Soft Tissue
Fibroblastic / myofibroblastic tumors
Lipofibromatosis

Author: Ali Chaudhri, M.D. (see Authors page)
Editor: Jerad M. Gardner, M.D.

Revised: 26 August 2016, last major update June 2013

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Lipofibromatosis [title]

Cite this page: Lipofibromatosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuelipofibromatosis.html. Accessed December 4th, 2016.
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)
Epidemiology
  • 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
Micro 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
Micro Images

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.

Positive Stains
Negative Stains
Molecular / Cytogenetics Description
Differential Diagnosis