Pigmented Villonodular Synovitis

Diffuse outgrowth arising from the SYNOVIAL MEMBRANE; SYNOVIAL BURSA; or TENDON sheath around the joint cavity, with extension to surrounding soft tissue. It is characterized by pigmented HEMOSIDERIN-containing MACROPHAGES; FOAM CELLS; and multinucleated GIANT CELLS. It usually occurs in the hands and feet, and around large joints, such as in the ankle and knee joints.

Also Known As:

Networked: 275 relevant articles (17 outcomes, 24 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Giant Cell Tumors (Giant Cell Tumor)
2.	Neoplasms (Cancer)
3.	Joint Diseases (Joint Disease)
4.	Giant Cell Tumor of Tendon Sheath
5.	Hyperplasia

Experts

1.	Zhang, Xin: 3 articles (03/2022 - 01/2020)
2.	Blay, Jean-Yves: 3 articles (01/2018 - 03/2012)
3.	Italiano, Antoine: 3 articles (01/2018 - 03/2012)
4.	Stacchiotti, Silvia: 3 articles (01/2018 - 03/2012)
5.	Ferguson, Peter C: 3 articles (01/2016 - 04/2008)
6.	Wunder, Jay S: 3 articles (01/2016 - 04/2008)
7.	Heyd, Reinhard: 3 articles (07/2015 - 04/2006)
8.	He, Jin: 3 articles (03/2015 - 02/2011)
9.	Calabrese, F: 3 articles (05/2011 - 10/2006)
10.	Fiocco, U: 3 articles (05/2011 - 10/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pigmented Villonodular Synovitis:

