eMedicine Specialties > Orthopedic Surgery > Neoplasms

Giant Cell Tumor of the Tendon Sheath

Author: James R Verheyden, MD, Consulting Surgeon, Department of Orthopedic Surgery, The Orthopedic and Neurosurgical Center of the Cascades
Coauthor(s): Timothy A Damron, MD, David G Murray Endowed Professor, Department of Orthopedic Surgery, Professor, Orthopedic Oncology and Adult Reconstruction, Vice Chair, Department of Orthopedics, State University of New York Upstate Medical University at Syracuse
Contributor Information and Disclosures

Updated: Jun 26, 2009

Introduction

Giant cell tumors of the tendon sheath are the second most common tumors of the hand, with simple ganglion cysts being the most common. Chassaignac first described these benign soft-tissue masses in 1852, and he overstated their biologic potential in referring to them as cancers of the tendon sheath.

Radiograph demonstrates the bony erosion associat...

Radiograph demonstrates the bony erosion associated with some giant cell tumors of the tendon sheath and shows the unmineralized soft-tissue shadow of the mass.

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Radiograph demonstrates the bony erosion associat...

Radiograph demonstrates the bony erosion associated with some giant cell tumors of the tendon sheath and shows the unmineralized soft-tissue shadow of the mass.



Typical T2-weighted MRI appearance of a giant cel...

Typical T2-weighted MRI appearance of a giant cell tumor of the tendon sheath. Most of the tumor has intermediate signal intensity, and portions of the tumor have low signal intensity; the latter finding likely reflects signal attenuation due to hemosiderin deposition.

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Typical T2-weighted MRI appearance of a giant cel...

Typical T2-weighted MRI appearance of a giant cell tumor of the tendon sheath. Most of the tumor has intermediate signal intensity, and portions of the tumor have low signal intensity; the latter finding likely reflects signal attenuation due to hemosiderin deposition.



Giant cell tumors of the soft tissue are classified into 2 types: the common localized type and the rare diffuse type. The rare diffuse form is considered the soft tissue counterpart of diffuse pigmented villonodular synovitis (PVNS) and typically affects the lower extremities.1 Its anatomic distribution parallels that of PVNS, with lesions most commonly found around the knee, followed by the ankle and foot; however, the diffuse form occasionally affects the hand. Typically, these lesions, like those of PVNS, occur in young patients; 50% of cases are diagnosed in patients younger than 40 years. The diffuse form is often locally aggressive, and multiple recurrences after excision are common.

Because of the similarities in age, tumor locations, clinical presentations, and symptoms for patients with PVNS and patients with the diffuse form of giant cell tumors of the tendon sheath, the diffuse form probably represents an extra-articular extension of a primary intra-articular PVNS process. Findings from flow cytometric DNA analysis suggest that PVNS and giant cell tumors of the tendon sheath are histopathologically similar but clinically distinct lesions.2,3,3   When the origin of these poorly confined soft-tissue masses is uncertain, Enzinger and Weiss4 classify these tumors as the diffuse type of giant cell tumors of the tendon sheath, whether or not they involve the adjacent joint.5

This article focuses on the common localized form of giant cell tumors—that is, the giant cell tumors of the tendon sheath that are often found in the hands and feet.6,7,8,9,10

History of the Procedure

Giant cell tumors of the tendon sheath are usually painless masses that have been present for a long time. The reported duration of symptoms ranges from weeks to as long as 30 years. These tumors usually cause no symptoms, except for occasional distal numbness; however, mild disability may result from impaired function of the digit secondary to the size of the lesion.

Frequency

Giant cell tumors of the tendon sheath are the second most common tumors in the hand; simple ganglion cysts are the most common. Giant cell tumors of the tendon sheath most commonly occur in patients aged 30-50 years, with a peak incidence in those aged 40-50 years. Rarely are these tumors found in patients younger than 10 years or older than 60 years. The female-to-male ratio is 3:2.

