pigmented villonodular snynovitis

Pigmented villonodular synovitis (PVNS)

Dr. Ahmed Al Ibraheemi

• Is a benign proliferative disorder of uncertain etiology that affects synovial lined joints, bursae, and tendon sheaths .• characterized by inflammation

and overgrowth of the joint lining

Pigmented villonodular synovitis (PVNS)

• It results in various degrees of villous and/or nodular changes in the affected structures.• PVNS lesions are monoarticular or

solitary. Polyarticular disease is uncommon but more likely in children.

• It can also occur in the shoulder, ankle, elbow, hand or foot.• Two primary forms are described,

including a diffuse form that affects the entire synovial lining of a joint, bursa, or tendon sheath, and a rare focal, or localized, form.

• The diffuse form typically involves the large joints , while the localized form typically occurs around the small joints of the hands and fee

Etiology

• The etiology of PVNS remains uncertain.• Neoplasia is the presently accepted

underlying etiology. • Evidence supporting this theory is both

empirical and genetic. PVNS has demonstrated the capability of autonomous growth and rare malignant transformation.

Histology

• PVNS lesions on histology demonstrate

synovial cell proliferation, xanthomatous cell accumulation, hemosiderin deposition, and the presence of multinucleated giant cells

Epidemiology• PVNS is an uncommon disease.• The prevalence is approximately 9.2

cases of extra-articular and 1.8 cases of intra-articular disease per 1 million population.

• Localized lesions are more common than diffuse involvement, comprising 77% of total lesions in one review, with a 3.3:1 localized-to-diffuse predominance ratio.

Epidemiology

• Diffuse PVNS affects predominantly large joints, with the knee being the most common (66-80%).• The hip, ankle, shoulder, and elbow

follow in descending frequency.

Epidemiology • Diffuse PVNS has nearly equal

incidence in male and female patients, while the localized form demonstrates a female-to-male predominance ratio of 1.5-2:1.• Diagnosis is more common between

ages 20 and 50 years, with a median age of 30 years.

Clinical presentation

• In general, pigmented villonodular synovitis often manifests initially as sudden onset, unexplained joint swelling and pain; the joint swelling is disproportionate to the amount of pain the patient feels at first. Decreased motion and increased pain occur as the disorder progresses as well as locking of the joint.

Clinical presentation

• The localized form often manifests initially as a painless, slow-growing mass and progresses to the other common symptoms of PVNS. The swelling often feels warm to the touch.

Treatment and prognosis

• Although a benign condition, PVNS may result in significant morbidity if left untreated. Pain, loss of function, and eventual joint destruction may result. The primary treatment options include surgical resection via synovectomy or radiation therapy.

Treatment and prognosis

• Recurrence is reduced with complete resection, and it is achieved more often with localized disease.

• Radiation therapy may be used as the primary treatment method or in concert with surgical excision.

• PVNS is overwhelmingly benign, with only a few cases of malignant transformation reported.

Radiotherapy • The literature review demonstrate

that RT is a very safe and effective treatment option for the prevention of disease progression or recurrence in PVNS after primary surgical interventions.

Radiotherapy • The planned treatment volume should

include the whole synovial space and eventually all invasive components of the disease. Currently, total doses in the range of 30-36 Gy are recommended

• In Other article the total doses ranged from 30 to 50 Gy (median, 36 Gy), the median single dose was 2.0 Gy.

Radiotherapy • Other articles shown that Radiation

therapy is a safe and effective treatment for PVNS in the postoperative setting after incomplete resection, and also as a salvage option for treatment of recurrences it provides a high rate of local control.

Radiotherapy • Local control was achieved

95.1%, and 82.9% had no or only slight functional impairment. The early and late toxicity was mild

Thank you