Clinical characteristics of small functioning adrenocortical tumors in children

Clinical characteristics of small functioning adrenocortical tumors in children

Author Michalkiewicz, E. L. Google Scholar
Sandrini, R. Google Scholar
Bugg, M. F. Google Scholar
Cristofani, L. Google Scholar
Caran, E. Google Scholar
Cardoso, AMS Google Scholar
deLacerda, L. Google Scholar
Ribeiro, R. C. Google Scholar
Institution ST JUDE CHILDRENS RES HOSP
UNIV FED PARANA
Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
UNIV TENNESSEE
COLL MED
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 less than or equal to 200 cm(3) and/or weighing less than or equal to 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 cm(3) (median, 38.7 cm(3)), and weight was 3.7-100 g (median, 36 g). 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. (C) 1997 Wiley-Liss, Inc.
Keywords childhood adrenocortical carcinoma
prognostic factors
treatment
Language English
Date 1997-03-01
Published in Medical and Pediatric Oncology. Hoboken: Wiley-liss, v. 28, n. 3, p. 175-178, 1997.
ISSN 0098-1532 (Sherpa/Romeo, impact factor)
Publisher Wiley-Blackwell
Extent 175-178
Origin http://dx.doi.org/10.1002/(SICI)1096-911X(199703)28:3<175
Access rights Closed access
Type Article
Web of Science ID WOS:A1997WF15600003
URI http://repositorio.unifesp.br/handle/11600/25697

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