Abstract
Purpose of Study: To present the clinical features, investigations, histopathology, differential diagnosis and treatment options for lipoblastoma, based on a series of six encountered in our paediatric orthopaedic practice.
Method: The records of six children with lipoblastoma who attended Alder Hey Hospital between 2000 and 2006 were reviewed. Mean age was 17 months and mean follow up was 26 months.
Results: The youngest was a six month old infant with a swelling on his right instep. The second patient, a three year old girl, presented with a limp and swelling in her foot. The third patient was an 18 month old boy with a swelling on the dorsum of his left forearm. The fourth patient had a swelling of his left thigh and two patients had swellings in their backs. Each was investigated by MRI (1), CT (1) or US (4) and surgical excision planned accordingly. There were no post operative complications. None has shown recurrence during follow-up.
Conclusions: All patients were originally thought to have simple lipomata or soft tissue swellings. This is primarily because lipoblastoma is a rare tumour, yet lipoblastoma is the most likely diagnosis of a fatty lump in a child aged less than two. Differential diagnoses include myxoid liposarcoma, well-differentiated liposarcoma, spindle cell lipoma, typical lipoma and soft tissue sarcoma.
Lipoblastomata need thorough imaging. Cytogenetic evaluation of tumour cells often reveals chromosomal anomalies, such as abnormalities of the long arm of chromosome 8 leading to rearrangement of the PLAG1 gene. Biopsy of the lesion is recommended for accurate diagnosis, as clinical and radiological diagnoses can be misleading.
Lipoblastomata tend to spread locally and may recur after incomplete resection; metastatic potential has not been reported. Complete surgical resection is mandatory to prevent recurrence.
Correspondence should be addressed to: Mr J. B. Hunter, BSCOS, c/o BOA, The Royal College of Surgeons, 35–43 Lincoln’s Inn Fields, London WC2A 3PE.
