Gardner fibroma (GAF) is a benign soft tissue lesion with a predilection for childhood and adolescence and an association with familial adenomatous polyposis (FAP) and desmoid type fibromatosis (desmoid).
Gardner fibroma shows morphological overlap with nuchal-type fibroma (albeit lacking the neural component). It shows a striking association with familial adenomatous polyposis (FAP) with APC gene mutations.
GAF has a predilection for childhood and early adulthood, a strong association with FAP/APC, an association with concurrent or subsequent development of desmoids, and overexpression of beta-catenin and other proteins in the APC and Wnt pathways. The proportion of sporadic GAFs that have APC mutation remains to be determined.
Synopsis
associations
- known FAP or adenomatous polyposis coli (APC) (69%)
- no history of familial polyps or soft tissue tumors (22%)
- individual or family history of soft tissue masses and/or desmoids (13%)
age: 2 months to 36 years
sites:
- back and paraspinal region 61%
- head and neck 14%
- extremities 14%
- chest and abdomen 11%
bland hypocellular proliferation of haphazardly arranged coarse collagen fibers with a bland hypocellular proliferation of inconspicuous spindle cells, small blood vessels
sparse mast cell infiltrate
Immunohistochemistry
nuclear reactivity for beta-catenin (64%)
nuclear reactivity for both cyclin-D1 and C-myc (100%)
References
Coffin CM, Hornick JL, Zhou H, Fletcher CD. Gardner fibroma: a clinicopathologic and immunohistochemical analysis of 45 patients with 57 fibromas. Am J Surg Pathol. 2007 Mar;31(3):410-6. PMID: 17325483
Wehrli BM, Weiss SW, Yandow S, Coffin CM. Gardner-associated fibromas (GAF) in young patients: a distinct fibrous lesion that identifies unsuspected Gardner syndrome and risk for fibromatosis. Am J Surg Pathol. 2001 May;25(5):645-51. PMID: 11342777
Michal M. Non-nuchal-type fibroma associated with Gardner’s syndrome. A hitherto-unreported mesenchymal tumor different from fibromatosis and nuchal-type fibroma. Pathol Res Pract. 2000;196(12):857-60. PMID: 11156330
Dawes LC, La Hei ER, Tobias V, Kern I, Stening W. Nuchal fibroma should be recognized as a new extracolonic manifestation of Gardner-variant familial adenomatous polyposis. Aust N Z J Surg. 2000 Nov;70(11):824-6. PMID: 11147449
Diwan AH, Graves ED, King JA, Horenstein MG. Nuchal-type fibroma in two related patients with Gardner’s syndrome. Am J Surg Pathol. 2000 Nov;24(11):1563-7. PMID: 11075861