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)

Eosinophilic cystitis is an uncommon inflammatory disorder of the urinary bladder which causes irritative voiding symptoms and may mimic rhabdomyosarcoma radiographically. In children, eosinophilic cystitis has been previously reported to be self-limiting and requires no specific therapy. Reported herein is a case of a nine-year-old girl in whom eosinophilic cystitis recurred following antireflux surgery, raising the question of an association of eosinophilic cystitis with local trauma such as bladder surgery. Consideration should be given to pretreatment with steroids and antihistamines prior to surgery in these patients.
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PMID:Eosinophilic cystitis in children. 203 90

One hundred twenty-four children and young adults with recurrent tumors, predominantly sarcomas, were treated with the combination of ifosfamide, etoposide, and the uroprotector, mesna (2-mercaptoethane sulphonate), in a phase II trial. The treatment regimen consisted of 12 cycles of therapy administered every 3 weeks. After evaluation of the tumor response to chemotherapy alone, radiation or surgery was used to eradicate residual sites of metastatic disease where possible. At the present time, 77 patients are evaluable for response to the chemotherapy; 43 of the patients have experienced a significant reduction in the tumor size in response to the chemotherapy alone (39 partial responses [PR] and four complete responses [CR]). Sixteen of 17 patients with Ewing's sarcoma, nine of 13 with rhabdomyosarcoma, four of eight with peripheral neuroepithelioma, three of eight with osteosarcoma, and 11 of 31 with other tumors have responded with a PR or CR. The toxicity of the regimen was acceptable. Moderate or severe toxicity evaluated on a per cycle basis included: neutropenia, 97%; thrombocytopenia, 32%; nephrotoxicity, less than 1%; mucositis, 1%; neurologic toxicity, 2%; nausea and vomiting, 13%; hemorrhagic cystitis, less than 1%. Fever was present after 33% of cycles and sepsis following 7%. One patient died due to sepsis and pancytopenia. At the present time, only seven of the 43 patients who responded to the chemotherapy regimen have relapsed, with a median follow-up of 10 weeks after the response. This drug combination is highly active in the treatment of recurrent sarcomas and other tumors in children and young adults.
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PMID:Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults. 311 35

We have evaluated the activity of ifosfamide in 75 patients with recurrent sarcomas and pediatric solid tumors. All patients had previously received cyclophosphamide in combination with other chemotherapeutic agents. Ifosfamide was administered as a continuous 5 day infusion at a dose of 1800 mg per M2, except in the last 14 patients who received the drug as a daily one hour infusion at the same dose level. Partial response was observed in 9 of 20 patients with Ewing's sarcoma, 2 of 9 patients with rhabdomyosarcoma, 3 of 17 patients with osteogenic sarcoma and 4 of 29 patients with various other neoplasms. A further 6 patients had stable disease, defined as the absence of progression for at least 6 cycles of therapy. Thus overall response rate was 24%, with the highest response rate of 45% being observed in Ewing's sarcoma. Toxicity was acceptable, although there was quite marked leucopenia (median nadir 700) with less profound thrombocytopenia (median nadir 87,000). Sepsis occurred in 3 patients but no patient died as a result of infection. Hematuria occurred in 43% of patients who did not receive mesna, and in 26% of patients who did, although prior pelvic irradiation was found to be a significant risk factor for hematuria. Only 1 of 14 patients without prior pelvic irradiation or hematuria developed hemorrhagic cystitis when treated with ifosfamide and mesna. Confusional states developed in 6 patients. We conclude that ifosfamide is an active agent in patients with relapsed sarcomas and childhood solid tumors, even when such patients have been previously treated with cyclophosphamide.
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PMID:A phase II study of ifosfamide in the treatment of recurrent sarcomas in young people. 381 17

