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)

This report describes two cases of pulmonary rhabdomyosarcoma occurring in children, ages 21 and 30 months at diagnosis. Both patients presented with spontaneous pneumothorax and had cystic changes in the affected lung by chest x-ray. Ultrastructural and immunohistochemical studies supported the myogenic phenotype of these neoplasms. The authors were unable to confirm that either of these tumors had unequivocally originated in a congenital cyst although previous authors have reported this association. An apparent morphologic spectrum of embryonic appearing neoplasms including pulmonary blastoma and a malignant mesenchymoma have been observed in the lungs of children.
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PMID:Primary pulmonary rhabdomyosarcoma of the lung in children. Report of two cases presenting with spontaneous pneumothorax. 381 59

From 1987 to 1992, 22 children (age 0 days-14 years) were operated for congenital bronchopulmonary disease. One patient had a hamartoma. Four children had a bronchogenic cyst, in 1 patient combined with an esophageal duplication. Intrapulmonary sequestration was diagnosed in 3 children; one of whom had an esophageal duplication as well. Four children had localized emphysema. In 1 patient histology showed rhabdomyosarcoma. A lobectomy was performed following chemotherapy but recurrence was not resectable at a third thoracotomy. Ten patients had cystic adenomatoid malformation, 6 were dependent on artificial ventilation before surgery. Three patients with cystic adenomatoid malformation died in hospital: 2 after pneumonectomy who were shown to have bilateral disease and 1 died after bilobectomy and was shown to have a hypoplastic contralateral lung. Hospital morbidity involved 1 patient with recurrent pneumothorax, 2 with atelectasis of the operated lung and 3 with prolonged artificial ventilation. Late mortality involved 1 patient due to pulmonary rhabdomyosarcoma. After 1-26 months of follow-up there were no late complications. Adequate multidisciplinary treatment allows acceptable mortality and low morbidity in surgery for congenital bronchopulmonary disease in children.
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PMID:Surgical treatment of congenital bronchopulmonary disease in children. 846 Nov 43

The authors report a case of primary rhabdomyosarcoma of the diaphragm, an extremely rare presentation with only 14 cases reported in the literature. An 18-year-old male presented 2 spontaneous occurrences of pneumothorax. Computed tomography and magnetic resonance showed a tumoral mass on the right diaphragmatic surface, and after biopsy, the diagnosis was compatible with spindle cell rhabdomyosarcoma. Because the visceral pleura was invaded by the tumoral mass, a right pleuropneumonectomy was performed. The patient received adjuvant chemotherapy, and there was no evidence of disease 15 months after the operation. Based on the Intergroup Rhabdomyosarcoma Study Group (IRSG) criteria, which consider the extent of the disease and its surgical resectability, rhabdomyosarcomas can be classified into 4 groups. In clinical group I, which was the classification of our patient, the tumor is localized and completely resectable, which implies a good prognosis. Rhabdomyosarcoma is a rare tumor, and a good outcome may result if it is completely resected.
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PMID:Primary rhabdomyosarcoma of the diaphragm: case report and literature review. 1198 87

Primary pulmonary rhabdomyosarcoma in children is rare. Three children aged three, nine and three years were evaluated for abnormal shadows on radiological examination with pneumothorax in two cases. Resection and histopathological examination revealed embryonal rhabdomyosarcoma in all and cystic malformation in first case. All the three children were treated with surgery and first two received adjuvant chemotherapy. The disease free duration was 160 months, 19 months and seven months respectively. The literature on primary pulmonary rhabdomyosarcoma in children was reviewed.
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PMID:Primary pulmonary rhabdomyosarcoma in children: Report of three cases with review of literature. 2387 87

We present 2 patients who developed spontaneous pneumothorax (SP) following rapid regression of lymphoma and rhabdomyosarcoma with lung metastases. Case 1, a 43-year old man was admitted to our hospital with dyspnea 10 days before admission. He denied any recent trauma or previous treatment for lung tuberculosis. Three weeks prior to admission, he received first cycle of CHOP for non-Hodgkin's lymphoma stage II BE. Chest X-ray consistent with right pneumothorax. After treatment with chest tube drainage for about 1 month, the patient recovered and chemotherapy could be continued without further complications. Case 2, a 35- year old man was admitted to other hospital with dyspnea and chest pain on day 4 after second cycle of systemic combined chemotherapy for rhabdomyosarcoma stage IV (lung metastases) with doxorubicin, ifosfamide, mesna, and dacarbazine. Chest X-ray showed hydropneumothorax on right and left lung. After treatment with chest tube drainage about 2 weeks, the patient recovered and chemotherapy could be continued without further complications. The mechanism of pneumothorax following chemotherapy is not clearly understood yet, however, several hypotheses have been considered: 1) the rupture of a subpleural bulla after chemotherapy; 2) the rupture of an emphysematous bulla in an over expanded portion of the lung which is partially obstructed by a neoplasm; 3) tumor lyses or necrosis due to cytotoxic chemotherapy directly induces the formation of fistula. Dyspnea and chest pain suddenly appear during successful chemotherapy for metastatic chemosensitive tumors should alert the physician to the possibility of SP. The treatment is directed toward lung re-expansion. Chemotherapy induced pneumothorax should be considered as oncologic emergency.
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PMID:Chemotherapy-induced Spontaneous Pneumothorax: Case Series. 2755 Aug 83