RESUMO
The triad described by J.A. Carney in 1977 is characterized by the successive or concomitant occurrence of three rare tumours of different origin: gastric leiomyoblastoma, functioning extra-adrenal paraganglioma and pulmonary chondroma. It is found mainly in young female patients with prevalent blood group A and may be incomplete, with only two of the three tumours occurring. The case of a young woman who developed gastric leiomyoblastoma and, six years later, pelvic paraganglioma is reported; she died of metastases from the paraganglioma with malignant hypertension. The 16 cases already published are reviewed. The authors insist upon the severity of paragangliomas and the need for a systematic search for the other tumours in patients who present with one tumour of the triad. A histogenetic interpretation relating the tumours to pathology of the neural crest is suggested.