1.	pexidartinibIBA 01/01/2022 - "Complementary Effects of Surgery and Pexidartinib in the Management of Patients with Complex Diffuse-Tenosynovial Giant Cell Tumor." 11/16/2022 - "Use of neoadjuvant pexidartinib with limb salvage surgery for diffuse tenosynovial giant cell tumor: A case report." 01/01/2023 - "The double-blind, randomized, placebo-controlled phase 3 study of orally administered PLX3397 in patients with pigmented villonodular synovitis or giant cell tumor of the tendon sheath (ENLIVEN) showed that pexidartinib provides a robust objective tumor response in adults with tenosynovial giant cell tumors (TGCT) not amenable to improvement with surgery. " 01/01/2020 - "The drug was approved based on the ENLIVEN study, which looked at pexidartinib (brand name, Turalio™), a medication taken by mouth (orally) for people with TGCT (also known as giant cell tumor of the tendon sheath [GCTTS] and pigmented villonodular synovitis [PVNS]) who are not able to have surgery because of the location and/or the size of the tumor. "
2.	nilotinibFDA Link 01/01/2018 - "Randomised trials investigating the efficacy of nilotinib for patients with unresectable pigmented villonodular synovitis are warranted. " 01/01/2018 - "Nilotinib in locally advanced pigmented villonodular synovitis: a multicentre, open-label, single-arm, phase 2 trial." 01/01/2018 - "More than 90% of patients with locally advanced unresectable progressive pigmented villonodular synovitis achieved disease control with 12 weeks of nilotinib treatment. " 01/01/2018 - "Between Dec 15, 2010, and Sept 28, 2012, we enrolled 56 patients with pigmented villonodular synovitis and treated them with nilotinib. " 01/01/2018 - "Nilotinib in locally advanced pigmented villonodular synovitis: challenges of a new targeted therapy."
3.	HemosiderinIBA 11/01/2022 - "The diagnosis of pigmented villonodular synovitis (PVNS) was suspected intraoperatively by the presence of hemosiderin into the tendon sheath and distal articulation. " 07/01/2020 - "Pigmented villonodular synovitis (PVNS) is a rare synovial proliferative disease featuring hemosiderin deposits. " 10/04/2016 - "Pigmented villonodular synovitis (PVNS) is a rare, benign, but potentially locally aggressive and recurrent condition characterized by synovial proliferation and hemosiderin deposition inside the joints, tendon sheaths, and bursae. " 12/01/2015 - "Pigmented villonodular synovitis is a disorder resulting in a villous, nodular, or villonodular proliferation of the synovium, with pigmentation related to the presence of hemosiderin. " 07/01/2015 - "Pigmented villonodular synovitis (PVNS) is a proliferative condition of the synovium, which is composed of nodules and/or villi and has an abundant number of hemosiderin-laden macrophages. "
4.	YttriumIBA 08/01/2008 - "Pigmented villonodular synovitis managed by Yttrium 90 after debulking surgery." 02/01/2008 - "Our initial and highly satisfactory experience with the injection of radioactive yttrium 90 to treat pigmented villonodular synovitis of the ankle joint eroded with time so much so that we discontinued its use in the ankle and believe that it is important to alert our colleagues to the complications that we observed. " 10/01/2002 - "The use of surgery and yttrium 90 in the management of extensive and diffuse pigmented villonodular synovitis of large joints." 11/01/1998 - "Synoviorthesis with Yttrium 90 seems to be a good adjuvant for the treatment of recurrent pigmented villonodular synovitis. " 06/01/1992 - "Treatment of pigmented villonodular synovitis with yttrium-90: changes in immunologic features, Tc-99m uptake measurements, and MR imaging of one case."
5.	Imatinib Mesylate (Gleevec)FDA Link 01/01/2016 - "Imatinib mesylate induces mitochondria-dependent apoptosis and inhibits invasion of human pigmented villonodular synovitis fibroblast-like synovial cells." 04/01/2011 - "Pigmented villonodular synovitis responsive to imatinib therapy." 03/15/2012 - "Efficacy of imatinib mesylate for the treatment of locally advanced and/or metastatic tenosynovial giant cell tumor/pigmented villonodular synovitis." 04/01/2008 - "Complete response to imatinib in relapsing pigmented villonodular synovitis/tenosynovial giant cell tumor (PVNS/TGCT)." 06/01/2010 - "The identification of these abnormal ways of road marking to licence the development of effective targeted therapeutic agents against certain rare histological connective subcategories of sarcomas and tumors with local aggressiveness, in particular DFSP, PVNS, GCST, PEComes, endometrial stromal sarcomas, Ewing sarcomas, etc. Imatinib is used in the treatment of DFSP, characterized by a translocation of the gene PDGF, or in pigmented villonodular synovitis (PVNS), a tumor of soft part also locally aggressive, caused by an abnormality of the gene coding for the M-CSF. "
6.	Capsules (Microcapsules)IBA 01/01/2010 - "Pigmented villonodular synovitis was confirmed during the operation when incising a yellowish nodular mass protruding from the capsule. " 06/01/1995 - "A case report is presented in which a patient with well-documented pigmented villonodular synovitis sustained a disruption of the popliteal artery without evidence of penetration of the posterior capsule. " 11/01/2022 - "We introduce a technique for arthroscopic excision of a posterior intra-articular hip mass consistent with pigmented villonodular synovitis, also known as tenosynovial giant cell tumor, using a modified T-capsulotomy based on the lateral aspect of the hip capsule. " 11/01/1998 - "Relatively homogeneous iso- or marginal hyperintensity on T2-weighted sequences, lack of susceptibility effects due to hemosiderin, the demonstration of a fibrous capsule and the absence of adjacent joint involvement may help to differentiate localized and diffuse forms of giant-cell tumors of tendon sheaths and pigmented villonodular synovitis."
7.	Radioisotopes (Radionuclides)IBA 06/01/1975 - "This radionuclide scanning technique is therefore helpful in distinguishing pigmented villonodular synovitis from other arthropathies." 01/01/2021 - "Is intra-articular infliximab therapy a good alternative to radionuclide synovectomy for a patient with refractory pigmented villonodular synovitis?"
8.	Monoclonal AntibodiesIBA 01/01/2016 - "Small molecule and monoclonal antibody targeted therapies are being investigated as novel treatment methods for pigmented villonodular synovitis, with promising results." 12/01/2004 - "Using a monoclonal antibody with a standard immunohistochemical technique on paraffin sections, we evaluated expression of Mitf in 89 various giant cell lesions including giant cell tumor of bone (n26), giant cell tumor of tendon sheath/pigmented villonodular synovitis (n24), giant cell reparative granuloma (n3), aneurysmal bone cysts (n11), chondroblastomas (n7), foreign body giant cell reaction (n10), and sarcoidosis (n8). "
9.	SteroidsIBA 09/07/2011 - "Although her systemic lupus erythematosus went into remission with another course of steroids and higher doses of mycophenolate mofetil, the pigmented villonodular synovitis persisted and she had to undergo open synovectomy to control her symptoms. "
10.	Mycophenolic Acid (Cellcept)FDA LinkGeneric 09/07/2011 - "Although her systemic lupus erythematosus went into remission with another course of steroids and higher doses of mycophenolate mofetil, the pigmented villonodular synovitis persisted and she had to undergo open synovectomy to control her symptoms. "

