FaCD Online Syndrome Fact Sheet

Last updated: 24 Jun 2008

Name: Multiple Spindle Cell Lipomas, Familial

Mode of Inheritance: AD?

Tumor features

spindle cell lipoma

Tumor features (possible)

dermatofibrosarcoma protuberans

Comment

The presence of multiple lipomas of the spindle cell type is rare. Fanburg-Smith et al.[1] reported on 4 families with this disorder. Males were more often, and more severely, affected than females. Some patients developed over 200 lipomas. Most of the lesions appeared on the posterior neck or back, shoulders, upper torso, axillae and upper arms. This distribution is similar to that in familial multiple symmetric lipomatosis. Average age at diagnosis was 58 years (range 39-71). One of the families featured 4 successive affected generations, with 12 affected family members (7 male, 5 female). Male-to-male transmission was observed. Possibly this a variant of familial multiple symmetric lipomatosis given the similarity in distribution.
Harvell reported a patient with multiple spindle cell lipomas and dermatofibrosarcoma protuberans[2].

References

[1] Fanburg-Smith JC, Devaney KO, Miettinen M, Weiss SW. Multiple spindle cell lipomas: A report of 7 familial and 11 nonfamilial cases. Am J Surg Pathol 22[1], 40-48. 1998.
[2] Harvell JD. Multiple spindle cell lipomas and dermatofibrosarcoma protuberans within a single patient: evidence for a common neoplastic process of interstitial dendritic cells?. Journal of the American Academy of Dermatology 2003; 48(1):82-5.