Soft tissue tumours- Synovial Tissue Flashcards Preview

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Flashcards in Soft tissue tumours- Synovial Tissue Deck (26):
1

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

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2

What is the pathology of  Pigmented  Villonodular Synovitis?

  • 1/2 of pts report hx of trauma
  • thought to be a reactive process

3

What is the genetics of  Pigmented  Villonodular Synovitis?

  • 5q33 Chromosomal Rearrangment
  • increased expression of CSF1 gene

4

What is the prognosis of  Pigmented  Villonodular Synovitis?

  • Local reoccurrance is common

5

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

6

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

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7

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

8

what is seen on Arthroscopy in a pt with pigmented villonodular synovitis?

  • Brownish/Reddich inflamed synovium = pvns

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9

What is see on histology of PVNS?

  • Haemosiderin Stained multinucleated Giant cells
  • Highly vascular villi with hyperplastic synovial cells

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10

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%
  •  

11

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

12

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

13

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

14

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

15

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

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16

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




     

 

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17

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

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18

 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

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19

What is synovial sarcoma?

  • Malignant soft tissue sarcoma which arises nr joints but rarely within joints
  • the cell of origin is unknown
    • its is not the synovial cell so the name synovial sarcoma is a misnomer
  • Most common sarcoma in young adults 15-40 yrs
  • most common malignant sarcoma of the foot
  • M>F
  • Genetics= Chromosomal translocation t(X;18) in 90% cases-> SYT-SSX1,2 or 4 fusion protein
  • Prognosis- overall poor
    • 5 yr survival approx 50% (w lymph node resectn)
    • 10 yr survival approx 25%

20

What is the risk of metastatic spread in synovial sarcoma?

  • Show high histological grade
  • Mets may develop in 30-60% pts
  • Lung is most common site (like other sarcomas)
  • Can metastasize to Lymph nodes ( rare for Sarcomas)

    • poor prognostic sign
       but not as a lung mets 

  • can stage with lymph node biopsy
  • Mets more common w large, deep high grade sarcomas

21

Name the sarcomas that can metastasize to lymph nodes?

  • Clear cell sarcomas
     

  • Epitheloid sarcomas

  • Angiosarcomas
  • Rhabdomyosarcomas

= CLEAR

22

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

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23

What is seen on xrays of synovial sarcoma?

  • Soft tissue mineralisation ( calcification) in these tumours
  • may resemble Heterophic ossification

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24

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

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25

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

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26

What is the tx for synovial sarcoma?

  • Operative
    • Wide Surgical Resection with Adjuvant radiotherapy
      • most pts
      • radiotx either neoadjuvant/adjuvant
      • chemotx may improve local control and overall survival