Pigmented villonodular synovitis of the temporomandibular joint with intracranial extension: A case series and systematic review.
BACKGROUND
Pigmented villonodular synovitis (PVNS) is a rare proliferative disorder of the synovial membrane. PVNS generally affects large joints but occasionally involves the temporomandibular joint (TMJ), with occasional extension into the middle cranial fossa. The purpose of this study was to report our experience with PVNS along with a focused literature review.


METHODS
Patients with PVNS of the TMJ treated at the University of California - San Francisco from 2007 to 2013 were reviewed. A PubMed search was performed to identify additional cases.


RESULTS
Five patients underwent surgical resection, with 1 recurrence at 61 months. A literature review identified 58 patients, 19 of which had intracranial involvement. Interestingly, intracranial extension was more common in men. Intracranial extension was not associated with an increased rate of recurrence.


CONCLUSIONS
PVNS of the TMJ is a rare entity associated with excellent outcomes, even with intracranial extension. Management should consist of maximal resection, with radiotherapy reserved for extensive or recurrent lesions.
Department of Neurological Surgery, University of California - San Francisco, San Francisco, California.
Michael
M
Safaee
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, Illinois.
Taemin
T
Oh
Department of Neurological Surgery, University of California - San Francisco, San Francisco, California.
Matthew Z
MZ
Sun
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, Illinois.
Andrew T
AT
Parsa
Department of Neurological Surgery, University of California - San Francisco, San Francisco, California.
Michael W
MW
McDermott
Department of Otolaryngology - Head and Neck Surgery, University of California - San Francisco, San Francisco, California.
Ivan H
IH
El-Sayed
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, Illinois.
Orin
O
Bloch
D000328:Adult; D035301:Cranial Fossa, Middle / Q000473:pathology* / Q000601:surgery*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D035583:Rare Diseases; D012008:Recurrence; D012189:Retrospective Studies; D013586:Synovitis, Pigmented Villonodular / Q000175:diagnosis* / Q000601:surgery*; D013705:Temporomandibular Joint Disorders / Q000175:diagnosis* / Q000601:surgery*; D016896:Treatment Outcome
intracranial extension; outcomes; pigmented villonodular synovitis; recurrence; temporomandibular joint