PVNS and Tenosynovial Giant Cell Tumor

Chapter 3: PVNS and Tenosynovial Giant Cell Tumor

These very rare connective tissue tumors, including pigmented villonodular synovitis (PVNS), arise most commonly near the knee joint, but they can also affect large and small joints alike. They are initially removable with surgery, but some have a high risk of recurrence.

PVNS and Tenosynovial Giant Cell Tumor

Alternative diagnostic terms
Histiocytic Sarcoma

Common anatomical locations
Joints and tendon sheaths.

Pathology comment
These tumors often arise from the synovium joints and adjacent tendon sheath soft tissues and demonstrate a wide spectrum of activity and aggressiveness, ranging from benign aggressive to malignant pathology and clinical behavior. Recent systemic therapies have proven useful and efficacious; see reference below.

Source
Van De Sande M, Tap WD, Gelhorn HL, Ye X, Speck RM, Palmerini E, Stacchiotti S, Desai J, Wagner AJ, Alcindor T, Ganjoo K, Martin-Broto J, Wang Q, Shuster D, Gelderblom H, Healey JH. Acta Orthop. 2021 Aug; 92(4):493-499. doi: 10.1080/17453674.2021.1922161. Epub 2021 May 12. PMID: 33977825

Tap WD, Gelderblom H, Palmerini E, Desai J, Bauer S, Blay JY, Alcindor T, Ganjoo K, Martin-Broto J, Ryan CW, Thomas DM, Peterfy C, Healey JH, van de Sande M, Gelhorn HL, Shuster DE, Wang Q, Yver A, Hsu HH, Lin PS, Tong-Starksen S, Stacchiotti S, Wagner AJ; ENLIVEN investigators. Lancet. 2019 Aug 10; 394(10197):478-487. doi: 10.1016/S0140-6736(19)30764-0. Epub 2019 Jun 19. PMID:  31229240

For diagnosis or treatment
Memorial Sloan Kettering Cancer Center or any center listed in the Sarcoma Centers Directory »

Patient advocacy
Any organization with an established program for patient triage, support, and education. See Patient Advocacy Directory »

Clinical trials
See past Memorial Sloan Kettering Cancer Center Trial – Pexidartinib (Turalio™) is now standard therapy.

SARC Clinical Trials – None
Find clinical trials worldwide »

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