Long-Term Outcome in 215 Children and Adolescents with Papillary Thyroid Cancer Treated During 1940 Through 2008

Controversy exists regarding the aggressiveness of initial therapy in childhood papillary thyroid cancer (PTC). Few studies with long-term outcome exist and second primary malignancies have rarely been analyzed. We studied 215 PTC patients younger than 21 years old managed during 1940 through 2008. The patients were aged 3-20 year old (median age = 16 years); the median follow-up was 29 years. Recurrence and mortality details were taken from a computerized database. Median primary tumor size was 2.2 cm. Six percent had distant metastases at presentation, 5% had incomplete tumor resection, 86% had nodes removed at initial surgery, and 78% had nodal metastases. After complete surgical resection, PTC recurred in 32% by 40 years. At 20 years, the recurrence rates at local, regional, and distant sites were 7, 21, and 5%, respectively. During 1940-1969, local and regional recurrence rates after unilateral lobectomy (UL) were significantly (P < 0.001) higher than after bilateral lobar resection (BLR). During 1950-2008 radioiodine remnant ablation (RRA) was administered within 18 months to 32%; it did not diminish the 25-year regional recurrence rate of 16% seen after BLR alone (P = 0.86). Only two fatal events from PTC occurred at 28 and 30 years, for a cause-specific mortality at 40 years of only 2%. All-causes mortality rates did not exceed expectation through 20 years, but from 30 through 50 years, the number of deaths was significantly (P < 0.001) higher than predicted. Fifteen of 22 deaths (68%) resulted from nonthyroid malignancy. Survival from childhood PTC should be expected, but later death from nonthyroid malignancy is disconcerting. Seventy-three percent of those who died from nonthyroid malignancy had received postoperative therapeutic irradiation.