Lipoblastoma: Pathophysiology and surgical management

Background/Purpose: Lipoblastoma/lipoblastomatosis is a poorly understood a nd uncommon soft tissue tumor of infancy and early childhood. Twenty-four p atients that presented to a single institution over a 15-year period were r eviewed for clinical features and outcome. Methods: A retrospective review was conducted. Results: Twenty-five cases were identified; 1 chart was not available for r eview. Fourteen girls and 11 boys with a median age of 20 months (2 months, 10 years) presented with 26 separate tumors. Nineteen were focal and 7 dif fuse. The tumors were located on the trunk (n = 13), leg (n = 6), arm (n = 3), and head and neck (n = 4). Five patients (all with diffuse type) had up to 3 recurrences. Conclusions: Patients with focal lipoblastoma are unlikely to require furth er surgery after initial resection. Patients with diffuse lipoblastoma (lip oblastomatosis) are likely to have recurrent disease (usually within 2 year s) and should undergo close follow-up. Genetic analysis of the specimen wil l help exclude liposarcoma. Recurrent lesions are best imaged with magnetic resonance imaging to assess extent and plan reconstruction if necessary. J Pediatr Surg 36:229-231. Copyright (C) 2001 by W.B. Saunders Company.