Giant cell tumors of the tendon sheath are associated with degenerative joint disease, especially in the distal interphalangeal (DIP) joint. Jones et al11 noted degenerative joint disease in the joint from which a tumor arose or in the joint nearest to the mass in 46 of 91 cases in which radiographs were reviewed. An occasional association with rheumatoid arthritis has been reported;12 however, to the authors' knowledge, no pathogenetic relationship between rheumatoid arthritis and giant cell tumor of the tendon sheath has been demonstrated, and their simultaneous occurrence may be coincidental. Antecedent trauma occurs in a variable number of these patients, but its association with these tumors is also probably coincidental.

Etiology

As is true for most soft-tissue tumors, the etiology of giant cell tumors of the tendon sheath is unknown. Pathogenetic theories have included trauma, disturbed lipid metabolism, osteoclastic proliferation, infection, vascular disturbances, immune mechanisms, inflammation, neoplasia, and metabolic disturbances.13 Probably the most widely accepted theory, as Jaffe et al14 proposed, is that of a reactive or regenerative hyperplasia associated with an inflammatory process.

Histochemical evidence shows that the mononuclear cells and giant cells present in these lesions resemble osteoclasts,15,16 suggesting a bone marrow–derived monocyte/macrophage lineage for these tumors. Recent polymerase chain reaction (PCR) assays have shown that giant cell tumors of the tendon sheath are polyclonal proliferations,17 suggesting that these masses are nonneoplastic proliferations, if one accepts the premise that a population of cells forming a tumorous mass must show clonality to be classified as a neoplasm.

Presentation

Typically, these masses occur along the volar aspect of the hand and fingers and are most commonly adjacent to the DIP joint. Two thirds of these masses are located along the volar aspect of the fingers (see Image 1). The index and long fingers are most commonly involved. Despite the prevalence of volar lesions, a dorsal location is not uncommon. A slight predominance for the right hand exists. The second most common site is the toe. Less common sites include extra-articular areas around larger joints, such as the knees, wrists, and ankles.

Giant cell tumors of the tendon sheath are firm, lobulated, nontender, slow-growing masses that are firmly fixed to the underlying structures. Usually, the overlying skin is freely mobile over proximal masses in the fingers. The skin is adherent to distal tumors. In digital lesions, mild numbness in the distal part of the involved fingertip is occasionally present. The lesion is not transilluminating. (Transillumination is more consistent with a cystic structure.)

The clinical differential diagnosis may include foreign body granuloma, necrobiotic granuloma, tendinous xanthoma,18 fibroma of the tendon sheath, infection, ganglion cyst, rheumatoid nodule, epidermoid cyst, lipoma, and a knuckle pad, among other less common entities. Many of these entities can often be excluded with careful history taking and physical examination.

When the pressure of the mass causes cortical erosion or when the mass has intralesional calcification, the radiographic differential diagnosis includes synovial chondromatosis, calcific tendinitis, and periosteal chondroma. Other entities that cannot be excluded on the basis of clinical findings in many cases include fibrokeratoma, myxoid cyst, reticulohistiocytoma, metastasis, and soft-tissue sarcomas (particularly epithelioid sarcoma and synovial sarcoma); these entities can only be definitively distinguished by means of histologic review.

Relevant Anatomy

See Workup, Histologic Findings.

Contraindications

A patient's poor medical health and the presence of life-threatening illnesses are contraindications to the surgical resection of these tumors.

More on Giant Cell Tumor of the Tendon Sheath

Overview: Giant Cell Tumor of the Tendon Sheath
Workup: Giant Cell Tumor of the Tendon Sheath
Treatment: Giant Cell Tumor of the Tendon Sheath
Follow-up: Giant Cell Tumor of the Tendon Sheath
Multimedia: Giant Cell Tumor of the Tendon Sheath
References
Further Reading
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References

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  2. Abdul-Karim FW, el-Naggar AK, Joyce MJ. Diffuse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: a clinicopathologic and flow cytometric DNA analysis. Hum Pathol. Jul 1992;23(7):729-35. [Medline].

  3. Mathews RE, Gould JS, Kashlan MB. Diffuse pigmented villonodular tenosynovitis of the ulnar bursa--a case report. J Hand Surg [Am]. Jan 1981;6(1):64-9. [Medline].

