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Tendosynovial Giant Cell Tumor

Synonyms: Nodular Tendosynovitis, Fibrous Histiocytoma of Tendon Sheath, Benign Synovioma, Xanthogranuloma, Giant Cell Tumor of the Tendon Sheath - Localized Type

Definition

Probably neoplastic lesion composed of a circumscribed proliferation of mononuclear cells of synovial type, accompanied by varying proportions of multinucleated giant cells of osteoclastic type.

Clinical Features

  • Common
  • More frequent in women
  • Usually:
    • young or middle-aged
    • distributed between:
      • wrist and fingertips
      • ankle and toe tips
    • More often:
      • proximal than distal on both hands and feet
      • on flexor surfaces
    • Other sites can be affected, including vertebral column1
    • Nearly always benign:
      • very rare malignant counterpart described
    • May erode contiguous bone by pressure

Pathogenesis

  • Nature controversial
  • Considered by some as reactive2
  • Most regard it as neoplastic:
    • supported by clonal chromosomal aberrations3

Gross Pathology

  • Single mass
  • Usually 1–3cm diameter
  • Fairly well-defined capsule
  • May be somewhat lobulated (Figs 1 and 2
    Gross appearance of tendosynovial giant cell tumor. The lesion is small, well circumscribed, solid, and with a brownish cast.

    Fig. 1: Gross appearance of tendosynovial giant cell tumor. The lesion is small, well circumscribed, solid, and with a brownish cast.

    Whole-mount appearance of a lesion located in the finger. Note the lobulated quality.

    Fig. 2: Whole-mount appearance of a lesion located in the finger. Note the lobulated quality.

    )
  • Varies in color from whitish gray to yellowish brown
  • So-called diffuse form tends to:
    • be larger
    • have infiltrative margins4,5

Histopathology

Differential Diagnosis

  • Sarcoma:
    • due to:
      • great cellularity
      • variable pattern
      • mitotic figures
    • Epithelioid sarcoma:
      • main differential diagnosis
      • epithelioid sarcoma favored by:
        • granuloma-like formations
        • necrosis
        • invasiveness
        • epithelioid features
        • keratin immunoreactivity

Management

  • Removal:
    • if incomplete, may recur locally

References

1 Furlong MA, Motamedi K, Laskin WB, Vinh TN, Murphey M, Sweet DE, et al. Synovial-type giant cell tumors of the vertebral column: a clinicopathologic study of 15 cases, with a review of the literature and discussion of the differential diagnosis. Hum Pathol. 2003;34:670–679.

2 Jaffe HL, Lichtenstein L, Sutro CJ. Pigmented villonodular synovitis, bursitis, and tenosynovitis. Arch Pathol. 1941;31:731–765.

3 Sciot R, Rosai J, Dal Cin P, De Wever I, Fletcher CD, Mandahl N, et al. Analysis of 35 cases of localized and diffuse tenosynovial giant cell tumor: a report from the Chromosomes and Morphology (CHAMP) Study Group. Mod Pathol. 1999;12:576–579.

4 Ferrer J, Namiq A, Carda C, Lopez-Gines C, Tawfik O, Llombart-Bosch A. Diffuse type of giant-cell tumor of tendon sheath: an ultrastructural study of two cases with cytogenetic support. Ultrastruct Pathol. 2002;26:15–22.

5 Somerhausen NS, Fletcher CD. Diffuse-type giant cell tumor: clinicopathologic and immunohistochemical analysis of 50 cases with extraarticular disease. Am J Surg Pathol. 2000;24:479–492.

6 Alguacil-Garcia A, Unni KK, Goellner JR. Giant cell tumor of tendon sheath and pigmented villonodular synovitis. An ultrastructural study. Am J Clin Pathol. 1978;69:6–17.

7 O'Connel JX, Fanburg JC, Rosenberg AE. Giant cell tumor of tendon sheath and pigmented villonodular synovitis. Immunophenotype suggests a synovial cell origin. Hum Pathol. 1995;26:771–775.

8 Tashiro H, Iwasaki H, Kikuchi M, Ogata K, Okazaki M. Giant cell tumors of tendon sheath. A single and multiple immunostaining analysis. Pathol Int. 1995;45:147–155.

9 Darling JM, Goldring SR, Harada Y, Handel ML, Glowacki J, Gravallese EM. Multinucleated cells in pigmented villondular synovitis and giant cell tumor of tendon sheath express features of osteoclasts. Am J Pathol. 1997;150:1383–1393.

10 Chung EB, Enzinger FM. Fibroma of tendon sheath. Cancer. 1979;44:1945–1954.

11 Maluf HM, DeYoung BR, Swanson PE, Wick MR. Fibroma and giant cell tumor of tendon sheath. A comparative histological and immunohistological study. Mod Pathol. 1995;8:155–159.

12 Bertoni F, Unni KK, Beabout JW, Sim FH. Malignant giant cell tumor of the tendon sheaths and joints (malignant pigmented villonodular synovitis). Am J Surg Pathol. 1997;21:153–163.

13 Ushijima M, Hashimoto H, Tsuneyoshi M, Enjoji M, Miyamoto Y, Okue A. Malignant giant cell tumor of tendon sheath. Report of a case. Acta Pathol Jpn. 1985;35:699–709.

Last updated: 6 Mar 2006

Tendosynovial Giant Cell Tumor

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