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)

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

Ifosfamide/mesna was given to 97 patients who had malignant solid tumors diagnosed before they were 21 years of age. Patients received 1.6 g/m2 ifosfamide daily x 5, given i.v. over 15 min, followed by 400 mg/m2 i.v. mesna at 15 min and 4 and 6 h after ifosfamide. Responses were noted in patients with osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma and other soft-tissue sarcomas, rhabdoid tumor, neuroblastoma, Wilms' tumor, primitive neuroectodermal tumor, retinoblastoma, germ-cell tumors, and B-cell lymphoma. Toxicity included mild to moderate nausea and vomiting, transient, reversible myelosuppression, transient elevations of serum blood urea nitrogen (BUN) and creatinine and liver enzymes, infections, and self-limiting neurotoxicity characterized by changes in mental status, motor dysfunction, cranial nerve palsy, cerebellar dysfunction, and seizures. Neurotoxic symptoms were generally seen in patients who had previously received cisplatin. Ifosfamide is an important alkylating agent that should be combined with other agents in phase II and III trials. Alternate dose schedules should also be investigated.
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PMID:Ifosfamide in pediatric malignant solid tumors. 250 57

Non phylloides sarcomas of the breast are infrequent malignant tumors that can be identified only at histologic examination since clinical presentation may not differ from the more frequent phylloides type. In addition to fibrosarcoma, leiomyosarcoma, rhabdomyosarcoma, angiosarcoma and liposarcoma, the stromal sarcoma of the breast has been recently identified. We report 3 such cases with heterologous components of osteosarcoma, chondrosarcoma and liposarcoma. A third case with an anti-sarcoma appearing post radiotherapy for breast carcinoma is also reported.
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PMID:[Unusual sarcomas of the breast. Report of 3 cases]. 251 99

We treated 14 patients with high grade sarcomas by angiotensin II-induced hypertension chemotherapy. The chemotherapy protocol described by Rosen was selected according to histological classification of sarcomas (small cell sarcoma, spindle cell sarcoma, pleomorphic sarcoma). The level of angiotensin-induced hypertension was one and half times as high as blood pressure at rest. Induced hypertension was maintained for 30-60 minutes. In three cases of 5 primary osteosarcomas, induced hypertension resulted in the increase of tumor stain and/or vascularity angiographically, and chemotherapeutic effects were CR or PR. The six cases with soft-tissue sarcomas were 2 cases each of CR, PR, and NC. The decrease of relative tumor blood flow under the condition of angiotensin II-induced hypertension was detected in 5 cases of 6 soft-tissue sarcomas by 133Xe clearance method. In the case of rhabdomyosarcoma, the decrease of tumor stain and vascularity by induced hypertension was observed on angiogram. As the side effects accompanying induced hypertension, nausea and chest oppression were noted in 2 cases, respectively. In this study it was suggested that angiotensin II-induced hypertension chemotherapy was effective for osteosarcoma, but that it might be ineffective for soft-tissue sarcomas.
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PMID:[Angiotensin II-induced hypertension chemotherapy of bone and soft-tissue sarcomas]. 254 22

The case records of and histopathologic findings in 57 dogs with nonangiogenic and nonlymphomatous splenic sarcomas were reviewed. Splenic neoplasms in these dogs included leiomyosarcoma, fibrosarcoma, undifferentiated sarcoma, liposarcoma, osteosarcoma, chondrosarcoma, myxosarcoma, rhabdomyosarcoma, and fibrous histiocytoma. The clinical signs associated with splenic sarcoma included anorexia or decreased appetite, abdominal distention, polydipsia, lethargy, vomiting, weight loss, and weakness. An abdominal mass was detected in 86% of the dogs by use of abdominal palpation (63%), and/or abdominal radiography (74%). The diagnosis was based on histopathologic findings in the spleen. Abdominal exploratory surgery was performed on 43 of the 57 dogs. Twenty-seven dogs were treated by splenectomy, and 16 were euthanatized at the time of surgery because of widespread metastatic lesions. Of the 14 dogs on which surgery was not performed, 11 were euthanatized on the basis of results of preoperative diagnostic tests, and the remaining 3 dogs had splenic neoplasms that were incidental findings at necropsy. Of the 27 surgically treated dogs, 5 died in the immediate postoperative period, 12 died or were euthanatized within 1 year after splenectomy, and only 5 dogs survived greater than or equal to 1 year. Three dogs were lost to follow-up evaluation, and 2 were still alive 6 and 7 months after surgery. The median survival time of the 22 dogs for which survival was known was 2.5 months. The median survival time for 11 dogs with no obvious metastasis at the time of splenectomy was 9 months.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nonangiogenic and nonlymphomatous sarcomas of the canine spleen: 57 cases (1975-1987). 255 65

