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Giant Cell Tumor of Tendon Sheath

DEFINITION AND PATHOGENESIS

  • Aka "benign synovioma"---no relationship to synovial sarcoma; tenosynovial giant cell tumor, localized type, nodular tenosynovitis, fibrous histiocytoma of synovium, fibrous xanthoma of synovium, pigmented nodular synovitis)
  • First described by Chassaignac as "cancer of tendon sheath"
  • Unclear as to etiology
  • Classification
    • Localized form
    • Intraarticular form (most in the infrapatellar fat)
    • Diffuse form (florid proliferative synovitis, extraarticular PVNS)

IMPORTANCE

  • Most common neoplasm of the hand

CLINICAL FEATURES

  • Most common between 30-50 years, F>M, 20-40 yrs in diffuse form
  • Develop over a long period, often without change for several years
  • Fixed to deep structures
  • Distal lesions adjacent to IPJs
  • Has been the reported cause of osteomalacia in a single pt
  • Compressive neuropathy reported
    • Carpal tunnel syndrome
    • Ulnar tunnel syndrome at Guyon's canal
  • Reported to be a cause of trigger wrist
  • Reported as a cause of hallux valgus with EDL involvement
  • Reported at the site of a previous tendon laceration

RADIOLOGIC FEATURES

  • Circumscribed soft tissue mass in ½ of cases
  • 10-11% with cortical erosion or perforation (more common in lesions of the feet)
  • US
    • Hypoechoic nodules with homogeneous or heterogeneous echogenicity
  • MRI
    • T1-weighted images: muscle isointensE
    • T2-weighted images: hypointense with black regions (hemosiderin)
    • ? homogeneous enhancement
  • + uptake on FDG-PET scan often seen when staging is done for a malignant dx

GROSS PATHOLOGY

  • Lobulated, 0.5-4cm in size, larger lesions on the feet
  • Mottled cut surface, pink to gray with yellow-brown areas of lipid and hemosiderin

HISTOLOGIC FEATURES

  • Dense capsule continuous with nodule septae does not totally contain tumor, especially where it is adjacent to synovial membrane
  • Moderately cellular with sheets of rounded to polygonal cells
  • Multinucleated giant cells numerous to sparse (esp. In recurrent lesions)
  • Formed by fusion of mononuclear cells with 3-60 nuclei
  • Xanthoma cells contain hemosiderin granules
  • Hyalinization, and cartilaginous/osseous metaplasia rarely found
  • Mitotic figures in 50%, with ? 3/hpf in > 10%
  • Tumor thrombi in1-5% within small veins (does not indicate malignancy)
  • Immunohistochemistry
    • nm23 expression (antimetastasis and antiinvasion gene) associated with a significant risk of LR
    • p63 expression
  • EM: both Type A (synovial cell) and Type B (fibroblastic cell) synovial membrane cell types present
    • Cell surface markers suggest osteoclastic lineage
  • Flow cytometry: diploid pattern in 50% of localized form, aneuploid pattern in 50% of diffuse form

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

  • Foreign body granuloma (more inflammatory cells)
  • Necrobiotic granuloma (degenerating collagen rimmed by histiocytes and proliferating capillaries)
  • Tendinous xanthoma (hyperlipidemia with intratendinous xanthomas, "cholesterol clefts")
  • Fibroma of tendon sheath
  • Calcifying aponeurotic fibroma
  • Epithelioid sarcoma with numerous giant cells (+ keratin)

DISEASE COURSE AND TREATMENT

  • 10-20% locally recur, possibly higher with longer followup
  • Possibly related to more cellular lesions with ? mitoses
  • More common intralesional excisions, where tumor is left at deep margins
  • 20 Gy in 10 divided doses has been reported to ? LR in selected pts (no long term FU however)
  • Local excision with a small cuff of normal tissue
  • Surface mold brachytherapy has been used after resection
  • Amputation of digit for extensive disease may be required

