CARCINOMA MEDULAR DE TIROIDES: ESTUDIO MULTICÉNTRICO, PRESENTACION Y EVOLUCION EN 219 PACIENTES

CASTRO JOZAMI L; CALIFANO I; FASSI J;; *DEUTSCH S; LOWENSTEIN A; BALZARETTI M; NOVELLI JL; FIGARI M;; OLSTEIN G; SANSÓ G; BARONTINI M;; IORCANSKY S; CABEZON C

CABA

Congreso; CARCINOMA MEDULAR DE TIROIDES: ESTUDIO MULTICÉNTRICO, PRESENTACION Y EVOLUCION EN 219 PACIENTES; 2011

Sociedad Argentina de Endocrinología y Metabolismo

Due to the low frequency of medullary thyroid cancer (MTC), an observational, cohort, retrospective multicentric study was made in the Thyroid Department of the Endocrine and Metabolism Argentine Society (SAEM). 219 patients with histological proven MTC were included, with a mean age of 39+20ys (range between 1-84 years). 65% were women and 44% were familiar cases. In 58% the initial manifestation was a thyroid nodule. Genetic screening of relatives of patients with MTC was the initial presentation in 22% of the cases. Only in 39% of patients diagnosis of MTC was made by fine needle aspiration, but cytology led to surgery in 79%. Cytology, calcitonin (CT) levels or genetic studies were able to diagnose MTC in 47% of patients prior to initial surgery. 65% of patients had advanced stages of the disease (III or IV) at diagnosis. Proto-oncogene RET was studied in 162 patients (74%). In 49% a mutation was described, most frequently in codon 634 (76%). Regarding hereditary forms of MTC, MEN 2A was the most frequent (57%), followed by familiar MTC in 25% and MEN 2B in 13% of cases. Familiar cases were younger and had more frequently a pre-surgery diagnosis. Index cases were older, with higher CEA and CT levels, presented in more advanced stages and had evidence of disease at final assessment than patients who were diagnosed by genetic screening. Follow-up records of 143 patients were analyzed (65%), median time was 44 months; 21 patients died (14.6) and 122 were alive (86%), 76 showed no evidence of disease (NED) (54%). High risk factors for evidence of disease in final evaluation were male sex, sporadic MTC, higher CT pre-surgery levels, stage IV and metastasis. Post surgery CT levels were lower in patients with NED. Stage at initial diagnosis was the main prognostic factor of patients with MTC, determining the importance of early detection in order to perform curative surgery in less advanced stages.