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GIANT CELL TUMOR OF TENDON SHEATH (XANTHOMA)

Introduction

A giant cell tumor of the tendon sheath (GCTTS) is a slow growing, usually painless benign lesion localized at the extremities. It is the second most common benign tumor of the hand after ganglion cyst.5 Local recurrence after excision is common.1,2.  After surgical excision of xanthomas, the reported recurrence rate is highly variable (4-44%).3,4

Pathophysiology

  • The localized Type I GCTTS consists of synovial-like mononuclear cells, accompanied by a variable number of multinucleate osteoclast-like cells, foam cells, siderophages and inflammatory cells.  Type I GCTTS may be multilobulated but has a signicant pseudocapsule.3
  • The diffuse Type II GCTTS has no or minimal encapsulation, has satellite lesions, has multiple interdigitations of the tumor tissue and has a higher chance of recurrence.4,5
  • There is some controversy as to whether the etiology is neoplastic or non-neoplastic, but is not a malignant lesion and metastases have not been reported.5
  • It is generally believed to be a reactive or regenerative hyperplasia associated with an inflammatory process.

Related Anatomy

  • Most tumors are small (0.5–4cm), well circumscribed, typically lobulated and white to grey with yellowish and brown areas.
  • Approximately 85% of tumors occur in the fingers. Other sites include the wrist, ankle, knee and foot.
  • GCTTS's are more frequently seen on volar aspect of hand; the index finger is the most prevalent location.5
  • Type I tumors (single lesions) are more frequent (78.7%) than type II tumors (≥2 distinct tumors not joined together; 21.3%).1-5

Incidence and Related Conditions

  • Overall incidence is of 1:50,000 individuals.
  • Usually affects individuals between the ages of 30–50 years.5
  • GCTTSs are more common in women than in men (3:2).5
  • GCTTSs can cause bone erosions and reactive bone remodeling but invasion of the intermedullary portion of the bone has not been reported.5
  • The DIP joint is the most frequently involved joint.

Differential Diagnosis3,4,5

  • Haemangioma
  • Glomus tumor
  • Enchondroma
  • Osteoid osteoma
  • Osteoblastoma
  • Giant cell tumors of bone
  • Periosteal chondroma
  • Synovial chondromatosis
  • Schwannoma
  • Fibrolipomatous hamartoma
  • Neurofibromas
  • Mucous cysts
  • Nodular fasciitis
  • Pyogenic granuloma
  • Scar tissue
  • Circumscribed fibromatosis
  • Ganglion
  • Lipoma
  • Fibroma of tendon sheath
  • Foreign body granuloma
  • Tophaceous gout
ICD-10 Codes

  • GIANT CELL TUMOR OF TENDON SHEATH (XANTHOMA)

    Diagnostic Guide Name

    GIANT CELL TUMOR OF TENDON SHEATH (XANTHOMA)

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    GIANT CELL TUMOR OF TENDON SHEATH (XANTHOMA) BENIGN (UPPER LIMB) D21.12D21.11 

