pigmented villonodular synovitis

1. PIGMENTED VILLONODULAR
SYNOVITIS
DR. KUMAR SUPRASHANT
DNB RESIDENT
HINDU RAO HOSPITAL AND NDMC MEDICAL
COLLEGE

2. OVERVIEW
Origin & classification
Clinical feature
Pathology
Radiology
Differential diagnosis
Diagnosis
Treatment

3. • Most common disorder of synovium
• Controversy of origin been reactive(itis) or
neoplastic(oma)
• Reactive- evidence of trauma , multifocal
origin
• Neoplastic-autonomous growth, apoptosis
resistance
• Mitochondrial dysfunction is the primary
factor in pathogenesis

4. • Proliferation of both type A & type B cells
(predominantly type A)
• Monocyte lineage
(suggested by)
• Enzymatic- acid phosphatase,
β glucuronidase, α naphthyl acetate esterase
• Immunohistochemical- CD68, HAM56,
MAC386, PG-M1

5. Classification
• LOCALIZED- identical histologically to GCT of
tendon sheath
• DIFFUSE- histologically similar to localized
form but involve the entire synovium

6. LOCALIZED PVNS
• Age- 30 to 50yrs
• Female preponderance
• Predominantly in hand –adjacent IP joint
• Foot, ankle, wrist less commonly involved

7. Clinical feature
• Long gradual progression
• Non tender, firm, irregular, nodule of varying
size fixed to underlying structures, free from
skin (except in distal phalanx)
PVNS near PIP
of index finger

8. pathology
• Gross- 0.5 -4 cm lobulated mass
• Cut section- pink gray background with
yellow brown areas
• Microscopy- polyhedral cells surrounded by
collagen, giant cells ,xanthoma cells, mitotic
figures, hemosiderin deposition

9. Clinical image
Gross picture showing brown &
yellow nodular mass

10. Microscopic picture with hemosiderin deposition

11. Radiology
• Plain radiograph
–Circumscribed soft tissue mass
–Cortical erosion in some cases
• MRI not useful for localized form due to
variable hemorrage

12. Radiograph
with cortical
erosion Clinical image Intraoperative
image

13. Intraoperative image showing yellowish brown
thickened nodular synovium

14. D/D
• Foreign body granuloma
• Fibroma of tendon sheath
• Necrobiotic granuloma
• Tendon xanthoma
• Fibrosis of tendon sheath

15. DIFFUSE PVNS
• Younger age
• Female preponderance
• Can involve joints & extra-articular locations
• MC joint is knee
• Hip, ankle, foot, shoulder, wrist, sacroiliac
joint, posterior elements of spine

16. Clinical features
• Long h/o pain in joint
• Swelling disproportionate to degree of pain
• Joint effusion
• Hemarthrosis
• Locking
• Limitation of movement

17. Pathology
• Gross-absence of collagenous capsule
• Microscopy- polyhedral cells with deep blue
cytoplasm(hemosiderin), polymorphic
appearance due to spindle cell, xanthoma
cell, giant cell.
Coarse villi- shag carpet appearance
Fine villi- fernlike villi
• Aspiration-serosanguineous or blood tinged
fluid

18. Radiology
Plain radiograph
Soft tissue- intraarticular effusion, lobulated
mass
Bony
• Envade subchondral bone – cyst formation
• Marginal & pressure erosion
Concentric erosion of femoral neck- apple
core deformity
Marginal erosion- bubbly appearance
• Joint narrowing

19. CT
• Lesion show high attenuation due to
hemosiderin
• Useful in delineating bone cyst formation and
erosion

20. MRI- most informative
• Nodular thickened synovial masses
throughout joint cavity, multiple areas of
hypointensity on T1 & variable intensity on
T2 ( due to presence of hemosiderin)
• Blooming artifact from hemosiderin(seen on
gradient echo sequence)- nearly
pathognomic of PVNS at MRI
• Reveals lesion not visible directly by
arthoscopy such as posterior to cruciate
ligament or within popliteal cyst

21. Normal
radiograph
MRI-Joint effusion & synovial thickening
in anterior portion of joint
19 yr girl with c/o Pain &
swelling of right knee

22. Extensive erosion
of distal femur
with sclerotic
margins & normal
joint space
MRI- tissue
replacing
entire knee
joint
MRI- cortical
erosion of lower
femur and upper
tibia
53 yr woman with recurrent hemarthrosis & swelling right knee

23. Anterior soft
tissue mass at
ankle
MRI- mass presents with intermediate
signals without bone erosion
19 yr girl with ankle pain & swelling

24. D/D
Chronic hemarthrosis & hemosiderin deposition
• Hemophilic arthopathy- classical history and
invsn
• Synovial hemangioma- common in children
and young
Bone erosion, subchonral cyst, joint narrowing
• Osteoarthritis- characteristic osteophytes
• RA- polyarticular(PVNS monoarticular)
• Tuberculous arthritis- periarticular
osteoporosis & abscess

25. Work up & diagnosis of PVNS
• Clinical feature
• Laboratory invs- non specific
• Radiography- normal/ soft tissue mass/
erosion
• MRI- most informative
• Joint aspiration
• Biopsy
• Arthroscopy
• Synovectomy

26. Treatment
• Localized form- marginal excision
• Diffuse form- Total synovectomy
oOpen synovectomy – complicated by
development of painful adhesions &
stiffness
oArthroscopic synovectomy- t/t of choice
contraindicated for inaccessible lesions
oArthroplasty considered if significant
secondary degenerative changes of joint
surfaces

27. Newer advances
• Intraarticular radiation synovectomy
yttrium-90, dysprosium-165
( still experimental)
• TNF-α blockade therapy- in resistant cases of
PVNS

28. • PVNS is a benign lesion but recur locally
• Local PVNS- recurrence of 10-25%
• Diffuse PVNS- 15% at 5 years
35% at 25 years

29. Conclusion
PVNS represents an uncommon benign
hypertrophic synovial process
 It is characterized by villous, nodular, and
villo nodular proliferation and pigmentation
from hemosiderin
 The MR imaging is useful for diagnosis and is
optimal for identifying the extent of synovial
disease, surveying and detecting recurrence.
Treatment is to remove the lesion as
completely as possible