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 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

A phase II study of VP-16, a semisynthetic Podophyllotoxin, was performed in patients with solid tumors. VP-16 was administered orally at a dose of 200mg/day for 5 consecutive days at 3 to 4-week intervals. Out of 41 patients who were entered into the study, 35 patients comprising 17 lung cancer, 10 hepatoma and 8 other tumors were evaluable. There were 4 partial responses (23.5%) for lung cancer, 1 (10.0%) for hepatoma and 1 for rhabdomyosarcoma. Overall response rate was 18.2% for patients with prior chemotherapy and 15.4% for those given no prior chemotherapy respectively. Thus the results indicated VP-16 has no cross-resistance to other antitumor agents. Leukopenia (less than 4,000/mm3) and thrombocytopenia (less than 10 X 10(4)/mm3) were observed in 72.7% and 29.4% of the patients, respectively. Other toxicities were alopecia (59.5%) and gastrointestinal disturbances such as nausea (46.2%), vomiting (20.5%) and anorexia (20.5%), but these were all well tolerated.
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PMID:[Phase II study of VP-16 (capsule) in solid tumors. A cooperative study]. 298 32

Children undergoing ABMT, a procedure which entails massive doses of chemotherapy along with total-body irradiation, are candidate to develop severe gastrointestinal toxicity and prolonged anorexia requiring administration of Parenteral Nutrition (PN) for variable periods. We report a series of 35 consecutive children affected by malignancies who underwent 37 courses of PN after ablative therapy followed by ABMT. Age ranged from 8 months to 17 years; 16 were females, 19 males. There were 23 cases of neuroblastoma, 5 of Wilms' tumor, 3 of acute myelogenous leukemia, 2 of Ewing's sarcoma, 1 case each of rhabdomyosarcoma and acute lymphoblastic leukemia. All patients developed severe neutropenia for 9-42 days (median 18 d). Fever occurred in all patients; sepsis was documented in 10. Duration of PN ranged from 10 to 64 days (23 +/- 9; mean +/- SD). PN solution, containing crystalline L-Aminoacids (8.5%) mixed with 33% glucose, minerals, trace elements and vitamins provided for children a caloric intake of 49.8 +/- 17.3 Kcal/Kg/day with a nitrogen intake of 0.26 +/- 0.27 g/Kg/day. Nutritional assessment, utilizing percent ideal body weight, serum protein electrophoresis, C3, pseudocholinesterase and fibrinogen, was performed at the beginning and at the completion of each course of PN. Mean percent ideal body weight was 95.8 before PN, 98.5 on last day of PN (p less than 0.0005). Other parameters did not change significantly. No metabolic complication nor severe electrolyte imbalance were observed except for 5 patients who developed hypokalemia in coincidence with administration of Amphotericin B.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Autologous bone marrow transplantation in children. Use of parenteral nutrition]. 311 38

Ten cases of rhabdomyosarcoma of the biliary tree (RMS/BT) in children are reported in this review of the Intergroup Rhabdomyosarcoma Study (IRS) I and II. RMS/BT constitutes 0.8% of evaluable tumors on IRS I and II. Intermittent obstructive jaundice with or without abdominal distention, fever, and loss of appetite is the typical presentation. Attribution of these symptoms to hepatitis commonly delayed definitive treatment. Demonstration of a mass in the porta hepatitis by ultrasound led quickly to surgical consultation and an exploratory laparotomy. Intraoperative cholangiography was a valuable technique in establishing the level of biliary tree obstruction and verifying a functioning drainage procedure. Resection of the mass with only microscopic or minimal gross residual disease was possible at the initial surgery in six of the ten patients. Included in this group are all four of the surviving patients. The continuity of bile flow was usually maintained by variations of a Roux-en-Y jejunostomy. Histology was embryonal RMS with some botryoid elements. Consultation with a surgical pathologist during the course of the surgery was of invaluable assistance. Multi-drug chemotherapy and radiotherapy consistent with the IRS protocols were given postoperatively. A second-look surgery was useful in evaluating residual or recurrent disease. Four patients are surviving free of disease at 6 months, 3, 6 1/4, and 6 1/2 years from diagnosis, respectively. This review demonstrates that a multidisciplinary approach in RMS/BT can lead to long-term survival.
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PMID:Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study. 400 14

A 14 year old girl presented in 1986 with a huge perineal swelling which was progressively increasing in size and associated with loss of weight and loss of appetite. Biopsy from the mass revealed rhabdomyosarcoma of the vulva. She was treated with chemotherapy and radium implant. She responded well to the regime. Fibrosis of the vulva and vagina caused difficulty in consummation. Once it was corrected, she conceived easily and proceeded to a normal pregnancy and delivery.
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PMID:A successful pregnancy outcome in treated vulval rhabdomyosarcoma. 1104 67

