Soft tissue tumours- Synovial Tissue Flashcards
PVNS Synovial Chromatosis Synovial sarcoma
What is Pigmented Villonodular Synovitis?
- An Idopathic Monoarticular Reactive Synovial Disease
- characterised by Exuberant Proliferation of synovial villi and nodules
- Common adults 30-50 yrs
- M=F
- location
- Knee 80%
- hip , shoulder /ankle
- when extra-articular extension= Giant Cell Tumour of Tendon Sheath
What is the pathology of Pigmented Villonodular Synovitis?
- 1/2 of pts report hx of trauma
- thought to be a reactive process
What is the genetics of Pigmented Villonodular Synovitis?
- 5q33 Chromosomal Rearrangment
- increased expression of CSF1 gene
What is the prognosis of Pigmented Villonodular Synovitis?
- Local reoccurrance is common
What is the hx and presentation of a pt with Pigmented Villonodular Synovitis?
Hx
- 50% pts will have prior hx of trauma to the area
Symptoms
- Pain and swelling
- Mechanical Pain and limited motion
- Recurrent atraumatic haemathrosis ** hallmark**
O/E
- extra-articular GCT tendon sheath= Painless, soft, mass along tendon sheath
- intra-articular PVNS- joint effusion/erytherma
What is seen on imaging of Pigmented Villonodular Synovitis?
Xrays
- Cystic erosion w sclerotic margins on both sides of the joint- see pic
CT
- Cystic erosions on both sides of joint
MRI
- Both low signal intensity on T1/T2= due to haemosiderin deposits
What is seen on aspiration of the knee with Pigmented Villonodular Synovitis?
- Grossly bloody effusion
- Synovial biopsy should be done if any doubt of dx
what is seen on Arthroscopy in a pt with pigmented villonodular synovitis?
- Brownish/Reddich inflamed synovium = pvns
What is see on histology of PVNS?
- Haemosiderin Stained multinucleated Giant cells
- Highly vascular villi with hyperplastic synovial cells
What is the tx of PVNS?
Non operative
- Observation
- minimal role for non operative if disease symptomatic
Operative
-
Total Synovectomy
- in grossly symptomatic and painful disease
- partial arthroscopic synovectomy vs open synovectomy- open can deal with posterior knee disease not arthroscopic
- Freq recurrence is common= incomplete synovectomy
- for extra-art disease
- Marginal excision is adequate for GCT tendon sheath
- recurrence ts with rpt excision
-
external beam Irradiation
- reduced rate of recurrence 10-20%
*
- reduced rate of recurrence 10-20%
What are the adv/dis of athroscopic synovectomy?
- preform as through resection as possible
- excellent for focal or limited PVNS
- minimally invasive proceedure
- quick return to function
- unable to access posterior portion of joint
- unable to address extra-articular disease
Decribe the technique for comnbined arthroscopis synovectomy and open posterior synovectomy of knee for PVNS?
- approach
- post approach to knee via transverse /S incision across popliteal fossa
- approach between heads of gastronemius
- retract NV bundle to access posterior joint caspule
- technique
- disease often seen posterior & extra-articular
- complete post synovectomy & resection of extra-articular disease
- Adv
- address all disease sections
- thorough synovectomy
- Dis
- Post approach to knee avoid NV bundle
What is the tx for advanced PVNS disease?
-
Total joint athroplasty and synovectomy
- in advanced disease w severe degenerative joint
- applicable to knee, hip , shoulder
-
Total Synovectomy and arthrodesis
- in severe disease of ankle
What is the complication of PVNS?
-
Reoccurrance
- for both intra-articular and extra-articular disease
- 30-50% recurrence rate despite synovectomy
- reduced to 10-20% w external beam radiation
What is synovial chondromatosis?
- A proliferative disease of the synovium assoc with cartilage metaplasia
- -> multiple intra-articular loose bodies
- ranges from synovial tissue to firm nodules of cartilage
- adults 30-50 yrs
- 2M:1F
- Knee most common location
- genetics
- occasional chromosome 6 abnormalies found
What is the presentation of synovial chondromatosis?
- Slow progression of symptoms
Pain and swelling - Pain worse with activity
- Mechanical symptoms including stiffness can occur in bursa overlying an osteochondroma
O/E
- reduced rom
- warmth, erytherma, tenderness
What is seen on imaging of synovial chondromatosis?
xrays
- varible depends on stage of disease
- may show stippled calcification
MRI
- Initally cartilage nodules are obly visible on MRI
- lobular appearance
- in later stage, signal drop out consistent with calcification
tx of synovial chondromatosis
Non operative
- Observation
- mild symptoms not affecting rom
Operative
-
Open or Arthroscopic Synovectomy and loose body resection +/- chondroplasty +/- labral debridement
- severe symptoms affecting rom
Outcomes
- tx is symptomatic but may help prevent degenerative joint disease
What is the presentation of a pt with synovial sarcoma?
- Typically a Growing Mass in Proximity to joint
- Painless or painful
- Occur in Para-Articular locations
- knee, shoulder, elbow, foot
- 60% in lower extremity
O/E
- Examine for regional Lymphadenopathy
What is seen on xrays of synovial sarcoma?
- Soft tissue mineralisation ( calcification) in these tumours
- may resemble Heterophic ossification
What is seen on Ct of synovial sarcoma?
- Can show calcification in the soft tissue mass
MRI
- Heterogenous mass
- typical dark on T1 and bright on T2
What is seen in histology with synovial sarcoma?
- Biphasic appearance
- spindle cells
- epithelial cells
- glands, nest or cyst like cells- see pics
- Immunostaining for
- Vimentin
- epithelial membrane antigen
- sporadic S-100
- epithelial cells stain positive for Keratin
