Carney triad: A case report, characteristics and literature review of this rare entity

INTRODUCTION AND IMPORTANCE: Carney triad is a rare entity of unknown etiology, characterized by the association of tumors with low incidence such as: gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma. We show a case of Carney triad diagnosed in our center that has some different characteristics to the typical presentation of this patology, and a review of the literature. CASE PRESENTATION: We present the case of a 47-year-old men who was admitted to our hospital for upper gastrointestinal bleeding and anemia. The patient was diagnosed of gastric GIST (gastrointestinal stromal tumor), pulmonary nodule and retroperitoneal nodule close to iliac bifurcation. A review of his medical history revealed that he had undergone surgery at 18-year-old for gastric leiomiosarcoma, subtotal gastrectomy performed with gastroenteric anastomosis type Billroth II. The patient underwent total gastrectomy with a end-lateral esophago-jejune mechanical anastomosis and retroperitoneal tumor was also excised. Pathological result confirmed gastric GIST, and extra-adrenal paraganglioma. CLINICAL DISCUSSION: We review the available literature to determinate the characteristics and the most appropriate management for this syndrome. The trend is surgical treatment of gastric GIST and paraganglioma, preferred conservative treatment in pulmonary chondroma. It is under discussion whether gastric tumors Carney syndrome is considered GIST, because the Carney triad lesions in stomach are different clinically, pathologically, and etiologically from GIST of stomach. CONCLUSION: Carney's triad is a low prevalence entity, with a limited number of publications, so the presentation of the syndrome may be different from the presentation described in the literature.