereafter.
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PMID:Primary intracranial rhabdomyosarcoma: report of two cases. 279 Aug 36

Isolation in cell culture is currently the most sensitive and reliable way to demonstrate enterovirus (EV) in clinical specimens. During July-October 1982 and 1983, we studied the impact of adding two new cell lines, Buffalo green monkey kidney (BGM) and human rhabdomyosarcoma (RD), to the more traditional cell combination used for EV isolation, human embryonic lung (HEL) and primary cynomolgus monkey kidney (CMK) cells; 2,558 specimens were studied: 632 fecal, 677 respiratory, 537 CSF, and 712 blood. An EV was isolated from 417 (16%); of these, 77 (18%) were positive only in BGM or RD; 35% (146/417) of the specimens were positive in BGM, RD, or both, at least one day earlier than in the traditional cells. BGM cells were helpful in isolation of group B coxsackieviruses (CB): 99% of 121 positive specimens were detected in BGM vs 73% in CMK and 23% in HEL; 72% of the CB isolates were detected by day 2 in BGM vs 48% in CMK and 0% in HEL. RD cells were helpful in the isolation of echoviruses: 59% of the 189 positive specimens were detected in RD vs 67% in HEL and 58% in CMK. RD was the only positive cell type in 28/189 (15%) positive specimens; 31% of the echovirus isolates were detected by day 2 in RD, vs 20% in HEL and 19% in CMK. Using the cell types described, we provided the clinician with results in 42% of the EV-positive specimens by day 2 after inoculation and in 61% by day 4.
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PMID:A combination of four cell types for rapid detection of enteroviruses in clinical specimens. 301 65

Reducing agents such as glutathione (GSH), glutathione ester (GSE), and N-acetylcysteine (NAC) have been shown to suppress the induction of HIV expression in chronically infected cells stimulated by cytokines. We present data which show the effects of the organic thiophosphate WR-151327 on the expression of latent HIV in U1 cells. The chronically infected promonocytic cell line U1 constitutively expresses low levels of HIV that can be increased by 13-phorbol 12-myristate acetate (PMA), tumor necrosis factor alpha (TNF-alpha), and granulocyte/monocyte colony-stimulating factor (GM-CSF). WR-151327 suppressed, in dose-dependent fashion, the reverse transcriptase (RT) activity induced by TNF-alpha, GM-CSF, and PMA. The maximal decrease in RT activity was 70, 80, and 50%, respectively. Pretreatment with WR-151327 also suppressed the induction of total HIV protein synthesis, as shown by Western blot analysis. In addition, WR-151327 suppressed HIV-LTR-CAT activity in transfected human rhabdomyosarcoma cells (RD). Suppression of HIV expression by WR-151327 was observed in the absence of a cytotoxic or cytostatic effect. Incubation of WR-151327 with human recombinant TNF-alpha for 6 hr at 37 degrees C did not alter the capacity of TNF-alpha to induce the expression of HIV. Our observations further support the hypothesis that reducing agents are important in the control of HIV replication and that the clinical evaluation of WR-151327 may be indicated.
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PMID:Organic thiophosphate WR-151327 suppresses expression of HIV in chronically infected cells. 752 Nov 93

Neutropenic pediatric patients with solid tumors and malignant lymphomas were treated with recombinant granulocyte-macrophage colony stimulating factor (rh-GM-CSF). Eleven patients, including seven lympho-reticular malignancies, two Ewing's sarcoma and one patient in each group with the diagnosis of nasopharyngeal rhabdomyosarcoma, malignant mesenchymal tumor, entered the study. Six were females and five were males, the mean age was 10.4 yr, the range was 4 to 21 years. rh-GM-CSF was given at the dose of 5 micrograms/kg s.c. daily, starting either on the day following the last day of cytotoxic chemotherapy or when ANC < 1000/ml was determined. All patients received rh-GM-CSF for a total of seven days. Hematopoietic recovery occurred in all children except one. The response to rh-GM-CSF was achieved in a mean time of 7.4 days. Tolerance to rh-GM-CSF treatment was good. Adverse events were documented as fever, nausea, vomiting, fatigue, chills and itching. Sagittal sinus thrombosis developed in one patient 5 days following the completion of chemotherapy and rh-GM-CSF cycle. In conclusion, rh-GM-CSF can be applied during the intensive chemotherapy schedules of pediatric cancer patients.
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PMID:Granulocyte-macrophage colony stimulating factor (rh-GM-CSF) in the treatment of chemotherapy-induced neutropenia. 859 35

Patients with primary metastatic or recurrent rhabdomyosarcoma (RMS) have a very poor prognosis. Since high-dose chemotherapy (HDC) +/- TBI was thought to improve survival, many centers performed this therapy using different types of hematopoietic rescue (auto BM or PBSC, allo BM). This is a retrospective, multi-center analysis of the results of treatment in 36 patients with primary metastatic or relapsed RMS who were given HDC +/- TBI and hematopoietic rescue between 1986 and 1994. The median age was 6 years (< 1-22 years). Primary therapy was given according to either one of the Cooperative German Soft Tissue Sarcoma Studies CWS-81, -86, -91 or the European Study for Stage IV Malignant Mesenchymal Tumors in Childhood. There were 22 alveolar RMS, 13 embryonal RMS and one undifferentiated sarcoma. The indication for HDC was primary metastatic disease (27 patients) or a relapse of a primary localized tumor (nine patients). Thirty-two patients were in 1st or 2nd CR when given HDC and four in VGPR. The median time from last event to HDC was 44 weeks (21-110). HDC consisted of fractionated melphalan ((4 x 30-45 mg/m2), VP16 40-60 mg/kg, carboplatin 3 x 400-500 mg/m2) in 26 patients, 10 of whom received additional FTBI. Seven patients were treated with melphalan alone or in combination with carboplatin. Two patients received cyclophosphamide/busulphan with TLI (total lymphoid irradiation) and one cyclophosphamide with FTBI. Thirty-one patients were given autologous BM or PBSC as hematopoietic rescue and five allogeneic bone marrow from HLA-identical siblings. Fourteen patients received GM-CSF or G-CSF after hematopoietic stem cell transfusion (HSCT). Ten patients received adjuvant IL-2. There was one toxic HDC-related death. Nine patients are alive and free of disease with a median observation time of 57 months (32-108). The median time from HDC to relapse was 4 months (1-17). The tumor recurred in the majority of patients at previously known sites; in three cases new metastatic sites were observed. Patients with primary localized tumors who had been treated with HDC because of relapse did slightly better (four of nine alive with NED) than patients with primary metastatic disease (five of 27 alive with NED). HDC is still of uncertain value in the therapy of poor-risk rhabdomyosarcoma and should be performed only as part of controlled clinical trials.
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PMID:Do patients with metastatic and recurrent rhabdomyosarcoma benefit from high-dose therapy with hematopoietic rescue? Report of the German/Austrian Pediatric Bone Marrow Transplantation Group. 902 50


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