Pediatric lipoblastoma in the head and neck: a systematic review of 48 reported cases

Background: Lipoblastoma is an exceedingly rare cause of pediatric head and neck masses. There have been 47 cases previously reported in the English literature. We present an additional case and review of the available literature on this rare neoplasm.

Objective: To review and assess the current published literature regarding the efficacy of preserving neurovascular structures in the surgical management of pediatric lipoblastoma.

Methods: Literature analysis of case reports was performed. MEDLINE was searched for the terms "neonatal lipoblastoma", "lipoblastomatosis", and "benign lipoblastoma". Results in the English literature were mined for relevant clinical data when available. The citations of case reviews found were searched to find additional cases.

Results: Including our new case, a total of 48 cases of head and neck lipoblastoma have been reported in the English literature within 23 manuscripts. Four manuscripts presented cases series (Evidence Based Medicine Level 4) and 19 were case reports (Level 5). The median sample size was 1 (range 1-4). For those 14 articles (N=23 cases) reporting follow-up, the median follow-up duration was 22 months. Male to female ratio was 2.1:1 with an average age at presentation of 2.1 years (range: newborn to 12 years). Lesions ranged from 3 to 12 cm in longest diameter. Recurrence was seen in 27% of patients in which there was at least 1-year follow-up. The most common presenting symptoms were painless enlarging neck mass (53%, 17/32) and respiratory distress (12%, 4/32). An exact binomial sign test indicated that most authors recommend conservative complete excision with preservation of vital structures with 10 of 11 authors giving a stance supporting conservative surgical resection, p=.012.

Conclusions: Our findings suggest that although total excision is ideal and curative, subtotal resection may be a viable treatment alternative for lipoblastoma of the head and neck. This tumor presents a clinical challenge and should be considered in infants presenting with a cervical mass. It is difficult to differentiate from the much more common lymphangioma on clinical and radiological examination. Additionally, the potential for rapid growth and adhesion to neurovascular tissue makes surgical resection arduous. Nonetheless, recurrence rates for head and neck lipoblastomas are similar to those rates observed elsewhere in the body.