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Tenosynovial Giant Cell Tumor, Extraarticular
Eric Walker, MD, MHA, FACR, FACHE; Stephanie A. Bernard, MD; Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Tenosynovial giant cell tumor (TSGCT)
          • Synonyms

            • Giant cell tumor of tendon sheath; nodular tenosynovitis
            • No longer recommended: extraarticular pigmented villonodular synovitis (PVNS)
          • Definitions

            • TSGCT: group of lesions most often arising from synovium of joints, bursae, and tendon sheaths, which demonstrates synovial differentiation
              • Similar pathologic entity to what formerly was referred to as PVNS
            • Usually divided according to site (intra- vs. extraarticular) and growth pattern (localized vs. diffuse)
              • Diffuse: most intraarticular; rarely extraarticular
              • Localized: intraarticular at knee (focal nodular synovitis) and extraarticular lesions
          • Tenosynovial Giant Cell Tumor, Extraarticular

            • Uncommon, rarely metastasizing fibrohistiocytic tumor representing extraarticular soft tissue form of TSGCT
          • Malignant Tenosynovial Giant Cell Tumor

            • De novo or transformation after multiple recurrences
            • Prevalence of malignant transformation of TSGCT is ~ 3%

          IMAGING

          • General Features

            • Radiographic Findings

              • MR Findings

                • Imaging Recommendations

                  • Ultrasonographic Findings

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          Selected References

                                          1. Bernthal NM et al: The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study. Orphanet J Rare Dis. 16(1):191, 2021
                                          2. Ota T et al: Tumor location and type affect local recurrence and joint damage in tenosynovial giant cell tumor: a multi-center study. Sci Rep. 11(1):17384, 2021
                                          3. De Saint Aubain Somerhausen N et al: Tenosynovial giant cell tumor. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 133-6, 2020
                                          4. Brahmi M et al: Long term term follow-up of tyrosine kinase inhibitors treatments in inoperable or relapsing diffuse type tenosynovial giant cell tumors (dTGCT). PLoS One. 15(5):e0233046, 2020
                                          5. Savvidou OD et al: Extra-articular diffuse giant cell tumor of the tendon sheath: a report of 2 cases. Arch Bone Jt Surg. 4(3):273-6, 2016
                                          6. Righi A et al: Metastasizing tenosynovial giant cell tumour, diffuse type/pigmented villonodular synovitis. Clin Sarcoma Res. 5:15, 2015
                                          7. Palmerini E et al: Tenosynovial giant cell tumour/pigmented villonodular synovitis: outcome of 294 patients before the era of kinase inhibitors. Eur J Cancer. 51(2):210-7, 2014
                                          8. Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors and synovial tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 330, 462-9, 2014
                                          9. Bancroft LW et al: Imaging of benign soft tissue tumors. Semin Musculoskelet Radiol. 17(2):156-67, 2013
                                          10. Zeinstra JS et al: Multifocal giant cell tumor of the tendon sheath: case report and literature review. Skeletal Radiol. 42(3):447-50, 2013
                                          11. Fotiadis E et al: Giant cell tumour of tendon sheath of the digits. A systematic review. Hand (N Y). 6(3):244-9, 2011
                                          12. Walker EA et al: Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am. 49(6):1197-217, vi, 2011
                                          13. Pallas A et al: Intense FDG uptake in an intra-articular localized giant-cell tumor of the tendon sheath (pigmented villonodular synovitis) mimics metastatic melanoma. Radiol Case Rep. 4(4):343, 2009
                                          14. Bancroft LW et al: Imaging of soft tissue lesions of the foot and ankle. Radiol Clin North Am. 46(6):1093-103, vii, 2008
                                          15. Weiss SW et al: Benign tumors and tumor-like lesions of synovial tissue. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 770-7, 2008
                                          16. Murphey MD et al: Pigmented villonodular synovitis: radiologic-pathologic correlation. Radiographics. 28(5):1493-518, 2008
                                          17. Sanghvi DA et al: Diffuse-type giant cell tumor of the subcutaneous thigh. Skeletal Radiol. 36(4):327-30, 2007
                                          18. Posligua L et al: Diffuse-type tenosynovial giant cell tumor in association with neurofibromatosis type 1-Noonan syndrome: possibly more than a chance relationship. Am J Surg Pathol. 30(6):734-8, 2006
                                          19. Middleton WD et al: Giant cell tumors of the tendon sheath: analysis of sonographic findings. AJR Am J Roentgenol. 183(2):337-9, 2004
                                          20. Aoki J et al: FDG-PET for preoperative differential diagnosis between benign and malignant soft tissue masses. Skeletal Radiol. 32(3):133-8, 2003
                                          21. Kitagawa Y et al: MR imaging for preoperative diagnosis and assessment of local tumor extent on localized giant cell tumor of tendon sheath. Skeletal Radiol. 32(11):633-8, 2003
                                          22. Hughes TH et al: Pigmented villonodular synovitis: MRI characteristics. Skeletal Radiol. 24(1):7-12, 1995
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Tenosynovial giant cell tumor (TSGCT)
                                                  • Synonyms

                                                    • Giant cell tumor of tendon sheath; nodular tenosynovitis
                                                    • No longer recommended: extraarticular pigmented villonodular synovitis (PVNS)
                                                  • Definitions

