Follow-up and risk of tumors in overgrowth syndromes.
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.
Gracia Bouthelier
Ricardo
R
Department of Pediatric Endocrinology, Hospital Universitario La Paz, Autonoma University, Madrid, Spain.
Lapunzina
Pablo
P
D000008:Abdominal Neoplasms / Q000209:etiology*; D000015:Abnormalities, Multiple / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006130:Growth Disorders / Q000150:complications*; D006801:Humans; D007223:Infant; D008297:Male; D013577:Syndrome