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The Journal of Bone and Joint Surgery, Vol 75, Issue 2 220-230, Copyright © 1993 by Journal of Bone and Joint Surgery, Inc
The histopathology of fibrodysplasia ossificans progressiva. An endochondral process
FS Kaplan, JA Tabas, FH Gannon, G Finkel, GV Hahn and MA Zasloff
Department of Orthopaedic Surgery, University of Pennsylvania School of Medicine, Philadelphia.
In order to better characterize the biological features of fibrodysplasia
ossificans progressiva, we reviewed the histopathological specimens from
eleven patients (twelve biopsies) who had a confirmed diagnosis of the
disease. All of the biopsies had been performed in children, to exclude the
diagnosis of a malignant lesion. In no instance was the diagnosis of
fibrodysplasia ossificans progressiva considered before the biopsy. The
results of a lesional biopsy in all eleven patients revealed normal
endochondral osteogenesis at heterotopic sites. The results of biopsy of an
early lesion in six children were misinterpreted as revealing a diagnosis
of fibromatosis or sarcoma before the roentgenographic appearance of
ossification. Immunohistochemical studies of sections of the earliest
lesion demonstrated S-100 antigen positivity before the histological
appearance of differentiated osteochondral tissue. The presence of
congenital malformation of the great toes and of postnatal heterotopic
endochondral osteogenesis strongly suggests that fibrodysplasia ossificans
progressiva is a disorder of defective induction of endochondral
osteogenesis. This study established the predominant histopathological
findings associated with fibrodysplasia ossificans progressiva and can
serve as a basis for postulation of a candidate gene in the pathogenesis of
the disorder. A lesional biopsy is not needed to make the diagnosis; biopsy
uniformly exacerbates the condition and should be avoided.
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