Therapies and Procedures

1.	Synovectomy 11/01/2000 - "A combination of surgical synovectomy with radiosynovectomy is highly efficacious in treating clinical symptoms of pigmented villonodular synovitis. " 10/01/2022 - "Open or Arthroscopic Synovectomy Is the Preferred Management Option in Pigmented Villonodular Synovitis of the Hip Joint Without Evidence of Degeneration: A Systematic Review of 20 Studies." 11/01/2021 - "This study compares recurrence rates and functional outcomes of patients who underwent anterior arthroscopic synovectomy versus combined anterior arthroscopic/posterior open synovectomy for diffuse pigmented villonodular synovitis (DPVNS). " 05/01/2021 - "The purpose of this study is to evaluate the outcome and prognosis of complete synovectomy performed under arthroscopic surgery in the treatment of diffuse pigmented villonodular synovitis (DPVNS) of the knee. " 05/01/2019 - "The purpose of this study was to evaluate and describe the clinical results of complete arthroscopic synovectomy through the four arthroscopic portals in the knees affected by diffuse pigmented villonodular synovitis (DPVNS). "
2.	Therapeutics 01/01/2021 - "In pigmented villonodular synovitis, radiosynovectomy as an adjuvant therapy for relapse might not be effective when performed more than 6 months after surgery. " 10/01/2021 - "The Role of Medical Therapy in Refractory Pigmented Villonodular Synovitis." 04/01/2018 - "Long-term outcome of pigmented villonodular synovitis of the hip after joint preserving therapy." 03/01/2015 - "Surgical outcomes after excision of pigmented villonodular synovitis localized to the ankle and hindfoot without adjuvant therapy." 09/01/2012 - "After surgical therapy of diffuse pigmented villonodular synovitis (DPVNS), recurrence is seen in almost half of the patients. "
3.	Knee Replacement Arthroplasty (Total Knee Replacement) 09/01/2023 - "In this case report, total knee arthroplasty was performed in a patient with pigmented villonodular synovitis. " 02/25/2021 - "To determine the functional outcomes, complications and revision rates following total knee arthroplasty (TKA) in patients with pigmented villonodular synovitis (PVNS). " 02/25/2021 - "Systematic review: total knee arthroplasty (TKA) in patients with pigmented villonodular synovitis (PVNS)." 09/07/2020 - "Pigmented villonodular synovitis does not influence the outcomes following cruciate-retaining total knee arthroplasty: a case-control study with minimum 5-year follow-up." 01/01/2019 - "Complications After Total Knee Arthroplasty in Patients With Pigmented Villonodular Synovitis."
4.	Arthroplasty 07/01/1999 - "In our series of 1136 patients with 1388 arthroplasties, these costs could have been eliminated for all but the twelve patients who had a suspected malignant lesion and the one patient in whom pigmented villonodular synovitis was found. " 01/01/2018 - "Long-Term Outcome of Hip Arthroplasty in the Setting of Pigmented Villonodular Synovitis." 01/01/2014 - "Unusual cases of pigmented villonodular synovitis after arthroplasty." 02/01/2013 - "MRI diagnosis of recurrent pigmented villonodular synovitis following total joint arthroplasty." 11/01/2010 - "To explore the method and outcome of knee resurfacing arthroplasty in treating late-staged diffuse pigmented villonodular synovitis (PVNS). "
5.	Radiotherapy 09/01/2010 - "The German Cooperative Group on Radiotherapy in Benign Diseases (GCG-BD) conducted a pattern-of-care study (PCS) to analyze the radiation therapy (RT) practice for pigmented villonodular synovitis (PVNS). " 09/01/2010 - "Radiation therapy for treatment of pigmented villonodular synovitis: results of a national patterns of care study." 03/02/2022 - "Though radiotherapy has been widely used for knee pigmented villonodular synovitis (PVNS), there is few literatures about radiotherapy for the treatment of PVNS hip. " 08/01/2019 - "Pigmented villonodular synovitis of the temporomandibular joint: case report and the literature review for postoperative radiotherapy." 09/19/2018 - "To explore and evaluate the predictive value of preoperative Neutrophil-lymphocyte ratio (NLR) on the recurrence of pigmented villonodular synovitis (PVNS) of the knee joint treated by arthroscopic surgery combining local radiotherapy. "