Pigmented villonodular synovitis (PVNS) is a benign proliferative disorder of unknown cause that affects synovial joints, bursae, and tendon sheaths. It is characterized by inflammation and overgrowth of the joint lining. There are two primary forms - a diffuse form affecting the entire synovial lining and a rare localized form occurring in small joints. While the cause is unknown, it is thought to be neoplastic in nature. Treatment involves complete surgical resection or radiation therapy to prevent recurrence, as incomplete removal can allow regrowth. Radiation therapy provides effective local control with minimal side effects.

1. Pigmented villonodular synovitis
(PVNS)
Dr. Ahmed Al Ibraheemi

2. Pigmented villonodular synovitis (PVNS)
• 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

3. • 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.

4. • 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.

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

10. 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.

11. Histology
• PVNS lesions on histology demonstrate
synovial cell proliferation,
xanthomatous cell accumulation,
hemosiderin deposition, and the
presence of multinucleated giant cells

13. 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.

14. 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.

15. 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.

16. 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.

17. 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.

19. 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.

21. 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.

22. 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.

23. 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.

24. 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.

25. Radiotherapy
• Local control was achieved
95.1%, and 82.9% had no or only
slight functional impairment. The
early and late toxicity was mild

26. Thank you