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Clinical Photos GCTTS
  • GCTTS right index finger over middle phalanx - two views
    GCTTS right index finger over middle phalanx - two views
  • GCTTS left thumb over neck of proximal phalanx
    GCTTS left thumb over neck of proximal phalanx
  • GCTTS left ring finger over middle phalanx (arrow)
    GCTTS left ring finger over middle phalanx (arrow)
  • GCTTS right index DIP area with dorsal, side and palmar views
    GCTTS right index DIP area with dorsal, side and palmar views
  • GCTTS (arrow) left long finger masquerading as a mucoid cyst.
    GCTTS (arrow) left long finger masquerading as a mucoid cyst.
Pathoanatomy Photos and Related Diagrams
GCTTS Diagrams and Pathoanatomy
  • GCTTS invading PIP joint under cruciate pulley and involving the neuromuscular bundle
    GCTTS invading PIP joint under cruciate pulley and involving the neuromuscular bundle
  • GCTTS invading DIP joint, extensor tendon, collateral ligament and flexor tendon.
    GCTTS invading DIP joint, extensor tendon, collateral ligament and flexor tendon.
  • GCTTS invading thumb IP joint with tumor removed. Note tumor in RCL and radial edge of volar plate. Collateral and edge of volar plate excised. Collateral reconstructed with a small slip of the FPL.
    GCTTS invading thumb IP joint with tumor removed. Note tumor in RCL and radial edge of volar plate. Collateral and edge of volar plate excised. Collateral reconstructed with a small slip of the FPL.
  • Large chronic GCTTS compressing and flattening the middle phalanx (bone).
    Large chronic GCTTS compressing and flattening the middle phalanx (bone).
Symptoms
Complaining of an enlarging mass
Usually painless swelling for many years.
Large GCTTS may cause digital numbness
Cosmetic issues
Cancer concerns
Typical History

The typical patient is a young middle aged female complaining of a painless slowly enlarging bumpy mass that has developed over several years. After local excision, ~15% of cases recur, but are usually non-destructive and are controlled by surgical excision.  However, there is a significant chance of local recurrence.

Positive Tests, Exams or Signs
Work-up Options
Images (X-Ray, MRI, etc.)
X-rays of Fingers with GCTTS
  • GCTTS left ring finger AP X-ray. GCTTS over middle phalanx (arrow)
    GCTTS left ring finger AP X-ray. GCTTS over middle phalanx (arrow)
  • GCTTS left little finger AP and Lateral X-rays. GCTTS over middle phalanx (arrow)
    GCTTS left little finger AP and Lateral X-rays. GCTTS over middle phalanx (arrow)
Treatment Options
Treatment Goals
  • Remove the GCTTS lesion
  • Minimize the chance of recurrence
  • Maintain normal digit and hand function
Conservative
  • None
Operative
  • Complete surgical excision is the treatment of choice, but this is a benign lesion which produces a surgical dilemma. Aggressive excision can decrease the risk of recurrence while increasing the risk of injury to nearby nerves, arteries, tendons, etc.  Ultimately, a gentle yet meticulous excision while using magnification which completely removes the tumor while preserving the other vital structures is the goal.
Treatment Photos and Diagrams
GCTTS Surgical Treatment
  • GCTTS right index finger over middle phalanx. Note digital nerve superficial to the tumor.
    GCTTS right index finger over middle phalanx. Note digital nerve superficial to the tumor.
  • GCTTS right index finger over middle phalanx has been excised. 1 - digital nerve ; 2 - flexor tendon sheath.
    GCTTS right index finger over middle phalanx has been excised. 1 - digital nerve ; 2 - flexor tendon sheath.
  • GCTTS right index finger over middle phalanx specimen.
    GCTTS right index finger over middle phalanx specimen.
  • GCTTS right thumb exposed. Note superficial digital nerve barely visible.
    GCTTS right thumb exposed. Note superficial digital nerve barely visible.
  • GCTTS right thumb almost completely excised. Note ulnar digital nerve (arrow).
    GCTTS right thumb almost completely excised. Note ulnar digital nerve (arrow).
  • GCTTS right thumb excised. 1 - digital nerve; 2 - digital artery; 3 - FPL flexor tendon sheath.
    GCTTS right thumb excised. 1 - digital nerve; 2 - digital artery; 3 - FPL flexor tendon sheath.
  • GCTTS left ring finger over middle phalanx. Again note digital nerve proximity.
    GCTTS left ring finger over middle phalanx. Again note digital nerve proximity.
  • GCTTS right index finger DIP joint. Note digital nerve (arrow) and tumor behind retractor has destroyed the collateral ligament and is entering the DIP joint.
    GCTTS right index finger DIP joint. Note digital nerve (arrow) and tumor behind retractor has destroyed the collateral ligament and is entering the DIP joint.
  • GCTTS right index finger DIP joint involving volar and dorsal parts of the DIP joint.
    GCTTS right index finger DIP joint involving volar and dorsal parts of the DIP joint.
  • GCTTS right index finger DIP joint specimen.
    GCTTS right index finger DIP joint specimen.
  • GCTTS dorsal DIP joint left fifth finger
    GCTTS dorsal DIP joint left fifth finger
  • GCTTS dorsal DIP joint left fifth finger with skin involvement.
    GCTTS dorsal DIP joint left fifth finger with skin involvement.
  • GCTTS recurrent at two levels left thumb.
    GCTTS recurrent at two levels left thumb.
CPT Codes for Treatment Options

