Lipoblastoma: pathophysiology and surgical management

Abstract

Background/purpose: Lipoblastoma/lipoblastomatosis is a poorly understood and uncommon soft tissue tumor of infancy and early childhood. Twenty-four patients that presented to a single institution over a 15-year period were reviewed for clinical features and outcome.

Methods: A retrospective review was conducted.

Results: Twenty-five cases were identified; 1 chart was not available for review. 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 diffuse. 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 further surgery after initial resection. Patients with diffuse lipoblastoma (lipoblastomatosis) are likely to have recurrent disease (usually within 2 years) and should undergo close follow-up. Genetic analysis of the specimen will help exclude liposarcoma. Recurrent lesions are best imaged with magnetic resonance imaging to assess extent and plan reconstruction if necessary.