  4. Enzinger FM, Weiss SH. Benign tumors and tumorlike lesions of synovial tissue. In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors. St Louis, Mo: Mosby;1995:735-55.

  5. Abimelec P, Cambiaghi S, Thioly D. Subungual giant cell tumor of the tendon sheath. Cutis. Oct 1996;58(4):273-5. [Medline].

  6. Choudhury M, Jain R, Nangia A. Localized tenosynovial giant cell tumor of tendon sheath. A case report. Acta Cytol. May-Jun 2000;44(3):463-6. [Medline].

  7. Ciattaglia G, Filosa G, Bugatti L. Giant cell tumor of tendon sheath. J Am Acad Dermatol. Oct 1991;25(4):728-9. [Medline].

  8. LaRussa LR, Labs K, Schmidt RG. Giant cell tumor of tendon sheath. J Foot Ankle Surg. Nov-Dec 1995;34(6):541-6. [Medline].

  9. Schleicher SM. Giant cell tumor of tendon sheath. Cutis. Mar 1997;59(3):133-4. [Medline].

  10. Wu NL, Hsiao PF, Chen BF, et al. Malignant giant cell tumor of the tendon sheath. Int J Dermatol. Jan 2004;43(1):54-7. [Medline].

  11. Jones FE, Soule EH, Coventry MB. Fibrous xanthoma of synovium (giant-cell tumor of tendon sheath, pigmented nodular synovitis). A study of one hundred and eighteen cases. J Bone Joint Surg Am. Jan 1969;51(1):76-86. [Medline].

  12. Reginato A, Martinez V, Schumacher HR. Giant cell tumour associated with rheumatoid arthritis. Ann Rheum Dis. Jul 1974;33(4):333-41. [Medline].

  13. Hosaka M, Hatori M, Smith R, Kokubun S. Giant cell formation through fusion of cells derived from a human giant cell tumor of tendon sheath. J Orthop Sci. 2004;9(6):581-4. [Medline].

  14. Jaffe HL, Lichtenstein HL, Elsutro CJ. Pigmented villonodular synovitis, bursitis, and tenosynovitis. Arch Pathol. 1941;31:731-65.

  15. Darling JM, Goldring SR, Harada Y. Multinucleated cells in pigmented villonodular synovitis and giant cell tumor of tendon sheath express features of osteoclasts. Am J Pathol. Apr 1997;150(4):1383-93. [Medline].

  16. Wood GS, Beckstead JH, Medeiros LJ. The cells of giant cell tumor of tendon sheath resemble osteoclasts. Am J Surg Pathol. Jun 1988;12(6):444-52. [Medline].

  17. Vogrincic GS, O''Connell JX, Gilks CB. Giant cell tumor of tendon sheath is a polyclonal cellular proliferation. Hum Pathol. Jul 1997;28(7):815-9. [Medline].

  18. Stewart MJ. Benign giant-cell synovioma and its relation to "xanthoma". JBJS. 1948;30B:522-7.

  19. Jelinek JS, Kransdorf MJ, Shmookler BM. Giant cell tumor of the tendon sheath: MR findings in nine cases. AJR Am J Roentgenol. Apr 1994;162(4):919-22. [Medline].

  20. Bancroft LW, Peterson JJ, Kransdorf MJ. Imaging of soft tissue lesions of the foot and ankle. Radiol Clin North Am. Nov 2008;46(6):1093-103. [Medline].

  21. Wang Y, Tang J, Luo Y. The value of sonography in diagnosing giant cell tumors of the tendon sheath. J Ultrasound Med. Oct 2007;26(10):1333-40. [Medline].

  22. Agarwal PK, Gupta M, Srivastava A. Cytomorphology of giant cell tumor of tendon sheath. A report of two cases. Acta Cytol. Mar-Apr 1997;41(2):587-9. [Medline].

  23. Iyer VK, Kapila K, Verma K. Fine-needle aspiration cytology of giant cell tumor of tendon sheath. Diagn Cytopathol. Aug 2003;29(2):105-10. [Medline].

