Updated: 6/16/2019

Giant Cell Tumor of Tendon Sheath

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  • A benign nodular tumor that is found on the tendon sheath of the hands and feet
  • Also known as pigmented villonodular tumor of the tendon sheath (PVNTS)
  • Epidemiology
    • present in 3rd-5th decade of life
    • incidence
      • second most common soft-tissue tumor seen in the hand, following ganglion cyst
    • location
      • it is most common on palmar surface of radial three digits near DIPJ
    • no reports of metastisis in literature
  • Symptoms
    • enlarging mass
    • pain, worse with activity (or wearing shoes, for foot lesions)
  • Physical exam
    • firm, nodular mass that does not transilluminate
  • Differential diagnosis
    • ganglion cyst
      • cystic component
    • pigmented villonodular synovitis
      • histologically identical
      • involves larger joints
    • desmoid tumor
    • fibroma/fibrosarcoma
    • glomangioma
  • Radiographs
    • pressure-type bone erosion can be seen in up to 5% of patients on radiographs 
  • Ultrasound
    • able to demonstrate relationship of lesion with adjacent tendon
    • homogeneously hypoechoic, although some heterogeneity may be seen in echo-texture in a minority of cases
    • most have some internal vascularity
  • MRI
    • MRI may be helpful diagnostically
    • appearance of the focal form is generally decreased signal intensity on both T1-and T2-weighted MR imaging 
  • Characterized by
    • proliferating histiocytes, moderately cellular (sheets of rounded or polygonal cells) 
    • hemosiderin (brown color) may be present, but typically less than seen with PVNS 
    • multinucleated giant cells are common
  • Operative 
    • marginal excision
      • 5-50% recurrence rate 
        • more common if tumor extends into joints and deep to the volar plate
        • tendon involvement associated with high recurrence rate 
        • local recurrence is usually treated with repeat excision
      • operative approach is dependant on location and extent of the tumor

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