We describe 11 cases (8 females, 3 males) of papillary thyroid carcinoma in children treated at St. Jude Children's Research Hospital over a 33-year period, and review the literature. Ages ranged from 7-25 years (median, 16 years). Six patients had primary papillary thyroid carcinoma. Five patients had secondary papillary thyroid carcinoma after treatment of Hodgkin's disease (n = 2), acute lymphoblastic leukemia (n = 2), and neuroblastoma (n = 1) with chemotherapy and cervical radiation. The typical presentation was either cervical lymphadenopathy or a thyroid mass of short duration. Treatment consisted of thyroidectomy, cervical lymph node dissection, and postoperative thyroid hormone replacement (n = 1), parathyroid reimplantation (n = 1), 131I ablation (n = 4), external-beam irradiation (n = 1), and chemotherapy with doxorubicin (n = 1) or carboplatin and topotecan (n = 1). Nine patients are alive without evidence of disease 3.0-22.4 years from diagnosis. One patient has persistent but stable disease 17.3 years after diagnosis. One patient relapsed with metastatic lung disease 0.3 years after the initial diagnosis. He continues to do well after a brief but unsustained complete radiographic remission of disease to combination chemotherapy with carboplatin and topotecan. Our review supports excellent long-term outcome for primary or secondary papillary thyroid carcinoma in pediatric patients although complications may require close follow-up in a multidisciplinary setting.

This study was undertaken to explore the therapeutic and diagnostic value of splenectomy for children with acute myelocytic leukemia (AML) in remission. Of 29 consecutive untreated patients entering the study, 19 attained complete remission with mercaptopurine, vincristine, and 6-azauridine. Chemotherapy during remission consisted of mercaptopurine daily and cytosine arabinoside weekly. Because remission lasted 2 months or less in 5 patients, they did not undergo splenectomy. Fourteen patients underwent splenectomy while clinically in complete remission. Splenectomy was tolerated well with no major complications. Gross abnormalities of the abdominal organs were found in 11 patients. Histologic leukemia was found in 12 patients, most often in the spleen. Negro children did not respond to therapy as well as Caucasian children. Central nervous system (CNS) leukemia terminated clinical remission in 7 of the 14 patients. Four patients remain in initial CNS and hematologic remission for 18 to 24 months, and four others remain in initial hematologic remission for 16 to 18 months. These results suggest that in children with AML, splenectomy may influence the activity of leukemia and prolong the duration of remission.