2. • 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)
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. Types:
Monoarticular involvement (most common), occurs in two forms: localized and diff
Two variants as described by Granowitz –
Localized form (LPVNS): focal involvement of the synovium
– Nodular / Sessile or Pedunculated masses.
– Hands & feet
b. Diffuse form (DPVNS) (more common): affects virtually the entire synovium, eg.
– Intra-articular PVNS tends to be of the diffuse form.
– Tendon sheath PVNS (Giant cell tumour of tendon sheath[GCCTS]), the nodular fo
5. • The diffuse form typically involves the
large joints , while the localized form
typically occurs around the small joints
of the hands and feet
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.
12. Etiopathogenesis:
• Repetitive trauma (50%) causing recurrent local hemorrhage to affecte
• Proliferation of the synovium of joints, tendon sheaths or bursae.
• It is a reactive condition, and not a true neoplasm.
• PVNS classically presents as a monoarticular disease, mimicking arthrit
• Recurrent atraumatic haemarthrosis is a characteristic feature.
14. Histology
• PVNS lesions on histology demonstrate
synovial cell proliferation,
xanthomatous cell accumulation,
hemosiderin deposition, and the
presence of multinucleated giant cells
15.
16. 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.
17. • 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.
18. • 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.
19. 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.
20. 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.
21.
22. Investigation:
Aspiration of joint: characteristically reveals a blood tinged brownish-stained aspirate.
X-ray:
• Soft tissue swelling will be marked due to haemorrhage and lobulated synovial tissue.
• May reveal cysts or erosions in the joint mimicking gout.
• Bony erosions are usually from without, especially in the hip
• Periarticular erosions, with a thin rim of reactive bone
• Late feature of joint space narrowing indicates articular cartilage loss, is difficult to distinguish
MRI:
• Ideal investigation
• Nodular mass (periarticular or synovial) with bone erosion
23. Sonography:
• Loculated joint effusions, Complex heterogeneous echogenic masses and markedly
Arthroscopy:
• Direct visualisation of synovium
• Has both diagnostic and therapeutic value in resection of tumours
Histolopathology:
• LPVNS is pedunculated, lobular lesion localized to one area of the synovium.
• On microscopy, Histiocytes, lipid laden macrophages, hemosiderin containing cells a
25. 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.
26. Treatment:
• Synovectomy:
o Total synovectomy (open or arthroscopic):
– Open (anterior approach midline incision or medial parapatellar arthrotomy
– Arthroscopic synovectomy, has gained popularity, has several advantages ov
• Radiotherapy (3500- 4000 cGy) (Radiation induced synovectomy/ intra-articu
27.
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29.
30. Prognosis:
• LPVNS: excellent prognosis, low recurrence rate if managed surgically,
• DPVNS: surgical excision difficult, recurrence rate of up to 46%.