SPECIAL CONSIDERATION

  • TENOSYNOVIAL GIANT CELL TUMOR, DIFFUSE TYPE

    • (Proliferative Synovitis, Florid Synovitis, Extraarticular Pig-mented Villonodular Synovitis, Pigmented Villonodular Bursitis)
    • PVNS or GCTTS when it occurs extraarticularly or within a bursal sac, arising from the synovium of a tendon sheath or a bursa
    • ½ of pts <40yrs of age, F>M (slightly)
    • Sxs often of long duration include pain and local tenderness
    • X-rays may show adjacent osteopenia, cortical erosion
    • Bone scan may show ? soft tissue uptake
  • MULTIFOCAL GIANT CELL TUMOR OF TENDON SHEATH

    • Multifocal involvement of the same tendon in different locations has been described
    • Bilateral thumb involvement has been reported

REFERNECES

Abdul-Karim FW, El-Naggar AK, Joyce MJ, Makley JT, Carter JR: Diffuse and localized tenosynovial giant cell tumour and pigmented villonodular synovitis: a clinicopathologic and flow cytometric: DNA analysis. Human Pathology 23:729-35,1992.

Alguacil-Garcia A, Unni KK, Goeliner JR: Giant cell tumor of tendon sheath and pigmented villonodular synovitis, an ultrastructural study. Am J Clin Pathol 69:6-17,1978.
Al-Qattan MM: Giant cell tumours of tendon sheath: classification and recurrence rate. J Hand Surg 26:72-5,2001.Attaykan A, Yildiz K, Hapa O, Cukur S: Multifocal giant cell tumor of the tendon sheath occurring at different localizations of the same tendon of a finger: a case report and review of the literature. Eklem Hastalik Cerrahisi 20:119-23,2009.

Anazawa U, Hanaoka H, Shiraishi T, Morioka H, Morii T, Toyama Y: Similarities between giant cell tumor of bone, giant cell tumor of tendon sheath, and pigmented villonodular synovitis concerning ultrastructural cytochemical features of multinucleated giant cells and mononuclear stromal cells. Ultrastruct Pathol 30:151-8,2006.

Assopardi JG, Tanda F, Saim R: Tenosynovial fibroma. Diagn Histopathol 6:69-76,1983.

Baatra VV, Jain S, Singh DK, Kumar N: Cytomorphologic spectrum of giant cell tumor of tendon sheath. Acta Cytol 52:152-8,2008.

Bancroft LW, Peterson JJ, Kransdorf MJ: Imaging of soft tissue lesions of the foot and ankle. Radiol Clin North Am 46:1093-103,2008.

Buggiani FP, Rutan GM, Holt WP: Giant cell tumor of the tendon sheath: a case report. J Am Podiatry Assoc 71:166-7,1981.

Carstens HB: Giant cell tumors of tendon sheath. An electron microscoica; study of 11 cases. Arch Pathol Lab Med 102:88-103,1978.

Chalmers RL, Mandalia M, Contreras R, Schreuder F: Acute trigger wrist and carpal tunnel syndrome due to giant-cell tumour of the flexor sheath. J Plast Reconstr Aesthet Surg 61:1557,2008.
Chassaignac CME: Cancer de la gaine des tendons. Gax Hosp Civ Milit 47:185,1852.

Chou LB, Ho YY, Malawer MM: Tumors of the foot and ankle: experience with 153 cases. Foot Ankle Int 30:836-41,2009.

Collen J, Mount G, Pollock P, Shrout J, Malafronte P, Vangeertruyden P, Oglesby R: Giant cell tumor of the tendon sheath. J Clin Rheumatol 15:85-7,2009.

Coster AA: Giant cell tumor of tendon sheath (benign synovioma). J Am Podiatry Assoc 66:538-41,1976.

Dalai S, Bostock SH: Bilateral symmetrical giant cell tumours of the thumb. Hand Surg 8:237-8,2003.

Dandia SD, Pendse AK, Singh H, Bharadwaj TP: Giant cell tumor of tendon sheath---a case report. Indian J Pathol Bacteriol 14:189-91,1971.

Darwish FM, Haddad WH: Giant cell tumour of tendon sheath: experience with 52 cases. Singapore Med J 49:879-82,2008.

De Leonardis F, La Corte R, Brushi M, Cavazzini L, Trotta F: Wegener's granulomatosis and giant cell tumor of tendon sheaths, casual or causal combination? First case report. Rheumatol Int 29:1105-7,2009.

