A rare multilocular cystic disease of mandible: cherubism

Year 2014, Volume: 22 Issue: 3, 124 - 126, 01.12.2014

Nazım Gümüş Sarper Yılmaz

Abstract

Cherubism is a rare skeletal dysplasia located to the maxilla and mandible which is characterized by bilateral, symmetric, fibro-osseous and multilocular cystic lesions. In most of the cases, dominant mutations are detected in the SH3BP2 gene on chromosome 4p. While affected children seem to be clinically normal at birth, whose jaws start swelling and enlargement between 2 and 7 years of age. Full round cheek and upward tilting of the eyes consist of typical appearance of children. Apart from various cystic lesions of mandible, cherubism is a tumor which regresses spontaneously, ossifies over time and has a likelihood for leaving unrecognizable remnants after years, although radiologically, extensive bone involvement appears. Presented here was a patient who suffered from this rare disease and his clinical findings.

Keywords

cherubism, mandible, multilocular bone cyst

Mandibulanın nadir multiloküle kistik hastalığı: Şerubizm

Abstract

Çerubizm maksilla ve mandibulaya yerleşim gösteren iki yanlı, simetrik, fibroosseöz, multiloküler kistik lezyonlarla karakterize nadir bir iskelet displazisidir. Çoğu olguda 4p kromozomu dominant SH3BP2 gen mutasyonu saptanır. Etkilenen çocuklar doğum sonrasında normal görünüme sahipken, 2 ile 7 yaşlar arasında çenelerde şişlik ve genişleme gelişmeye başlar. Dolgun yuvarlak yanak ve gözlerin yukarı kayması çocukların tipik görüntüsünü oluşturur. Mandibulanın birçok kistik lezyonundan farklı olarak, radyolojik olarak yaygın kemik tutulumu gösterdiği halde kendiliğinden gerileyen, zaman içinde kemikleşen ve yıllar sonra tanınmaz izler bırakma ihtimaline sahip bir tümördür. Burada bu nadir görülen hastalığa sahip olgu ve klinik bulguları sunuldu.

Keywords

Çerubizm, mandibula, multiloküler kemik kisti

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