Lipoblastoma: Pathophysiology and surgical management

• Published in: Journal of pediatric surgery Vol. 36; no. 1; pp. 229 - 231
• Main Authors: Dilley, Anthony V., Patel, Dimple L., John Hicks, M., Brandt, Mary L.
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Elsevier Inc 01.01.2001; Elsevier
• Subjects: adipose tumor; benign; Biological and medical sciences; Child; Child, Preschool; Dermatology; Female; Humans; Infant; Lipoblastoma; lipoblastomatosis; Lipoma - pathology; Lipoma - surgery; Lipomatosis - pathology; Lipomatosis - surgery; Male; Medical sciences; Retrospective Studies; Soft Tissue Neoplasms - pathology; Soft Tissue Neoplasms - surgery; soft tissue tumors; Treatment Outcome; Tumors of the skin and soft tissue. Premalignant lesions; adipose tumor; benign; Lipoblastoma; lipoblastomatosis; soft tissue tumors; Human; Indication; Pathogenesis; Surgery; Surgical resection; Adipose tissue disorders; Benign neoplasm; Lipoma; Localization; Child
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1053/jpsu.2001.20060

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. A retrospective review was conducted. 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. 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.