A seven-year-old domestic shorthair (DSH) cat was presented with anorexia and dyspnea. Pleural-pericardial effusion was detected with thoracic radiographs and echocardiography. Echocardiography demonstrated a large, soft-tissue mass in the right ventricular wall, protruding both into the pericardial space and into the right ventricle. Postmortem examination findings included a large mass in the right ventricular wall and multiple smaller masses on the external surface of the left ventricle and on the internal surface of the pericardium. Results of the histopathological and immunohistochemical examinations of the masses were consistent with rhabdomyosarcoma. This is the first reported case of primary cardiac rhabdomyosarcoma in the cat.
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PMID:Primary cardiac rhabdomyosarcoma in a cat. 1130 May 23

Rhabdomyosarcoma accounts for 20% of primary malignant cardiac tumours. We describe a woman with cardiac rhabdomyosarcoma. She had loss of appetite, loss of weight, and symptoms of heart failure. A mobile tumour in the left atrium, entering the left ventricle during diastole, was detected by transthoracic echocardiography. The tumour was removed surgically in total; histological diagnosis was rhabdomyosarcoma; however, the patient died after 6 months. In conclusion, primary malignant cardiac tumours can simulate heart failure or systemic disorders. Cardiac rhabdomyosarcomas have a poor prognosis with average survival of 6 months after diagnosis.
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PMID:Heart failure caused by cardiac rhabdomyosarcoma. 1910 99

Pazopanib, an oral tyrosine kinase inhibitor, is the first molecular-targeted agent approved for the treatment of advanced soft tissue sarcoma(STS). Rhabdomyosarcoma in adults is rare, accounting for less than 3%of all adult STS cases. A 57-year old woman presented with cervical lymphadenopathy. Computed tomography revealed a heterogeneous mass in the retroperitoneum, replacing the entire right kidney. On the basis of the above findings, the patient was diagnosed with alveolar rhabdomyosarcoma. She was first treated with 4 courses of vincristine, actinomycin D, and cyclophosphamide(VAC), which resulted in a partial response. Dose reduction and delay occurred owing to hematological toxicity and febrile neutropenia. As second-line chemotherapy, the patient was administered a single daily dose of 800 mg of pazopanib. Because of an episode of hand-foot syndrome and hepatic impairment, the 800-mg daily dose of pazopanib was reduced to a daily dose of 600 mg, which had to be further reduced to a daily dose of 400 mg owing to fatigue and anorexia. The patient maintained a partial response for a total of 4.3 months when treated with pazopanib. Therefore, this drug may be a new treatment option for patients showing metastatic STS after previous chemotherapy.
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PMID:[Effective treatment of metastatic rhabdomyosarcoma with pazopanib]. 2513 42

We present a rare case of retroperitoneal dedifferentiated liposarcoma (DDLPS) masquerading as rhabdomyosarcoma. The patient was a 74-year-old man, complaining a loss of appetite. Abdominal computed tomography revealed a retroperitoneal mass, 10 cm in diameter, between the liver and the right adrenal gland. The tumor was resected and histologically diagnosed as conventional DDLPS, in which dedifferentiated component was highly cellular and composed of pleomorphic anaplastic cells. After 3 years, the tumor recurred in the right retroperitoneal space. The recurrent tumor consisted of 2 components: lipogenic and nonlipogenic. The latter differ from the dedifferentiated component of the primary tumor. The tumor cells were small, round to ovoid cells with monomorphous, round, hyperchromatic nuclei, and scant cytoplasm. Interestingly, they were diffusely positive for myogenin and desmin. To rule out the possibility of the second primary, we performed fluorescence in situ hybridization to detect FOXO1 rearrangement. We failed to demonstrate splits of the probes. In contrast, high-level amplification of MDM2 was detected by dual-color in situ hybridization. Given the morphologic and molecular findings, the neoplasm was identified as a peculiar DDLPS mimicking rhabdomyosarcoma. Retroperitoneal rhabdomyosarcoma-like tumors of adults, therefore, should be distinguished carefully from DDLPS. It could be challenging when lipogenic component was absent, but in situ molecular analyses can be helpful.
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PMID:Dedifferentiated Liposarcoma Masquerading as Rhabdomyosarcoma. 2780 31

A 13-year-old female scimitar-horned oryx (Oryx dammah) died after progressive anorexia, weight loss, and depression. The necropsy showed that the retroperitoneum was compressed by a large white-to-tan uterine mass and on several sections of the mass, the uterine wall was markedly thickened because of ill-defined transmural tumor tissue. Metastatic nodules were detected in the omentum, mesentery, diaphragm, and lung. The genital tract and pulmonary and abdominal nodules exhibited highly pleomorphic sarcoma. The primary and metastatic neoplastic cells showed positive results for vimentin, desmin, and sarcomeric actin, and negative results for smooth muscle actin. Uterine metastatic rhabdomyosarcoma was diagnosed on the basis of the gross, histopathology and immunohistochemistry results.
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PMID:Uterine metastatic rhabdomyosarcoma in a scimitar-horned oryx (Oryx dammah). 3018 25


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