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J Pediatr Endocrinol Metab. 2005 Dec;18 Suppl 1:1227-35.

Follow-up and risk of tumors in overgrowth syndromes.

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Department of Pediatric Endocrinology, Hospital Universitario La Paz, Autonoma University, Madrid, Spain.


A striking feature of the overgrowth syndromes (OGS) is the risk of cancer. In some OGS (Beckwith-Wiedemann, Perlman, Simpson-Golabi-Behmel syndromes and hemihypertrophy) tumors appeared mostly in the abdomen (more than 94% of tumors), are usually diagnosed before 10 years, and most of them are embryonal. Conversely, in other OGS, such as Sotos syndrome, the most frequent type is lympho-hematological tumors, about two-thirds are extra-abdominal and some of these tumors may appear after the second decade of life. Based on a previous literature review, a specific schedule protocol for tumor screening was suggested for many OGS. In this article we briefly review some aspects of the current knowledge of OGS and tumors, emphasizing the follow-up of these disorders.

[Indexed for MEDLINE]

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