RESUMO
A 12-year-old boy presented acutely after an episode of syncope with perioral cyanosis. He died 19 hours after admission due to cor pulmonale as a complication of metastatic desmoplastic small round cell tumor in the lungs with associated tumor thrombotic microangiopathy.
Assuntos
Tumor Desmoplásico de Pequenas Células Redondas/complicações , Neoplasias Pulmonares/complicações , Doença Cardiopulmonar/etiologia , Microangiopatias Trombóticas/etiologia , Criança , Evolução Fatal , Humanos , MasculinoRESUMO
The desmoplastic small round cell tumor is infrequent.That mainly affects male youngsters and is normally located at the abdomino-pelvic cavity, being its clinic unspecific. The diagnosis is confirmed by the presence of a specific chromosomal translocation: t (11; 22), (p13; q12). As to its treatment, it is mostly recommended to follow a multimodal aggressive one. We present the case of a man, whose atypicality is due to his advanced age (63 years old) and the symptoms he presents (lumbar ache).