Updated: 7/1/2021

Giant Cell Tumor of Tendon Sheath

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  • summary
    • Giant Cell Tumor of Tendon Sheath, also known as pigmented villonodular tumor of the tendon sheath, is a benign nodular tumor that is found on the tendon sheath of the hands and feet
    • Diagnosis is made clinically with a firm, nodular mass that does not transilluminate with MRI studies showing decreased signal intensity on both T1-and T2-weighted MR imaging.
    • Treatment is usually marginal excision of the mass. 
  • Epidemiology
    • Incidence
      • common
        • second most common soft-tissue tumor seen in the hand, following ganglion cyst
    • Demographics
      • present in 3rd-5th decade of life
    • Anatomic location
      • it is most common on palmar surface of radial three digits near DIPJ
  • Presentation
    • Symptoms
      • enlarging mass
      • pain, worse with activity (or wearing shoes, for foot lesions)
    • Physical exam
      • firm, nodular mass that does not transilluminate
  • Imaging
    • 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
  • Histology
    • 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
  • Differential 
    • Ganglion cyst
      • cystic component
    • Pigmented villonodular synovitis (PVNS)
      • histologically identical
      • involves larger joints
    • Desmoid tumor
    • fibroma/fibrosarcoma
    • glomangioma
  • Treatment
    • 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
  • Prognosis
    • No reports of metastasis in literature

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(OBQ18.146) A 39-year-old patient presents with a left thumb mass at the interphalangeal joint of eight months duration. The patient denies any pain but is bothered by the appearance and limited range of motion. The patient opts to undergo surgical excision. Figure A depicts a clinical photograph of the patient's hand. Figures B, C, and D depict axial T1, T2, and T1 with contrast MRI sequences of the thumb, respectively. Figure E depicts the histology of the lesion at the time of marginal excision. What is the next appropriate step?

QID: 213042
FIGURES:

Ray resection

8%

(158/1919)

Initiate chemotherapy

2%

(45/1919)

Routine follow-up

55%

(1061/1919)

Return to OR for wide tumor bed resection

28%

(536/1919)

Radiation of the tumor bed

5%

(97/1919)

N/A A

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