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Radiation therapy in treatment of fibrodysplasia ossificans progressiva: a case report and review of the literature
Authors:Soldić Zeljko  Murgić Jure  Radić Jasna  Dabelić Nina  Jazvić Marijana  Brozić Jasmina Marić  Kusić Zvonko
Institution:University of Zagreb, "Sestre milosrdnice" University Hospital Center, Department of Oncology and Nuclear Medicine, Zagreb, Croatia.
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.
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