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Common Procedure Name
Excision mass finger (includes glomus tumor and Xanthoma)
CPT Description
Excision of benign tumor; deep, subfascial, intramuscular
CPT Code Number
26116
CPT Code References

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Complications
  • Recurrence
  • Infection
  • Temporary numbness
  • Vascular injury
Outcomes
  • Local recurrence after excision is high and the rate is variable (4-44%).4,5
  • Type II tumors are associated with higher recurrence rates.
Key Educational Points
  • GCTTS has been called numerous names: villous arthritis, benign synovioma, fibroxanthoma, fibroma of tendon, fibrous xanthoma, xanthosarcoma, xanthogranuloma, myeloid endothelioma, localizing nodular synovitis, sclerosing hemangioma, tenosynovial giant cell tumour and pigmented villonodular tenosynovitis.3,4 
  • Numerous causes for the development of this tumor have been considered but the cause remains idiopathic i.e. unknown.3 
  • MRI evaluation may help define the extent and interdigitation of a GCTTS.  GCTTS have decreased signal on T-1 and T-2 weighted MRI images.4,5
  • GCTTS recurrence appears to be higher in cases where the tumor involves the extensor tendon, the joint capsule and/or the flexor tendon.4
  • Recurrent GCTTS that involve the joint may require joint arthrodesis. Whether arthrodesis helps prevent further recurrence because of better exposure of the tumor and its satellites or because of the excision of the capsule and synovium is unkown.5
  • Some diffuse GCTTS will extend into the skin and require skin grafting.
  • GCTTS satellites can be very small 1-3mm and the only way to distinguish these lesions from the surrounding fat is the difference in color.
References

Cited and New Articles

  1. Briët J, Becker S, Oosterhoff T, et al. Giant cell tumor of tendon sheath. Arch Bone Jt Surg 2015;3(1):19-21. PMID: 25692164
  2. Lautenbach M, Kim S, Millrose M, et al. Nodular giant cell tumour of the tendon sheath of the hand: analysis of eighty-four cases: diagnostic decisions and outcome. Int Orthop 2013;37(11):2211-5. PMID: 23835561
  3. Lanzinger WD, Bindra R. Giant cell tumor of the tendon sheath. J Hand Surg Am 2013; 38A: 154-157. PMID: 23261194
  4. Williams J, Hodari A, Janevski P, Siddiqui A. Recurrence of giant cell tumor in the hand: a prospective study. J Hand Surg Am 2010; 35A:451-456. PMID: 20193861
  5. Glowacki KA. Giant cell tumors of the tendon sheath.  J Hand Surg Am 2003; 3(2): 100-107.

Reviews

  1. Fotiadis E, Papadopoulos A, Svarnas T, et al. Giant cell tumour of tendon sheath of the digits. A systematic review. Hand (N Y) 2011 Sep;6(3):244-9. PMID: 22942846
  2. Darwish F, Haddad W. Giant cell tumour of tendon sheath: experience with 52 cases. Singapore Med J 2008;49(11):879-82. PMID: 19037553

Classics

  1. Chassaignac M. Cancer de la gaine des tendons. Gazette Hop Civils Mil 1852;47:185-186.
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