Primary embryonal rhabdomyosarcoma of the liver in a young male
JCPSP-Journal of the College of Physicians and Surgeons Pakistan. 2013; 23 (10): 750-751
em Inglês
| IMEMR
| ID: emr-140815
ABSTRACT
Rhabdomyosarcoma [RMS] occurs infrequently in the liver. Rhabdomyosarcomas are malignant tumours that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children. In adults however, these are very rare. We report a case of a primary embryonal rhabdomyosarcoma of the liver in a 17 years old boy. This was confirmed by histological examination using immunohistochemical analysis [LCA negative, desmin positive, myogenin focally positive and cytokeratin negative] and site was confirmed by PET CT scan. He received multiple chemotherapies including [doxorubicin, ifosfamide, dacarbazine; gemcitabine, paclitaxel; vincristine, actinomycin D, cyclophosphamide] but longest sustained stable disease was seen with gemcitabine-paclitaxel regimen. The patient died 31 months after the first presentation, secondary to complicated abundant abdominal progressive disease. The poor prognosis and early death of most previously reported cases imply the need for investigation of a more effective treatment method of this uncommon tumour
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Índice:
IMEMR (Mediterrâneo Oriental)
Assunto principal:
Paclitaxel
/
Rabdomiossarcoma Embrionário
/
Desoxicitidina
/
Tomografia por Emissão de Pósitrons
/
Neoplasias Hepáticas
Tipo de estudo:
Relato de Casos
Limite:
Humanos
/
Masculino
Idioma:
Inglês
Revista:
J. Coll. Physicians Surg. Pak.
Ano de publicação:
2013
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