Bone - Fibrodysplasia ossificans progressiva

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Bone

Developmental abnormalities

Fibrodysplasia ossificans progressiva

Reviewers: Dariusz Borys, M.D. (see Reviewers page)
Revised: 1 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare autosomal dominant disorder with congenital malformation of the great toes and progressive heterotopic ossification in defined anatomic patterns
● Early preosseous lesions resemble aggressive juvenile fibromatosis

Pathophysiology:
● Spontaneous and post-traumatic flareups heralded by intense connective tissue edema with perivascular lymphocytic infiltration into skeletal muscle
● Angiogenic fibroproliferative lesions that spread along muscle planes and evolve through endochondral ossification to form mature lamellar bone
● Leads to immobilization of joints making movement impossible, later death due to starvation (ankylosis of jaw) or from restrictive disease of chest wall
● May be mediated by mast cells (Hum Pathol 2001;32:842)

Treatment
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● None; surgical trauma induces further bone formation

Case reports
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● 7 year old girl with 13 cm thoracic tumor (with images)

Gross images
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FOP Skeleton

Additional references
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● Hum Pathol 1997;28:339

End of Bone > Developmental abnormalities > Fibrodysplasia ossificans progressiva

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