Familial nonmedullary thyroid cancer. An emerging entity that warrants aggressive treatment

Abstract

Objective: To determine whether familial nonmedullary thyroid carcinoma behaves like sporadic carcinoma of follicular cell origin.

Design: Retrospective review.

Setting: University medical center.

Patients: Fourteen patients were treated for familial nonmedullary thyroid carcinoma between 1980 and 1994. Thirteen families were identified, with 30 affected individuals.

Interventions: Patients were treated with total or completion total thyroidectomy. Thirteen additional operations were performed to control recurrent disease.

Main outcome measures: Stage, recurrence, and survival. Patients were followed up for a mean of 6.5 years.

Results: In our 14 patients, 13 tumors were multifocal, and six of these were bilateral. The incidences of lymph node metastasis and local invasion were both 57% (n = 8). Seven patients (50%) had recurrences during follow-up.

Conclusions: Familial nonmedullary thyroid carcinoma has a high incidence of multifocality and invasion and a high rate of local recurrence. Aggressive initial treatment and careful follow-up seem to be indicated.