Home   Chapter Home   Jobs   Conferences   Fellowships   Books



Advertisement

Soft tissue Tumors

Fibroblastic / myofibroblastic tumors

Gardner type fibroma


Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 20 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
=========================================================================

● Benign soft tissue lesion with thick, haphazard collagen and bland fibroblasts that entrap adjacent tissue
● 90% associated with FAP / Gardnerís syndrome / APC germline mutation
● Uncommon
● Affects infants, children and teenagers
● May be initial diagnostic clue to Gardnerís syndrome and APC mutations (Am J Surg Pathol 2001;25:645)
● 45% develop desmoid-type fibromatosis
● Similar histology to nuchal-type fibroma (Pathol Int 2004;54:523, Am J Surg Pathol 2000;24:1563)

Sites:
● Superficial or deep soft tissue
● Various sites

Case reports
=========================================================================

● Post-surgical desmoid tumor in Gardnerís syndrome patient (J Neurooncol 2009;91:107)

Gross description
=========================================================================

● 1-10 cm, poorly circumscribed, firm, rubbery, plaque-like, white to tan-pink cut surface with trapped fat in yellow areas

Micro description
=========================================================================

● Thick, haphazardly arranged collagen bundles, hypocellular bland fibroblasts, small blood vessels, plaque-like growth pattern with infiltration of adjacent structures

Micro images
=========================================================================



Gardner's Fibroma / Nuchal fibroma


Gardner associated fibromas and severe polyposis in 4 year old with FAP

Positive stains
=========================================================================

● CD34, cyclin D1, vimentin, nuclear beta-catenin (64%)

Negative stains
=========================================================================

● Muscle specific actin, smooth muscle actin, desmin, ER, PR

Additional references
=========================================================================

Am J Surg Pathol 2007;31:410, Pathologe 2010;31:97, Stanford University

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic tumors > Gardner type fibroma


This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).