title: Clinical characteristics of small functioning adrenocortical tumors in children.
abstract: Twenty of 67 children registered on the International Registry of Childhood Adrenocortical Tumors between May 1988 and December 1994 had small adrenocortical tumors (defined for this study as measuring < or = 200 cm3 and/or weighing < or = 100 g). We reviewed the records of these 20 patients to characterize the clinical and pathologic findings and outcomes of children with small adrenocortical tumors. Median patient age was 2 years (range, 4 months to 5 years). There was only one boy. All had clinical signs of virilization, and seven had signs or symptoms of Cushing syndrome. A median 5.5 months (range, 1-40 months) had elapsed between the first signs of endocrine dysfunction and diagnosis. All tumors were surgically resected. Tumor volume was 3.3-195 cm3 (median, -8.7 cm3), and weight was 3.7-100 g (median, 36 gm Tumor samples were histologically reviewed in 18 cases. Eight were adenomas, and 10 were carcinomas (6 low grade and 4 high grade). Pathology records described tumor with diagnostic features of adrenocortical carcinoma in two patients. One patient received mitotane for 8 months after surgery. Only one patient had recurrent disease, which was detected 6 months after diagnosis and proved rapidly fatal. Another has been lost to follow-up. The remaining 18 patients are alive with no evidence of disease at a median 2.3 years (range, 6 months to 6.1 years) after diagnosis. Our data suggest that children with small adrenocortical tumors have an excellent prognosis with surgery as the sole therapy, regardless of tumor histiotype.
authors.affiliation: Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA.
authors.forename: E L
authors.initials: EL
authors.lastname: Michalkiewicz
authors.forename: R
authors.initials: R
authors.lastname: Sandrini
authors.forename: M F
authors.initials: MF
authors.lastname: Bugg
authors.forename: L
authors.initials: L
authors.lastname: Cristofani
authors.forename: E
authors.initials: E
authors.lastname: Caran
authors.forename: A M
authors.initials: AM
authors.lastname: Cardoso
authors.forename: L
authors.initials: L
authors.lastname: de Lacerda
authors.forename: R C
authors.initials: RC
authors.lastname: Ribeiro
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D018246:Adrenocortical Adenoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D018268:Adrenocortical Carcinoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies