Complete tumor resection is the single most important prognostic indicator of pediatric adrenocortical tumors (ACT) (Schteingart, Doherty et al. 2005). Patients who have residual disease after surgery have a dismal prognosis. Of 57 patients in the IPACTR who had distant or local residual (gross or microscopic) disease after surgery, only 8 have remained free of disease. In children with complete tumor resection, tumor weight is independently associated with outcome. IPACTR data showed that among 192 children with ACT, those with tumors weighing more than 200 grams had an event-free survival (EFS) rate of 39% compared with 87% for those with smaller tumors. Tumor size has been consistently associated with prognosis in several studies of ACT (Michalkiewicz, Sandrini et al. 1997; Wieneke, Thompson et al. 2003; Michalkiewicz, Sandrini et al. 2004. Children whose tumors produce excess glucocorticoid appear to have a worse prognosis than those who have pure virilizing manifestations.
It is likely that prognostic factor analysis can be further refined by adding other predictive factors. For example, rupture of the tumor pseudocapsule during surgery and invasion of the vena cava were found to be associated with poor prognosis even among patients whose tumors were completely resected, but these variables are yet to be prospectively analyzed.
Based on a previous IPACTR series (Michalkiewicz, Sandrini et al. 2004), of the 254 patients with known outcomes, the 5-year EFS and overall survival estimates were 54.2% (95% CI, 48.2–60.2%) and 54.7% (95% CI, 48.7–60.7%), respectively. Five patients died of causes unrelated to tumor progression (2 died of infection, 1 of hypertensive complication, 1 of massive hemorrhage during surgery, and 1 of an unspecified complication).
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