                                                    • TSGCT: group of lesions most often arising from synovium of joints, bursae, and tendon sheaths, which demonstrates synovial differentiation
                                                      • Similar pathologic entity to what formerly was referred to as PVNS
                                                    • Usually divided according to site (intra- vs. extraarticular) and growth pattern (localized vs. diffuse)
                                                      • Diffuse: most intraarticular; rarely extraarticular
                                                      • Localized: intraarticular at knee (focal nodular synovitis) and extraarticular lesions
                                                  • Tenosynovial Giant Cell Tumor, Extraarticular

                                                    • Uncommon, rarely metastasizing fibrohistiocytic tumor representing extraarticular soft tissue form of TSGCT
                                                  • Malignant Tenosynovial Giant Cell Tumor

                                                    • De novo or transformation after multiple recurrences
                                                    • Prevalence of malignant transformation of TSGCT is ~ 3%

                                                  IMAGING

                                                  • General Features

                                                    • Radiographic Findings

                                                      • MR Findings

                                                        • Imaging Recommendations

                                                          • Ultrasonographic Findings

                                                            • Nuclear Medicine Findings

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  Selected References

                                                                                  1. Bernthal NM et al: The diffuse-type tenosynovial giant cell tumor (dt-TGCT) patient journey: a prospective multicenter study. Orphanet J Rare Dis. 16(1):191, 2021
                                                                                  2. Ota T et al: Tumor location and type affect local recurrence and joint damage in tenosynovial giant cell tumor: a multi-center study. Sci Rep. 11(1):17384, 2021
                                                                                  3. De Saint Aubain Somerhausen N et al: Tenosynovial giant cell tumor. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press. 133-6, 2020
                                                                                  4. Brahmi M et al: Long term term follow-up of tyrosine kinase inhibitors treatments in inoperable or relapsing diffuse type tenosynovial giant cell tumors (dTGCT). PLoS One. 15(5):e0233046, 2020
                                                                                  5. Savvidou OD et al: Extra-articular diffuse giant cell tumor of the tendon sheath: a report of 2 cases. Arch Bone Jt Surg. 4(3):273-6, 2016
                                                                                  6. Righi A et al: Metastasizing tenosynovial giant cell tumour, diffuse type/pigmented villonodular synovitis. Clin Sarcoma Res. 5:15, 2015
                                                                                  7. Palmerini E et al: Tenosynovial giant cell tumour/pigmented villonodular synovitis: outcome of 294 patients before the era of kinase inhibitors. Eur J Cancer. 51(2):210-7, 2014
                                                                                  8. Kransdorf MJ et al: Fibrous and fibrohistiocytic tumors and synovial tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 330, 462-9, 2014
                                                                                  9. Bancroft LW et al: Imaging of benign soft tissue tumors. Semin Musculoskelet Radiol. 17(2):156-67, 2013
                                                                                  10. Zeinstra JS et al: Multifocal giant cell tumor of the tendon sheath: case report and literature review. Skeletal Radiol. 42(3):447-50, 2013
                                                                                  11. Fotiadis E et al: Giant cell tumour of tendon sheath of the digits. A systematic review. Hand (N Y). 6(3):244-9, 2011
                                                                                  12. Walker EA et al: Magnetic resonance imaging of benign soft tissue neoplasms in adults. Radiol Clin North Am. 49(6):1197-217, vi, 2011
                                                                                  13. Pallas A et al: Intense FDG uptake in an intra-articular localized giant-cell tumor of the tendon sheath (pigmented villonodular synovitis) mimics metastatic melanoma. Radiol Case Rep. 4(4):343, 2009
                                                                                  14. Bancroft LW et al: Imaging of soft tissue lesions of the foot and ankle. Radiol Clin North Am. 46(6):1093-103, vii, 2008
                                                                                  15. Weiss SW et al: Benign tumors and tumor-like lesions of synovial tissue. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 770-7, 2008
                                                                                  16. Murphey MD et al: Pigmented villonodular synovitis: radiologic-pathologic correlation. Radiographics. 28(5):1493-518, 2008
                                                                                  17. Sanghvi DA et al: Diffuse-type giant cell tumor of the subcutaneous thigh. Skeletal Radiol. 36(4):327-30, 2007
                                                                                  18. Posligua L et al: Diffuse-type tenosynovial giant cell tumor in association with neurofibromatosis type 1-Noonan syndrome: possibly more than a chance relationship. Am J Surg Pathol. 30(6):734-8, 2006
                                                                                  19. Middleton WD et al: Giant cell tumors of the tendon sheath: analysis of sonographic findings. AJR Am J Roentgenol. 183(2):337-9, 2004
                                                                                  20. Aoki J et al: FDG-PET for preoperative differential diagnosis between benign and malignant soft tissue masses. Skeletal Radiol. 32(3):133-8, 2003
                                                                                  21. Kitagawa Y et al: MR imaging for preoperative diagnosis and assessment of local tumor extent on localized giant cell tumor of tendon sheath. Skeletal Radiol. 32(11):633-8, 2003
                                                                                  22. Hughes TH et al: Pigmented villonodular synovitis: MRI characteristics. Skeletal Radiol. 24(1):7-12, 1995