Adrenocortical tumors in children

Childhood adrenocortical tumors (ACT) are rare. In the USA, only about 25 n ew cases occur each year. In Southern Brazil, however, approximately 10 tim es that many cases are diagnosed each year. Most cases occur in the contigu ous states of Sao Paulo and Parana. The cause of this higher rate has not b een identified. Familial genetic predisposition to cancer (p53 mutations) a nd selected genetic syndromes (Beckwith-Wiedemann syndrome) have been assoc iated with childhood ACT in general but not with the Brazilian counterpart. Most of the affected children are young girls with classic endocrine syn d romes (virilizing and/or Cushing). Levels of urinary 17-ketosteroids and pl asma dehydroepiandrosterone sulfate (DHEA-S), which are abnormal in approxi mately 90% of the cases, provide the pivotal clue to a diagnosis of ACT. Ty pical imaging findings of pediatric ACT consist of a large, well-defined su prarenal tumor containing calcifications with a thin capsule and central ne crosis or hemorrhage. The pathologic classification of pediatric ACT is tro ublesome. Even an experienced pathologist can find it difficult to differen tiate carcinoma from adenoma, Surgery is the single most important procedur e in the successful treatment of ACT. The role of chemotherapy in the manag ement of childhood ACT has not been established although occasional tumors are responsive to mitotane or cisplatin-containing regimens. Because of the heterogeneity and rarity of the disease, prognostic factors have been diff icult to establish in pediatric ACT. Patients with incomplete tumor resecti on or with metastatic disease at diagnosis have a dismal prognosis. In pati ents with localized and completely resected tumors, the size of the tumor h as predictive value. Patients with large tumors have a much higher relapse rate than those with small tumors.