RESUMEN

Desmoplastic small round cell tumor (DSRCT) is rare and highly malignant neoplasm. DSRCT affects usually young males but can occur in adults also. Intra-abdominal pelvic region is the preferred site. Though confirmed by histology, immunohistochemistry (IHC) plays a key role in diagnosis. IHC profile is characteristic, it shows simultaneous expression of epithelial (epithelial membrane antigen (EMA) and cytokeratin (CK)), muscular (desmin), and neural (neuron-specific enolase) markers. Many cases of DSRCT are diagnosed as poorly differentiated carcinoma due to lack of proper panel of IHC. It is difficult to predict if there has been a true increase in incidence. Prognosis is uncertain in such an aggressive neoplasm as chemotherapy (CT) or radiotherapy (RT) shows various outcomes. Here in; we report four cases, all of which showed diagnostic dilemma and uncertain prognosis.

Asunto(s)

Neoplasias Abdominales/diagnóstico , Biomarcadores de Tumor/metabolismo , Tumor Desmoplásico de Células Pequeñas Redondas/diagnóstico , Neoplasias Abdominales/clasificación , Neoplasias Abdominales/tratamiento farmacológico , Neoplasias Abdominales/metabolismo , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Tumor Desmoplásico de Células Pequeñas Redondas/clasificación , Tumor Desmoplásico de Células Pequeñas Redondas/tratamiento farmacológico , Tumor Desmoplásico de Células Pequeñas Redondas/metabolismo , Femenino , Humanos , Técnicas para Inmunoenzimas , Masculino , Pronóstico