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Pediatric Radiology

, Volume 47, Issue 3, pp 361–365 | Cite as

Multifocal tenosynovial giant cell tumors in a child with Noonan syndrome

  • Arthur B. MeyersEmail author
  • Agboola O. Awomolo
  • Sara Szabo
Case Report

Abstract

Noonan syndrome is a genetic disorder with variable expression of distinctive facial features, webbed neck, chest deformity, short stature, cryptorchidism and congenital heart disease. The association of Noonan syndrome and giant cell granulomas of the mandible is widely reported. However, Noonan syndrome may also be associated with single or multifocal tenosynovial giant cell tumors, also referred to as pigmented villonodular synovitis. We report a child with Noonan syndrome, giant cell granulomas of the mandible and synovial and tenosynovial giant cell tumors involving multiple joints and tendon sheaths who was initially misdiagnosed with juvenile idiopathic arthritis. It is important for radiologists to be aware of the association of Noonan syndrome and multifocal giant cell lesions, which can range from the more commonly described giant cell granulomas of the mandible to isolated or multifocal intra- or extra-articular tenosynovial giant cell tumors or a combination of all of these lesions.

Keywords

Child Computed tomography Giant cell granuloma Magnetic resonance imaging Noonan syndrome Pigmented villonodular synovitis Tenosynovial giant cell tumor 

Notes

Compliance with ethical standards

Conflicts of interest

None

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Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Arthur B. Meyers
    • 1
    • 2
    Email author
  • Agboola O. Awomolo
    • 1
  • Sara Szabo
    • 3
    • 4
  1. 1.Department of RadiologyChildren’s Hospital of WisconsinMilwaukeeUSA
  2. 2.Department of RadiologyNemours Children’s Health System/Nemours Children’s HospitalOrlandoUSA
  3. 3.Department of PathologyMedical College of Wisconsin & Children’s Hospital of WisconsinMilwaukeeUSA
  4. 4.Division of Pathology and Laboratory MedicineCincinnati Children’s Hospital Medical CenterCincinnatiUSA

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