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Friday 6 April 2018



  • uncommon malignant mesenchymal neoplasm
  • 8-10% soft tissue sarcomas
  • does not arise from synovium
  • arise in paraarticular region close to joint capsules, bursae, tendon sheath
  • can also in region without any relation to synovial structures – eg. Abdominal, parapharyngeal
  • undetermined
  • translocation involving chromosomes X and 18
    • seen in 90% cases
    • fusion transcript of SYT gene on 18 and SSX gene on X occurs
    • SYT : synovial sarcoma translocation gene
    • SSX : synovial sarcoma breakpoint gene
Clinical presentation
  • young 16-40 yrs – rare after 40
  • increased male preponderance recently – otherwise no sex preference
  • lower limb, around knee MC
  • in the soft tissue outside the joint ( rarely intraarticular)
  • slow growing – indolent course – late stage : demonstrate aggressiveness and metastasis to lung
  • soft tissue swelling or mass and progressive pain
  • Gross appearance
    • synovial attachment rare to find
    • sharp, circumscribed, round, lobulated
    • expansive growth compresses surrounding tissues – attenuated to form a pseudo capsule
    • firm, grayish pink, contains dark blood stained or yellowish brown areas of necrosis
  • Microscopic appearance
    • three basic patterns indicating synovial origin
      • formation of tissue spaces
        • varies from slit like clefts lined with flattened cells to well formed glandular spaces lined with cuboid cells – multilayered
        • homogeneous mucinous like fluid occupies the slit like spaces
      • formation of cell tufts
        • compact oval or polygonal cells arranged in tuft like formations – in either solid portion of tumor or in papillary projections into gland like spaces
      • epithelial like cells
        • supporting stroma of compact tissue
        • elongated cells with small dark nucleus
  • Histological subtypes (several exist)
    • biphasic (fibrous and epithelial)
      • MC
      • spindle cells and epithelial cells arrange in glandular or nest like pattern
    • monophasic
      • rare
      • no glandular pattern, resembles fibrosarcoma
      • interdigitating fascicles and spindle cells formed into ball-like patterns
    • glandular
      • monophasic epithelial type
      • resemble metastatic carcinoma
    • calcifying
  • radiograph : soft tissue mass in close proximity to joint – may show bone invasion
    • soft tissue calcification – amorphous in type – 20-30% cases
  • MRI
    • heterogeneous, septate mass of low signal intensity with infiltrative margins in T1 weighted sequences and high signal in T2 images
Differential diagnosis
  • Radiological
    • soft tissue chondroma
    • soft tissue chondrosarcoma
    • soft tissue osteosarcoma
    • fibrosarcoma
    • MFH
    • myositis ossificans
    • gout
    • tumor calcinosis
  • Pathological
    • soft tissue osteosarcoma
    • soft tissue chondrosarcoma
    • parosteal osteosarcoma
    • periosteal fibrosarcoma
    • endotheliosarcoma
    • metastatic carcinoma

  • very slow growing and metastasis late
  • local excision inadequate – radical amputation needed
  • poor prognosis
    • large size >5cm
    • older patients
    • tumors of upper extremity
    • local recurrence
    • monophasic pathology, rhabdoid cells, aneuploid tumors
  • good prognosis
    • biphasic histological pattern
    • location in foot
    • younger age, size <5cm
    • calcifying synovial sarcoma

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