Oral rhabdomyosarcoma (RMS) was studied by analysis of eight such cases which presented over a 25-year period. Rhabdomyosarcoma was the fourth most common oral sarcoma (7.5%) from this period after osteosarcoma (32%), fibrosarcoma (19%), and chondrosarcoma (9%). In patients younger than 20 years, RMS was the second most common sarcoma (six cases) after osteosarcoma (ten cases). Combined analysis with 113 further cases documented in the literature showed the majority of cases (71.2%) were embryonal. The alveolar subtype was considered a distant second in frequency (12.3%) even though the pleomorphic subtype was apparently more common (16.4%). However, the pleomorphic cases were diagnosed before histologic criteria were established to discern this entity from other pleomorphic sarcomas and this data is probably unreliable. Site predilections were found for the soft palate, maxillary sinus and alveolus, posterior mandibular region, cheek and lip and possibly tongue. The gingiva and floor of mouth were uncommon sites. There was a predilection for occurrence in the first two decades with a decline in the third decade. Documentation of recent cases treated with a multidisciplinary approach indicated that lesions in the oral soft tissues have a good prognosis; 17 of 21 such cases showed no evidence of disease after a mean follow-up period of 7.2 years (SD = 4.4). In contrast, four of five cases in the posterior mandible resulted in death after a mean period of 1.1 years (SD = 0.3).
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PMID:Rhabdomyosarcoma of the oral and paraoral region. 264 20

Sinonasal neoplasms and neoplasm-like proliferations composed of light microscopically poorly differentiated or undifferentiated, small- to medium-sized cells cause considerable diagnostic confusion. Lesions in this category include lymphoepithelioma (undifferentiated carcinoma), olfactory neuroblastoma, small-cell undifferentiated (oat cell) carcinoma, sinonasal undifferentiated carcinoma, malignant melanoma, pituitary adenoma, lymphoid hyperplasia, malignant lymphoma, plasmacytoma, lymphomatoid granulomatosis, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, Ewing's sarcoma, and synovial sarcoma. Many of these lesions can be definitively diagnosed based on light microscopic features alone, but, in some instances, additional techniques such as immunohistochemistry are of value. The authors review the pertinent clinicopathologic features of the above lesions, with emphasis on light microscopic, immunohistochemical, and ultrastructural features of particular utility in differential diagnosis.
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PMID:"Undifferentiated" neoplasms of the sinonasal region: differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. 269 5

We studied hyperthermia for malignant tumors of the extremities, and obtained the following findings. In osteosarcoma cultured cells from OST (Human) and Dunn (Mouse), proliferation was clearly inhibited on being heated to 42 approximately 43 degrees C. On heat-treating the femurs of pigs, a rise in temperature to 42.5 degrees C or above was observed so that an antitumor effect could be anticipated. Moreover, no abnormal rise in temperature in the tissues surrounding the bone and light microscopy revealed no particular abnormalities. Clinically, a rise in temperature above 42.5 degrees C was observed in the majority of the malignant bone tumors (4 cases of osteosarcoma and 1 case of chordoma) and soft tissue tumors (1 case of epithelioid sarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma, malignant melanoma and osteosarcoma) of which 2 cases were metastatic tumors. Before administration, 7 patients complained of pain, 4 of whom (57%) experienced an alleviation following treatment. Also in 5 (50%) out of 10 cases a shrinking of the tumor was observed and especially, in the case of soft tissue tumors a tendency towards a softening of tumor texture was seen.
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PMID:[Hyperthermia in malignant tumors of the extremities--experimental heating by a radiofrequency applicator and its clinical significance]. 273 74

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.
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PMID:Aggressive management of second primary tumors in survivors of hereditary retinoblastoma. 277 44

Twenty-four patients with malignant tumors of the external auditory canal and middle ear, originally seen between 1960 and 1980, were reviewed retrospectively. Seventeen patients had epidermoid carcinoma, one had adenocarcinoma, three had rhabdomyosarcoma, and one had osteosarcoma. At presentation, four had disease confined to the external auditory canal, three had superficial invasion of the bony canal, four had deeply invasive disease, and thirteen had disease that extended beyond the temporal bone. Treatment consisted of radiation, surgery, and combination therapy. Four patients with osteosarcoma or rhabdomyosarcoma received adjuvant chemotherapy. Five years after therapy, one of four patients with external auditory canal tumor died of disease, and two died of intercurrent disorders. One of three patients with superficial temporal bone invasion and two of four patients with deeply invasive tumor died of their disease; another died of intercurrent disorder. Twelve of 13 patients with tumor beyond the temporal bone died.
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PMID:Malignant tumors of the middle ear and external auditory canal: a 20-year review. 282 5


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