Carney's triad: case report and review.

INTRODUCTION: Carney's triad is a rare pathogenic entity which consists of the association in young women of multiple condromatosis in the lung, gastric leiomyosarcoma, and extradrenal paraganglioma; although the presence of three at the same time is not required for its diagnosis. CASE REPORT: We present the case of a 27-year-old woman who was diagnosed of pulmonary multiple hamartomatosis and gastric stromal tumor. DISCUSSION: A review of the literature shows the most important prognosis factors and therapeutic options. Surgery for gastrointestinal stromal tumors and extradrenal paraganglioma seems to be the best treatment up to date.

[Gastrointestinal stromal tumors (GIST): factors predictive of survival after R0-cytoreduction]. / Tumores del estroma gastrointestinal (GIST): factores pronósticos de supervivencia tras citorreducción R0.

OBJECTIVE: To analyze the different factors predictive of survival associated with optimal R0-cytoreduction in c-kit-positive gastrointestinal stromal tumors. METHODS: Thirty-five patients were operated on in our Oncological Surgery Department from January 2002 to February 2007 because of CD117/c-kit-positive gastrointestinal stromal tumors, and an optimal surgical cytoreduction was obtained without macroscopical residual disease. Demographic, anatomical, clinical, pathological, and immunohistochemical variables were analyzed from a specific database. Survival and multivariate analyses were developed using Kaplan-Meier and multiple Cox regression models, respectively. RESULTS: Five-year overall survival was 77% with a mean survival of 52 months. Risk of malignant behaviour according to Fletcher s classification and tumor size higher than 10 cm had a significantly negative influence on overall survival in the univariate analysis (p < 0.05). Proliferative Ki-67 activity higher than 50% was the only statistically significant variable in the multivariate analysis. Twenty percent of tumors recurred. Only 3 patients with metastatic disease received adjuvant treatment with imatinib mesylate, all of them with Ki-67 > 50% and currently alive. CONCLUSIONS: The poliferative Ki-67 index could represent an excellent predictive factor for survival in patients with c-kit-positive stromal gastrointestinal tumors. Confirmation and an adequate cut-off level should be the main objectives for future prospective studies, mostly focused on the appropriate selection of optimal candidates to imatinib-mesylate-based treatment.