eMedicine Specialties > Orthopedic Surgery > Neoplasms

Giant Cell Tumor of the Tendon Sheath

James R Verheyden, MD, Consulting Surgeon, Department of Orthopedic Surgery, The Orthopedic and Neurosurgical Center of the Cascades
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

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 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 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.

Workup

Imaging Studies

Radiograph demonstrates cortical erosion from the pressure effect of the adjacent mass on the radial aspect of the proximal phalanx.

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.

Radiograph demonstrates cortical erosion from the pressure effect of the overlying giant cell tumor of the tendon sheath. This apple-core effect is indicative of a primary soft-tissue mass that is causing external erosion, which should not be confused with a primary bone process such as periosteal chondroma.

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.

Typical T1-weighted MRI appearance of a giant cell tumor of the tendon sheath. Portions of the tumor have decreased signal intensity.

Typical T1-weighted MRI findings in a giant cell tumor of the tendon sheath overlying the metacarpophalangeal joint. Note the low-signal-intensity areas.

Plain radiography
- Plain radiographs demonstrate a benign-appearing circumscribed soft-tissue shadow in 50% of cases. These radiographs also show cortical erosion of the bone due to a pressure effect of the adjacent mass on the cortex in 10-20% of cases (see Images 2-3).
- True bone invasion is not typical and is suggestive of an aggressive neoplasm.
- Cortical erosion from these tumors is more common in the feet than elsewhere because the strong ligaments in this region frequently prevent outward tumor growth.
- Occasionally, intralesional soft-tissue calcification is seen with giant cell tumors of the tendon sheath. This intralesional calcification can be confused with synovial chondromatosis, periosteal chondroma, or calcific tendinitis.
- On rare occasions when extensive cortical erosion is present, the lesion may have a radiographic appearance suggestive of a periosteal chondroma (see Images 4-7).
Magnetic resonance imaging (MRI)
- Giant cell tumors of the tendon sheath frequently have a unique MRI appearance for an extra-articular soft-tissue mass.^19,20 On both T1- and T2-weighted MRIs, at least some portions of the tumor have decreased signal intensity (see Images 8-11) similar to that seen with PVNS. However, this appearance is not entirely specific to giant cell tumors of the tendon sheath.
- The degree to which these low–signal-intensity areas are present depends on the amount of hemosiderin, which varies. PVNS often has more low–signal-intensity areas on T2-weighted images, secondary to its higher hemosiderin content resulting from characteristic intralesional bleeding.
Sonography: For the value of sonograms, see studies by Bancroft et al ²⁰ and Wang et al²¹

Histologic Findings

Gross pathologic findings

Giant cell tumors of the tendon sheath have a well-circumscribed multilobular appearance and often possess shallow grooves along their deep surfaces created by the underlying tendons. These tumors are usually small, with a diameter of 0.5-5 cm. Compared with other lesions, giant cell tumors in the hand digits are usually smaller and have a more regular appearance. Giant cell tumors in the feet and elsewhere are often larger and more irregular in appearance. On cut sections, these tumors have a mottled appearance, varying in color from grayish-brown to yellow-orange. The coloration depends on the amount of hemosiderin, collagen, and histiocytes in the sample. Tumors with more hemosiderin deposition due to bleeding have more of the yellow-orange or even reddish-brown color (see Images 12-14).

Intraoperative excision of the giant cell tumor of the tendon sheath shown in Image 9, which has the typical golden-yellow color secondary to hemosiderin deposition. The radial digital nerve is dissected free and slightly volar to the mass.

After excision, the bone is curetted, leaving the exposed radial aspect of the proximal phalanx, as shown here.

Giant cell tumor of the tendon sheath after marginal excision.

Microscopic findings

Most giant cell tumors of the tendon sheath are moderately cellular and composed of sheets of rounded or polygonal cells that blend with hypocellular collagenized zones. Variable numbers of giant cells are present (see Image 15). Hemosiderin-containing xanthoma cells are common and often localized at the periphery of the lesion (see Image 16).

Typical microscopic appearance of a giant cell tumor of the tendon sheath. Sheets of rounded or polygonal cells blend with hypocellular collagenized zones; variable numbers of giant cells are present.

High-power photomicrograph of giant cell tumor of the tendon sheath shows occasional numerous mononuclear cells, scattered giant cells, and hemosiderin-containing xanthoma cells.

In the localized form of the disease, a mature collagen capsule often surrounds the tumor. This capsule is continuous, with fibrous septa within the substance of the tumor that divide it into vague nodules. In the diffuse form, the tumor is not surrounded by this capsule and instead grows in expansive sheets.

Giant cells are also less common in the diffuse form. The histologic features of the localized and diffuse forms of giant cell tumor of the tendon sheath and the localized and diffuse forms of PVNS are essentially the same; therefore, these diseases form a histopathologic spectrum in which the tumors range from benign lesions to more locally aggressive lesions.

Cytopathologic findings

The predominant cell type is the mononuclear cell. These round-to-polygonal cells are found alone or in papillary clusters and have eccentrically located nuclei that lack pleomorphism. Varying amounts of refractile golden-brown crystals of hemosiderin are characteristically found within the histiocytes.^22,23

Treatment

Surgical Therapy

Marginal excision of giant cell tumor of the tendon sheath is the treatment of choice (see Images 17-18). Complete excision can be difficult, as the mass is frequently associated with the tendon sheath or synovial joint. Often, partial excision of the joint capsule or tendon sheath is necessary for complete removal of the tumor. Meticulous dissection and exploration are essential because satellite lesions are common. A Freer elevator or other blunt probe is often helpful in teasing these satellite lesions from beneath the surrounding tendons or other structures. Avoid puncturing these lesions because seeding of adjacent soft-tissue structures may be possible. Occasionally, bony debridement with a curette or rongeur is necessary if adjacent bony erosion is present.