One hundred one eligible children with soft-tissue sarcomas arising within the retroperitoneal space have been registered on Intergroup Rhabdomyosarcoma Study Committee (IRS) studies I and II and followed for at least 2 years or until death. The most common presenting symptoms and signs were pain in the abdomen or lower extremities, and/or an abdominal mass, usually noted by a parent or a physician seeing the child for other complaints. The median age at diagnosis was 6.5 years and the sex ratio (M/F) 1.7:1. Histologic types were embryonal or botryoid rhabdomyosarcoma (RMS) in 58 patients, alveolar RMS in 8, pleomorphic RMS in 2, undifferentiated sarcoma in 20, extraosseous Ewing's sarcoma in 4 and unspecified sarcoma in 9. Median tumor size was 10 cm, significantly larger than the 7.5 cm noted in the IRS studies as a whole (P less than 0.05). One patient had complete tumor removal (Group I); 12 had grossly complete removal with microscopic residual tumor (Group II). Fifty-one patients had residual local tumor after biopsy or partial resection (Group III) and 37 patients had distant metastases at diagnosis (Group IV). Treatment included surgery, radiation therapy (RT) and combination chemotherapy with vincristine and actinomycin D with or without cyclophosphamide and Adriamycin (doxorubicin) according to protocol. Thirty-nine of 99 patients (39%) had major difficulties in the delivery of specified RT. Seventy patients received sufficient therapy to be evaluable for treatment response. Forty-one (58%) achieved a complete remission and 16 (23%) achieved partial remission. Twenty-four of 41 children (59%) achieving a CR have relapsed. The proportion of children who remained relapse-free at 2 and 3 years of follow-up was 44% or 42%, respectively. Overall, 40 children have developed recurrent sarcoma and the median disease-free interval and overall survival times were 54 and 88 weeks, respectively. Most children experienced severe myelosuppression; there were three early deaths from infection which occurred during granulocytopenic periods. One third of the patients experienced cystitis. Children with soft-tissue sarcomas arising in the retroperitoneal space have a poor prognosis associated with large tumors which, because of location and/or extent (Groups III or IV), are unresectable and difficult to treat.
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PMID:Soft tissue sarcomas arising in the retroperitoneal space in children. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. 389 67

Twenty children with recurrent or unresponsive tumours (10 Wilms', 3 rhabdomyosarcoma, 4 Ewings's, 1 osteosarcoma, 1 hepatoblastoma, 1 hepatoma) and one untreated patient with renal carcinoma were given ifosfamide as a 24-h infusion (5 mg/m2), with mesna as uroprotective. The number of courses ranged from 1 to 13 (median 3), and the interval between them was 2-3 weeks. Sixteen of these patients had previously received cyclophosphamide. Complete clinical responses were seen in 3 cases (2 Wilms' and 1 Ewing's) and lasted 5, 7, and 9 months. Partial responses were seen in 3 instances, mixed response or stable disease in 4, and progressive disease in 11. Treatment was well tolerated in most patients, with no cystitis or severe myelosuppression, but 2 children developed transient neurological symptoms and 1 became hypertensive. Nausea and vomiting were controlled by high-dose dexamethasone in most children. Plasma ifosfamide levels were estimated by means of gas-liquid chromatography in 10 patients. Peak concentrations ranged from 38 to 125 micrograms/ml (median 80). The elimination half-life, at 2.5-5.2 h (median 3.2) was shorter than previously reported in adults. Future studies should test the possibility that ifosfamide-containing combination chemotherapy may be more effective than the regimens, usually including cyclophosphamide, that are currently used as front-line treatment of embryonal and Ewing's sarcoma.
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PMID:A phase II study of ifosfamide in children with recurrent solid tumours. 405 69

