Multifocal tenosynovial giant cell tumors in a child with Noonan syndrome

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 sy...

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Bibliographic Details
Published in:Pediatric radiology 2017-03, Vol.47 (3), p.361-365
Main Authors: Meyers, Arthur B., Awomolo, Agboola O., Szabo, Sara
Format: Article
Language:English
Subjects:
Biopsy
Case Report
Child, Preschool
Diagnosis, Differential
Giant Cell Tumor of Tendon Sheath - complications
Giant Cell Tumor of Tendon Sheath - diagnostic imaging
Giant Cell Tumor of Tendon Sheath - surgery
Giant Cell Tumors - complications
Giant Cell Tumors - diagnostic imaging
Giant Cell Tumors - surgery
Humans
Imaging
Magnetic Resonance Imaging
Male
Mandibular Neoplasms - complications
Mandibular Neoplasms - diagnostic imaging
Mandibular Neoplasms - surgery
Medicine
Medicine & Public Health
Neuroradiology
Noonan Syndrome - complications
Nuclear Medicine
Oncology
Pediatrics
Radiology
Synovitis, Pigmented Villonodular - complications
Synovitis, Pigmented Villonodular - diagnostic imaging
Synovitis, Pigmented Villonodular - surgery
Tomography, X-Ray Computed
Ultrasound
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Description
Summary: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.
ISSN:0301-0449
1432-1998
DOI:10.1007/s00247-016-3743-3