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  25. Moore JR, Weiland AJ, Curtis RM. Localized nodular tenosynovitis: experience with 115 cases. J Hand Surg [Am]. May 1984;9(3):412-7. [Medline].

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  27. Wright CJE. Benign giant-cell synovioma; an investigation of 85 cases. Br J Surg. Jan 1951;38(151):257-71. [Medline].

  28. Goda JS, Patil P, Krishnappan C, Elangovan D. Giant cell tumor of the tendon sheath treated by brachytherapy (surface mold) technique-A technical illustration. Brachytherapy. Oct 22 2008;[Medline].

  29. Rodrigues C, Desai S, Chinoy R. Giant cell tumor of the tendon sheath: a retrospective study of 28 cases. J Surg Oncol. Jun 1998;68(2):100-3. [Medline].

  30. Darwish FM, Haddad WH. Giant cell tumour of tendon sheath: experience with 52 cases. Singapore Med J. Nov 2008;49(11):879-82. [Medline].

  31. Messoudi A, Fnini S, Labsaili N, Ghrib S, Rafai M, Largab A. [Giant cell tumors of the tendon sheath of the hand: 32 cases]. Chir Main. Jun 2007;26(3):165-9. [Medline].

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Further Reading

Related eMedicine topics

Giant Cell Tumor (Orthopedic Surgery)

Giant Cell Tumor (Radiology)

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Keywords

giant cell tumor, localized nodular tenosynovitis, fibrous xanthoma, xanthoma of the synovium, xanthoma of the tendon sheath, xanthogranuloma, xanthosarcoma, fibroma of tendon, myeloid endothelioma, endothelioma, villous arthritis, fibrohemosideric sarcoma, giant cell fibrohemangioma, benign synovioma, sclerosing hemangioma, pigmented villonodular synovitis

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Contributor Information and Disclosures

Author

James R Verheyden, MD, Consulting Surgeon, Department of Orthopedic Surgery, The Orthopedic and Neurosurgical Center of the Cascades
James R Verheyden, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, and American Society for Surgery of the Hand
Disclosure: Nothing to disclose.

Coauthor(s)

Timothy A Damron, MD, David G Murray Endowed Professor, Department of Orthopedic Surgery, Professor, Orthopedic Oncology and Adult Reconstruction, Vice Chair, Department of Orthopedics, State University of New York Upstate Medical University at Syracuse
Timothy A Damron, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Surgeons, American Medical Association, Children's Oncology Group, Connective Tissue Oncology Society, Musculoskeletal Tumor Society, Orthopaedic Research Society, and Society for Experimental Biology and Medicine
Disclosure: Lippincott, Williams, and Wilkins Royalty Editing/writing textbook; Genentech Grant/research funds Clinical research; Orthovita Grant/research funds Clinical research; National Institutes of Health Grant/research funds Clinical research

Medical Editor

Timothy A Damron, MD, David G Murray Endowed Professor, Department of Orthopedic Surgery, Professor, Orthopedic Oncology and Adult Reconstruction, Vice Chair, Department of Orthopedics, State University of New York Upstate Medical University at Syracuse
Timothy A Damron, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Surgeons, American Medical Association, Children's Oncology Group, Connective Tissue Oncology Society, Musculoskeletal Tumor Society, Orthopaedic Research Society, and Society for Experimental Biology and Medicine
Disclosure: Lippincott, Williams, and Wilkins Royalty Editing/writing textbook; Genentech Grant/research funds Clinical research; Orthovita Grant/research funds Clinical research; National Institutes of Health Grant/research funds Clinical research

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Sean P Scully, MD, PhD, Professor, Department of Orthopedics, University of Miami
Sean P Scully, MD, PhD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, International Society on Thrombosis and Haemostasis, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

CME Editor

Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital
Dinesh Patel, MD, FACS is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Association of Physicians of Indian Origin, American College of International Physicians, and American College of Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Harris Gellman, MD, Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami School of Medicine
Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, and Arkansas Medical Society
Disclosure: Nothing to disclose.

 
 
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