Soft tissue

Fibroblastic / myofibroblastic



Last author update: 1 June 2013
Last staff update: 26 April 2023

Copyright: 2002-2023,, Inc.

PubMed Search: Lipofibromatosis [title]

Ali Chaudri, M.D.
Jerad M. Gardner, M.D.
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Cite this page: Chaudhri A. Lipofibromatosis. website. Accessed November 29th, 2023.
Definition / general
  • Pediatric tumor with predilection for hands and feet, composed predominantly of mature adipose tissue with proliferative fibroblasts in adipose septa (Stanford University)
  • Previously called infantile fibromatosis of non-desmoid type
  • First described in 2000 using "lipofibromatosis" (Am J Surg Pathol 2000;24:1491)
  • Rare childhood tumor (first surgery usually at age 1), 2/3 male, often of distal extremities
Clinical features
  • Associated with macrodactyly of foot (Foot Ankle 1991;12:40)
  • Clinically resembles lymphatic malformation or lymphedema
  • Recurs locally due to lack of securing clean margins at resection, no metastases
Case reports
Gross description
  • White-tan or yellow with obvious fatty component, 1 - 3 cm
Microscopic (histologic) description
  • Lobules of mature adipose tissue with fibroblastic foci consisting of bland fibroblasts involving adipose septa with a preserved lobular architecture
  • Often have minute small univacuolated cells at interface between fibroblasts and adipose
  • No atypia, no/rare mitotic figures
  • Rarely pigmented cells are associated with fibroblastic element; resemble those in Bednar tumor, pigmented neurofibroma, nevi
Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.
Positive stains
Negative stains
Molecular / cytogenetics description
Differential diagnosis
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