Pigmented villonodular synovitis of the knee confused with juvenile rheumatoid arthritis in a 3-year-old child: A case report

Abstract

Pigmented villonodular synovitis (PVNS) is rare and characterized by diffuse synovial cell proliferation with the development of villi, or round/ovoid lobulated nodules of varied sizes. It usually presents in adults between the ages of 30 and 40 years and is uncommon in the pediatric population. We herein report a 3-year-old female patient with PVNS causing a femoral subchondral lesion in the right knee. Her symptoms started at the age of one and she had gotten treatment for juvenile rheumatoid arthritis for 2 years. The patient was operated, and the tumor was excised. Arthrotomy was performed for diffuse PVNS and diagnosis was made histopathologically. She was followed-up for 18 months with clinical examinations and MRI, as necessary. She had no pain or limitation in range of motion. She is currently fully functional and ambulatory.

Keywords

• PVNS
• Pediatric
• Knee
• Juvenile Rheumatoid Arthritis

3 yaşındaki bir çocukta juvenil romatoid artrit olarak karışan dizde pigmente villonodüler sinovit: Olgu sunumu

Öz

Pigmente villonodüler sinovit (PVNS), farklı boyutlardaki yuvarlak veya oval loblu nodüllerin oluşturduğu sinovyal hücrelerdeki diffüz proliferasyona bağlı nadir görülen bir patolojidir. Genellikle 30-40 yaş arasındaki erişkinlerde görülür ve pediatrik popülasyonda nadirdir. Bu çalışmada, sağ diz femoral kondillerin subkondrol lezyonuna neden olan PVNS tanılı 3 yaşında bir kız sunuldu. Bir yaşında semptomları başlayan hasta 2 yıl boyunca juvenil romatoid artrit tedavisi aldı. Şikayetleri geçmemesi üzerine hasta opere edildi. Kitle, tanı ve tedavi için eksize edildi. Diffüz PVNS olan hasta için artrotomi yapıldı ve histopatolojik olarak incelemeyle tanısı kesinleştirildi. Klinik ve MRG ile 18 ay takip edildi. 18. ayındaki kontrollerinde hastanın sağ dizinde hareket kısıtlılığı veya ağrı mevcut değildi. Tüm semptomları geçen hasta tam olarak fonksiyonel olarak mobilize olmaktadır.

Anahtar Kelimeler

• PVNS
• Pediatri
• Diz
• Juvenil romatoid artrit

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