Contemporary management of lipoblastoma

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• 作者：Allison L. Speer ; Deborah E. Schofield ; Kasper S. Wang ; Cathy E. Shin ; James E. Stein ; Donald B. Shaul ; G. Hossein Mahour ; Henri R. Ford
• 关键词：Lipoblastoma ; Lipoblastomatosis ; Lipoma ; Myxoid liposarcoma
• 刊名：Journal of Pediatric Surgery
• 出版年：2008
• 出版时间：July 2008
• 年：2008
• 卷：43
• 期：7
• 页码：1295-1300
• 全文大小：117 K

文摘

<h4 class=""h4"">Purposeh3>Lipoblastoma is a rare, benign, adipose tissue tumor. We report the largest single institution experience managing these uncommon neoplasms.<h4 class=""h4"">Methodsh3>

We retrospectively reviewed 32 cases of lipoblastoma entered in the pathology database at our institution between January 1991 and August 2005. We conducted a comprehensive literature review of lipoblastoma and summarized the results of the largest series published.<h4 class=""h4"">Resultsh3>

Most patients presented with an enlarging, palpable, firm, nontender, mobile mass. The male-to-female ratio was 1.9:1. The anatomical distribution was trunk (n = 12), extremity (n = 12), groin (n = 5), and neck (n = 3). Average age at resection was 2.8 years (range, 2.6 months to 12 years). Thirty-one cases were completely excised, although 1 patient underwent staged partial excision to preserve nerve function. Chromosomal analysis performed in selected patients revealed characteristic aberrations in chromosome 8. Complications included keloid formation (n = 3), wound infection/dehiscence (n = 2), wound seroma (n = 1), and transient brachial plexus neurapraxia (n = 1). Average follow-up was 7.4 months (range, 1 day to 6.5 years); 2 patients were lost to follow-up. There were no recurrences.<h4 class=""h4"">Conclusionsh3>

A staged approach with meticulous sparing of the neurovascular bundle provides excellent functional outcome for patients with large tumors. Nonmutilating surgical excision is the treatment of choice.