Mandible / maxilla
Benign tumors / tumor-like conditions
Fibrous dysplasia

Author: Nat Pernick, M.D. (see Authors page)

Revised: 19 June 2018, last major update January 2004

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Fibrous dysplasia mandible maxilla

Cite this page: Pernick, N. Benign tumors / tumor-like conditions: fibrous dysplasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/mandiblemaxillafibrousdysplasia.html. Accessed August 16th, 2018.
Definition / general
  • Called craniofacial form of fibrous dysplasia if confined to jaw
  • Monostotic, polyostotic or associated with McCune-Albright syndrome
  • May be congenital or hereditary (but differs from cherubism)
  • Starts in childhood, usually diagnosed by age 20 years
McCune-Albright syndrome
  • Due to somatic mutation of c-fos oncogene that causes activation of cAMP pathway
  • Polyostotic fibrous dysplasia, cafe au lait skin pigmentation (large, dark lesions with serpiginous ["coast of Maine"] borders in chest, neck, back), almost exclusively in women
  • Also endocrine abnormalities (precocious puberty, hyperthyroidism, pituitary adenomas that secrete growth hormone primary adrenal hyperplasia)
Symptoms
  • Unilateral painless swelling of mandible or maxilla in men / women ages 25 - 35 years
  • No treatment since growth is self limited and responsive to pubertal hormonal changes
  • Surgical recontouring performed if facial deformity, although may regrow in 25%
Radiology description
  • Ill defined margins
  • Diffusely radiopaque with ground glass image
Microscopic (histologic) description
  • Uniformly distributed C shaped or Chinese figure-like trabeculae of woven / immature bone within proliferating fibroblastic and vascularized stroma
  • Usually no osteoblastic rimming and no / rare osteoclasts
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Kelly Magliocca, DDS, MPH

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