The neoplastic cells are S100 protein positive (approximately 80%), and show membrane and vacuole CD31 immunoreactivity.
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Interestingly, MEN1 gene (11q13.1) is most frequently deleted, while GARP gene (11q13.5) may also be involved.
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In general, imaging studies show a well-defined, heterogeneous mass, usually showing a mass which is hypointense to subcutaneous fat on magnetic resonance T1-weight images.
Other entities which are accompanied by multiple lipomas include Proteus syndrome, Cowden syndrome and related disorders due to PTEN gene mutations, benign symmetric lipomatosis (Madelung disease), familial lipodystrophy, hibernomas, and familial angiolipomatosis.