Intensive chemotherapy followed by infusion of cryopreserved autologous bone marrow (ABMR) was used in the treatment of 22 children with advanced tumours. In nine this was their initial therapy; in eight it was used after partial or complete remission had been achieved with standard therapy; and in five, after relapse had occurred. Recovery of marrow function occurred in 20 patients with a mean time of 13.2 and 18.2 days to recovery of neutrophils (greater than 0.5 X 10(9)/l) in newly diagnosed and previously treated patients respectively. Platelet count recovery to greater than 50 X 10(9)/l occurred in a mean time of 13.4 days in newly diagnosed and 20.4 days in previously treated patients. Control of extensive local tumour was obtained in three of three evaluable patients with abdominal non-Hodgkin's lymphoma (NHL). Metastatic bony and marrow disease was controlled in two of two patients with retinoblastoma. In Ewing's sarcoma, temporary control of widespread metastatic disease occurred in one patient. In the other, eradication of extensive local mass disease at the primary site had been achieved. Poor response to treatment has been seen in seven of eight patients with Stage III or IV rhabdomyosarcoma, three patients with neuroblastoma and four of five patients with recurrent disease. Apart from the anticipated bone marrow toxicity, the major complications were severe mucositis, anaphylaxis following bone marrow infusion and haemorrhagic cystitis. The presence of herpes simplex infection appeared to aggravate mucosal complication.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Experience with high dose multiagent chemotherapy and autologous bone marrow rescue in the treatment of twenty-two children with advanced tumours. 639 55

Seventeen (13 male, 4 female) patients, aged 13 months to 13 years, with benign cystitis had imaging findings that mimicked those of rhabdomyosarcoma. Our experience indicates that in the child with hematuria, dysuria, and frequency plus cystographic or sonographic demonstration of a bladder with reduced capacity and circumferential wall thickening or sonographic findings of isoechoic bladder wall thickening (focal, multifocal or circumferential distribution), intact mucosa, and bullous lesions should strongly suggest inflammation and not malignancy. When an inflammatory lesion is suspected, follow-up imaging should be performed in 2 weeks, which if normal will preclude biopsy.
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PMID:Benign cystitis in children mimicking rhabdomyosarcoma. 787 2

Because of its ballistic and physical characteristics, brachytherapy (BT) represents the optimal irradiation treatment in pediatric tumors, especially in prostate or bladder rhabdomyosarcoma. In a limited series of 15 children treated with BT (including three patients treated with salvage BT), 8 out of 9 patients surviving with NED benefited from conservative treatment among 12 patients treated with BT as first-line treatment. Sequelae were minimal consisting of one grade I cystitis and one asymptomatic vesical and ureteral reflux. BT allows administration of high doses to limited volumes sparing normal tissues. BT--whenever possible--represents the treatment of choice compared with external radiotherapy. Indications of BT depend on tumor accessibility and size (usually < or = 4 cm).
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PMID:[The role of brachytherapy in the therapeutic strategy of vesico-prostatic rhabdomyosarcoma in children]. 789 15

The therapeutic effects of Factor XIII (F XIII) concentrate against drug-induced hemorrhagic cystitis (HC) was investigated. HC occurred in 4 children with malignant disease during anti-cancer chemotherapy. Two (CML and T-ALL) of 4 patients developed HC after the administration of high dose cyclophosphamide as conditioning for allo bone marrow transplantation or peripheral blood stem cell autografts, and the other 2 patients (rhabdomyosarcoma, Wilm's tumor) developed HC after the administration of ifosfamide for relapse. When F XIII concentrate at a dose of 20 to 230 U/kg was administrated immediately after the onset of HC, the symptoms, i.e., bladder irritability and macrohematuria disappeared within a few days. The F XIII serum levels of those patients were low (27-57%), and the levels increased (63-230%) after administration of F XIII concentrate. The two patients with relapsed solid tumor showed no symptoms of HC during subsequent ifosfamide treatment when F XIII concentrate was administrated to maintain a normal F XIII range. These results suggest that the administration of F XIII concentrate may be useful for the prophylaxis and treatment of drug-induced HC in patients with a low F XIII level.
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PMID:[The clinical effect of factor XIII on drug-induced hemorrhagic cystitis]. 815 49

Pseudotumoral cystitis is an unusual lesion and since it may be confused with rhabdomyosarcoma, knowledge of its existence is important. We report eight cases.
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PMID:Pseudotumoral cystitis. 1121 84


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