Value of bone scanning and computed tomography in fibrodysplasia ossificans progressiva

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Abstract
Fibrodysplasia ossificans progressiva (FOP) (synonym: myositis ossificans progressiva) is an extremely rare congenital disorder characterized by unremitting heterotopic ossification associated with typical skeletal abnormalities (Rogers & Geho, 1979; Connor & Evans, 1982; Thickman et al, 1982). The prevalence in the UK population has been estimated to be less than 0.1 per million (Wynne-Davies et al, 1985) but despite this, or possibly because of it, there have been in excess of 700 publications worldwide on the subject (Illingworth, 1971) since the first description in 1692 (Patin, 1692). We report a case of FOP presenting as a suspected soft-tissue neoplasm and discuss the value of bone scanning and computed tomography (CT) in the diagnosis and evaluation of this rare condition. An 11-year-old boy first presented to his local Accident and Emergency department when his father noticed a large mass over the child's right scapula. The swelling was not tender and there was no history of trauma. Plain radiographs of the shoulder showed no abnormality and a clinical diagnosis of a haematoma was made. The swelling decreased in size over the next month but a further hot, tense mass developed over the left shoulder. Biopsy of both masses was reported as showing muscle infiltrated with slender spindle cells in a myxoid stroma, and a pathological diagnosis of fibromatosis was made with the recommendation that total excision should be performed because of the risks of local recurrence.