SYNOVIOMA SYNOVIAL SARCOMA

SYNOVIOMA

SYNOVIAL SARCOMA

• 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

Etiology

• 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

Pathology

• 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

Imaging

• 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

Treatment

• 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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