An 11-year-old girl presented with this firm nonfluctuant mass over her posterior medial left ankle that had been present for 5 months and had not increased in size. The mass was not transilluminating. Findings on frozen section were consistent with a benign giant cell tumor of the tendon sheath. The mass was marginally excised.

Giant cell tumor of the tendon sheath from the case shown in Image 17 after marginal excision.

Jones et al¹¹ noted an association between these lesions and arthritis at the DIP joint. If such arthritis is present, debridement or fusion may be necessary to completely eradicate the process. If the tumor involves the skin, consider the excision of an elliptical area of skin along with the mass. Skin excision may necessitate secondary skin grafting. Rarely, tendon reconstruction may be necessary if tumor excision compromises the associated tendon. Even with careful dissection, reported recurrence rates are 9-44%.

Intraoperative Details

The tumor may involve the tendon sheath, volar plate, capsular ligaments, and joints. Dorsal sites frequently involve the joints or tendinous attachments to bone. Volar sites are more frequently present near the joints, presumably because the fibrous flexor-tendon sheath is thinner at the level of the joints. In a review of 115 cases, 20% had extra-articular joint involvement. In the digits, these tumors are often intimately associated with the flexor or extensor tendon. If no intimate association exists, a stalk of tissue often connects the tumor to the tendon sheath. If the mass is relatively large, smaller satellite lesions extending into the surrounding tendon sheath and synovium may be found.

Outcome and Prognosis

The incidence of local recurrence is high, ranging from 9-44%. Researchers have reported the following rates:

Phalen et al,²⁴ 9% recurrence rate in 56 cases
Moore et al,²⁵ 9% recurrence rate in 115 cases
Jones et al,¹¹ 17% recurrence rate in 95 cases
Reilly et al,²⁶ 27% recurrence rate in 70 cases
Wright,²⁷ 44% recurrence rate in 69 cases

The variability in rates probably reflects incomplete excision of the lesions, especially the satellite nodules. Risk factors for recurrence include the presence of adjacent degenerative joint disease; an injury at the DIP joint of the finger or the interphalangeal joint of the thumb; and the radiographic presence of osseous pressure erosions. Goda et al have presented a new technique for the use of radiotherapy as an adjuvant modality to prevent local recurrence.²⁸ For retrospective studies, see Rodrigues et al,²⁹ Darwish and Haddad,³⁰ and Messoudi et al.³¹ For a significant study in children, see Gholve et al.³²

To the authors' knowledge, no cases of malignant degeneration of a benign giant cell tumor of the tendon sheath of the hand have been reported. These tumors also have no propensity to metastasize distally. A few sporadic cases of purported malignant giant cell tumors have been reported; however, most authors doubt that these malignant tumors exist, because this diagnosis is difficult to confirm.

Multimedia

Media file 1: Image in a 44-year-old right hand–dominant man who presented with a mass on the volar radial aspect of his left index finger. The mass was painless and had been slowly growing for 1.5 years.

Media file 2: Radiograph demonstrates cortical erosion from the pressure effect of the adjacent mass on the radial aspect of the proximal phalanx.

Media file 3: 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.

Media file 4: Radiograph demonstrates cortical erosion from the pressure effect of the overlying giant cell tumor of the tendon sheath. This apple-core effect is indicative of a primary soft-tissue mass that is causing external erosion, which should not be confused with a primary bone process such as periosteal chondroma.

Media file 5: Same tumor as in Image 4.

Media file 6: Histologic findings of tumor shown in Image 4.

Media file 7: High-power photomicrograph depicts the histologic findings of the tumor shown in Image 4.

Media file 8: 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.

Media file 9: Typical T1-weighted MRI appearance of a giant cell tumor of the tendon sheath. Portions of the tumor have decreased signal intensity.

Media file 10: Typical T1-weighted MRI findings in a giant cell tumor of the tendon sheath overlying the metacarpophalangeal joint. Note the low-signal-intensity areas.

Media file 11: Corresponding T2-weighted MRI findings in the tumor shown in Image 10. Note the areas of low signal intensity.

Media file 12: Intraoperative excision of the giant cell tumor of the tendon sheath shown in Image 9, which has the typical golden-yellow color secondary to hemosiderin deposition. The radial digital nerve is dissected free and slightly volar to the mass.

Media file 13: After excision, the bone is curetted, leaving the exposed radial aspect of the proximal phalanx, as shown here.

Media file 14: Giant cell tumor of the tendon sheath after marginal excision.

Media file 15: Typical microscopic appearance of a giant cell tumor of the tendon sheath. Sheets of rounded or polygonal cells blend with hypocellular collagenized zones; variable numbers of giant cells are present.

Media file 16: High-power photomicrograph of giant cell tumor of the tendon sheath shows occasional numerous mononuclear cells, scattered giant cells, and hemosiderin-containing xanthoma cells.

Media file 17: An 11-year-old girl presented with this firm nonfluctuant mass over her posterior medial left ankle that had been present for 5 months and had not increased in size. The mass was not transilluminating. Findings on frozen section were consistent with a benign giant cell tumor of the tendon sheath. The mass was marginally excised.

Media file 18: Giant cell tumor of the tendon sheath from the case shown in Image 17 after marginal excision.

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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

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

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.

Further Reading

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