Giant Cell Tumor of Tendon Sheath 

Updated: Aug 27, 2018
Author: James R Verheyden, MD; Chief Editor: Harris Gellman, MD 

Overview

Background

Giant cell tumors of the tendon sheath are the second most common tumors of the hand, with simple ganglion cysts being the most common.[1] 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.

Giant cell tumors of the soft tissue are classified into the following two types:

  • Localized (common)
  • Diffuse (rare)

The rare diffuse form is considered the soft-tissue counterpart of diffuse pigmented villonodular synovitis (PVNS) and typically affects the lower extremities.[2] Its anatomic distribution parallels that of PVNS, with lesions most commonly found around the knee, followed by the ankle and foot; however, it 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.[3, 4]  When the origin of these poorly confined soft-tissue masses is uncertain, Enzinger and Weiss[5] classify these tumors as the diffuse type of giant cell tumors of the tendon sheath, whether or not they involve the adjacent joint.[6]

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.[7, 8, 9, 10, 11]

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.[12] Probably the most widely accepted theory, as Jaffe et al proposed,[13] 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,[14, 15] and this resemblance suggests a bone marrow–derived monocyte/macrophage lineage for these tumors. Polymerase chain reaction (PCR) assays have shown that giant cell tumors of the tendon sheath are polyclonal proliferations,[16] which suggests 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.

Epidemiology

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 al[17] 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[18] ; however, to the authors' knowledge, no pathogenetic relation 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.

Prognosis

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

  • Phalen et al, [19] 9% recurrence rate in 56 cases
  • Moore et al, [20] 9% recurrence rate in 115 cases
  • Jones et al, [17] 17% recurrence rate in 95 cases
  • Reilly et al, [21] 27% recurrence rate in 70 cases
  • Wright, [22] 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 following:

  • Presence of adjacent degenerative joint disease
  • Injury at the DIP joint of the finger or the interphalangeal (IP) joint of the thumb
  • 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.[23] For retrospective studies, see Rodrigues et al,[24] Darwish and Haddad,[25] and Messoudi et al.[26] For a significant study in children, see Gholve et al.[27]

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.

 

Presentation

History

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.

Physical Examination

Typically, these masses occur along the volar aspect of the hand and fingers[28] and are most commonly adjacent to the distal interphalangeal (DIP) joint.[29, 30, 31] Two thirds of these masses are located along the volar aspect of the fingers (see the image below). 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.[32]

Image in a 44-year-old right hand–dominant man who 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.

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,[33] 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.

 

Workup

Imaging Studies

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 the images below).

Radiograph demonstrates cortical erosion from the 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 associate 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.

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 the images below).

Radiograph demonstrates cortical erosion from the 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.
Radiograph demonstrates cortical erosion from the Radiograph demonstrates cortical erosion from the pressure effect of the overlying giant cell tumor of the tendon sheath.

Magnetic resonance imaging

On magnetic resonance imaging (MRI), giant cell tumors of the tendon sheath frequently have a unique appearance for an extra-articular soft-tissue mass.[34, 35] On both T1- and T2-weighted MRI, at least some portions of the tumor have decreased signal intensity (see the images below) similar to that seen with pigmented villonodular synovitis (PVNS). However, this appearance is not entirely specific to giant cell tumors of the tendon sheath.

Typical T2-weighted MRI appearance of a giant cell 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 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 t Typical T1-weighted MRI findings in a giant cell tumor of the tendon sheath overlying the metacarpophalangeal joint. Note the low-signal-intensity areas.
Corresponding T2-weighted MRI findings in the tumo Corresponding T2-weighted MRI findings in the tumor shown in the image above. Note the areas of low signal intensity.

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.

Ultrasonography

For the value of sonograms in this setting, see studies by Bancroft et al[35] and Wang et al.[36]

Histologic Findings

Gross 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 the images below).

Intraoperative excision of the giant cell tumor of Intraoperative excision of the giant cell tumor of the tendon sheath, 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 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 margin 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. Hemosiderin-containing xanthoma cells are common and often localized at the periphery of the lesion. (See the images below.)

Typical microscopic appearance of a giant cell tum 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.
Histologic findings of a giant cell tumor of the t Histologic findings of a giant cell tumor of the tendon sheath.
High-power photomicrograph of giant cell tumor of High-power photomicrograph of giant cell tumor of the tendon sheath shows occasional numerous mononuclear cells, scattered giant cells, and hemosiderin-containing xanthoma cells.
High-power photomicrograph depicts the histologic High-power photomicrograph depicts the histologic findings of a giant cell tumor of the tendon sheath.

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 those of 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.[37, 38]

 

Treatment

Surgical Therapy

Marginal excision of giant cell tumor of the tendon sheath is the treatment of choice (see the images below). A patient's poor medical health and the presence of life-threatening illnesses are contraindications to the surgical resection of these tumors.

An 11-year-old girl presented with this firm nonfl 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 after margin Giant cell tumor of the tendon sheath after marginal excision from an 11-year-old girl who presented with a firm nonfluctuant mass over her posterior medial left ankle that had been present for 5 months and had not increased in size.

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.

Because the mass is frequently associated with the tendon sheath or synovial joint, complete excision can be difficult. 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.

Jones et al[17] noted an association between these lesions and arthritis at the distal interphalangeal (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%.

 

Questions & Answers