Preliminary communication
Radiation Therapy in Treatment of Fibrodysplasia Ossificans Progressiva: A Case Report and Review of the Literature
Željko Soldić
Jure Murgić
Jasna Radić
Nina Dabelić
Marijana Jazvić
Jasmina Marić Brozić
Zvonko Kusić
Abstract
Fibrodysplasia ossificans progressiva (FOP) is an extremely rare genetic disorder with diffuse extra-skeletal bone formation.
The genetic mutation responsible for FOP has recently been discovered and is connected with excessive activation
of bone morphogenetic protein receptor. This disease usually begins with typical ossification pattern in early childhood,
causing increasing disability and making patients totally disabled by the age of 30. Ectopic ossification develops
spontaneously and can be triggered by any trauma and even intramuscular injections. The symptoms of FOP are often
misdiagnosed as cancer, causing unnecessary biopsies, which can precipitate further progressive heterotopic ossification.
There is no effective treatment for this severe condition. Radiotherapy can be helpful in impeding ossification, although
the strict evidence for that is lacking. There are only two reports in the literature referring to the use of radiotherapy in
treatment of FOP. Herein, we present a 35-year-old patient successfully treated with small doses of fractionated radiotherapy
in several courses. This case indicates that radiotherapy can be useful in treating patients with FOP
Keywords
fibrodysplasia ossificans progressiva; heterotopic ossification; radiotherapy
Hrčak ID:
69161
URI
Publication date:
24.6.2011.
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