Cáncer tiroideo papilar. Reporte de 4 casos familiares

Abstract

Introduction: Papillary thyroid cancer (PTC) is the most common endocrine malignancy, representing 80% of all thyroid cancers; only 10% of cases are manifested before age 21 and have an estimated incidence of 0.54 cases per 100,000 people. In children it is diagnosed at a more advanced stage of the disease but with good therapeutic response and very low mortality. Objective: To present four family cases with PTC, discuss the particular characteristics and the importance of early diagnosis. Case reports: 4 families with members affected by family papillary thyroid carcinoma are presented, all pediatric cases were manifested after a direct member adult case was diagnosed, therefore pediatric patients were early detected, but despite a timely treatment, the disease was advanced at the time of diagnosis. The pediatric cases are 3 females and 1 male with an average age of 12 1/2 years old at diagnosis. Discussion: The family variety of papillary thyroid carcinoma (2 or more direct members affected), represents 5% of papillary cancers. It is transmitted through autosomal dominant inheritance with incomplete penetrance and variable expressivity. It occurs at a younger age than the sporadic type, and it is more aggressive with greater local invasion (32%), recurrence (20-50%) and lymphatic metastases (57 %), and it is associated with benign thyroid diseases and often, it is multifocal. Conclusion: The family papillary thyroid cancer is a disease with worse prognosis than the sporadic variety; therefore, a high index of suspicion is required in affected families for early diagnosis and treatment.