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

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.