You are in: eMedicine Specialties > Orthopedic Surgery > NEOPLASMS Giant Cell Tumor of the Tendon SheathArticle Last Updated: Jan 12, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 9
  Author: James R Verheyden, MD, Consulting Surgeon, Department of Orthopedic Surgery, The Orthopedic & Neurosurgical Center of the Cascades James R Verheyden is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, and American Society for Surgery of the Hand 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 Editors: 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; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Sean P Scully, MD, PhD, Professor, Department of Orthopedics, University of Miami; Dinesh Patel, MD, FACS, Associate Clinical Professor of Orthopedic Surgery, Harvard Medical School; Chief of Arthroscopic Surgery, Department of Orthopedic Surgery, Massachusetts General Hospital; 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 Author and Editor Disclosure Synonyms and related keywords: 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 INTRODUCTIONSection 2 of 9
  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. 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. 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. When the origin of these poorly confined soft-tissue masses is uncertain, Enzinger and Weiss (1995) classify these tumors as the diffuse type of giant cell tumors of the tendon sheath, whether or not they involve the adjacent joint. 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. History of the ProcedureGiant 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. FrequencyGiant 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 (1969) 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; 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. EtiologyAs 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. Probably the most widely accepted theory, as Jaffe et al (1941) 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, 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, 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. ClinicalTypically, 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, 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 ANATOMYSection 3 of 9
See Workup, Histologic Findings. CONTRAINDICATIONSSection 4 of 9
A patient's poor medical health and the presence of life-threatening illnesses are contraindications to the surgical resection of these tumors. WORKUPSection 5 of 9
Imaging Studies
Histologic FindingsGross 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). 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). 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. TREATMENTSection 6 of 9
Surgical therapyMarginal 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. Jones et al (1969) 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 detailsThe 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 PROGNOSISSection 7 of 9
The incidence of local recurrence is high, ranging from 9-44%. Researchers have reported the following rates:
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. 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. MULTIMEDIASection 8 of 9
REFERENCESSection 9 of 9
Giant Cell Tumor of the Tendon Sheath excerpt Article Last Updated: Jan 12, 2007 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
