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Giant Cell Tumor of Soft Parts

Definition

Rare soft tissue tumor, mainly of adults, usually located in the extremities, morphologically and clinically analogous to giant cell tumor of the bone.

Clinical Features

  • Rare
  • Mainly adults and elderly
  • Location:
    • usually:
      • extremities
      • deep:
    • also superficial variety in subcutaneous tissue and fascia1

Pathogenesis

  • Histogenesis unclear2,3

Gross Pathology

  • Fig. 1
    Malignant giant cell tumor of soft parts. The tumor is relatively well circumscribed, bulges on the cut surface, and is focally hemorrhagic.

    Fig. 1: Malignant giant cell tumor of soft parts. The tumor is relatively well circumscribed, bulges on the cut surface, and is focally hemorrhagic.

Histopathology

  • Admixture of:
    • osteoclast-like multinucleated giant cells:
      • resemble osteoclasts:
        • in content of hydrolytic enzymes, such as acid phosphatase (Fig. 2
          Intense positivity for acid phosphatase in the multinucleated cells of giant cell tumor of soft tissues.

          Fig. 2: Intense positivity for acid phosphatase in the multinucleated cells of giant cell tumor of soft tissues.

          )
        • ultrastructurally
    • stromal cells1,3,4 (Fig. 3
      Giant cell tumor of soft parts. The appearance is remarkably similar to that of giant cell tumor of bone.

      Fig. 3: Giant cell tumor of soft parts. The appearance is remarkably similar to that of giant cell tumor of bone.

      )
      • probably only neoplastic component
      • some elongated and fibroblast-like
      • others plump, resembling histiocytes
        • some may be multinucleated but different from osteoclast-like elements
  • May be metaplastic bone:
    • usually as peripheral shell
    • perhaps induced by secretion of transforming growth factor β1 and β2 by tumor cells5
  • Characteristic multinodular configuration on low-power examination
  • Vascularity pronounced6
  • Overall appearance highly reminiscent of giant cell tumor of bones

Differential Diagnosis

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Prognosis

References

1 Gould E, Albores-Saavedra J, Rothe M, Mnaymneh W, Menedez-Aponte S. Malignant giant cell tumor of soft parts presenting as a skin tumor. Am J Dermatopathol. 1989;11:197–201.

2 Alguacil-Garcia A, Unni KK, Goellner JR. Malignant giant cell tumor of soft parts. An ultrastructural study of four cases. Cancer. 1977;40:244–253.

3 Guccion JG, Enzinger FM. Malignant giant cell tumor of soft parts. An analysis of 32 cases. Cancer. 1972;29:1518–1529.

4 Salm R, Sissons HA. Giant-cell tumours of soft tissues. J Pathol. 1972;107:27–39.

5 Teot LA, O'Keefe RJ, Rosier RN, O'Connell JX, Fox EJ, Hicks DG. Extraosseous primary and recurrent giant cell tumors: transforming growth factor beta-1 and beta-2 expression may explain metaplastic bone formation. Hum Pathol. 1996;27:625–632.

6 Angervall L, Hagmar B, Kindblom L-G, Merck C. Malignant giant cell tumor of soft tissues. A clinicopathologic, cytologic, ultrastructural, angiographic, and microangiographic study. Cancer. 1981;47:736–747.

7 Folpe AL, Morris RJ, Weiss SW. Soft tissue giant cell tumor of low malignant potential: a proposal for the reclassification of malignant giant cell tumor of soft parts. Mod Pathol. 1999;12:894–902.

8 O'Connell JX, Wehrli BM, Neilsen GP, Rosenberg AE. Giant cell tumors of soft tissue: a clinicopathologic study of 18 benign and malignant tumors. Am J Surg Pathol. 2000;24:386–395.

9 Oliveira AM, Dei Tos AP, Fletcher CD, Nascimento AG. Primary giant cell tumor of soft tissues: a study of 22 cases. Am J Surg Pathol. 2000;24:248–256.

Last updated: 26 Feb 2006

Giant Cell Tumor of Soft Parts

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