De Schepper AM, Hogendoorn PC, Bloem JL: Giant cell tumors of the tendon sheath may present radiologically as intrinsic osseous lesions. Eur Roadiol 17:499-502,2007.

Dickson BC, Li SQ, Wunder JS, Ferguson PC, Eslami B, Werier JA, Turcotte RE, Kandel RA: Giant cell tumor of bone express p63. Mod Pathol 21:369-75,2008.
Dingle SR, Flynn JC, Flynn JC Jr, Stewart G: Giant-cell tumor of the tendon sheath involving the cervical spine. A case report. J Bone Joint Surg 84A:1664-7,2002.
Eisenstein R: Giant-cell tumor of tendon sheath. Its histogenesis as studied in the electron microscope. J Bone Joint Surg 50:476-86,1968.

Erler K, Demiralp B, Ozdemir MT, Kaya A, Basbozkurt M: Giant cell tumor of tendon sheath simulating giant cell tumor of bone: report of a case. J Surg Orthop Adv 13:124-7,2004.
Ferrer J, Namiq A, Carda C, Lopez-Gines C, Tawfik O, Llombart-Bosch A: Diffuse type of giant-cell tumor of tendon sheath: an ultrastructural study of two cases with cytogenetic support. Ultrastruct Pathol 26:15-21,2002.
Fogelson MH, Dao KD, Shin AY: Intraosseous metacarpal involvement of giant cell tumor of the tendon sheath: report of 2 cases. Am J Orthop 32:32-4,2003.

Francisco BS, Agarwal JP: Giant cell tumor of tendon sheath in Guyon's canal causing ulnar tunnel syndrome. A case report and review of the literature. Eplasty 9:e8,2009.
Furco A, Roger M, Mouchet B, Richard O, Martinache X, Fur A: Osteomalacia cured by surgery. Eur J Intern Med 13:67-9,2002.

Gholve PA, Hosalkar HS, Kreiger PA, Dormans JP: Giant cell tumor of tendon sheath; largest single series in children. J Pediatr Orthop 27:67-74,2007.
Gibbons CL, Khwaja HA, Cole AS, Cooke PH, Athanasou NA: Giant-cell tumour of the tendon sheath in the foot and ankle. J Bone Joint Surg 84:1000-3,2002.
Glowacki KA, Weiss A-PC: Giant cell tumors of tendon sheath. Hand Clin 11:245-53,1995.

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 8:79-83,2009.
Grover R, Grobbelaar AO, Richman PI, Smith PJ: Measurement of invasive potential provides an accurate prognostic marker for giant cell tumour of tendon sheath. J Hand Surg 23B:728-34,1998.
Gupta K, Dey P, Goldsmith R, Vasishta RK: Comparison of cytologic features of giant-cell tumor and giant-cell tumor of tendon sheath. Diagn Cytopathol 30:14-8,2004.

Guryel E, Coleridge S, Bendall S: Unusual presentation of a giant cell tumor of the tendon sheath in the foot. J Surg Orthop Adv 13:110-1,2004.
Hampton PJ, Leonard N, Lawrence CM: Case 5. Giant-cell tumor of tendon sheath. Clin Exp Dermatol 28:573-4,2003.
Hansen T, Petrow PK, Gaumann A, Keyszer GM, Otto M, Kirkpatrick CJ, Kriegsmann J: Expression of cysteine proteinases cathepsins B and K and of cysteine proeinase inhibitor cystatin C in giant cell tumor of tendon sheath. Mod Pathol 14:318-24,2001.

Hayes CW Jr: Ulnar tunnel syndrome from giant cell tumor of tendon sheath: a cse report. J Hand Surg 3A:187-8,1978.
Hitora T, Yamamoto T, Akisue T, Marui T, Nagira K, Ohta R, Yoshiya S, Kurosaka M: Multicentric localized giant cell tumour of the tendon sheath: two separate lesions at different sites in a finger. Br J Dermatol 147:403-5,2002.
Hosaka M, Hatori M, Smith RA, Kokubun S: A cell line with multinucleated giant cell formation established from a human giant cell tumor of tendon sheath--preliminary report. J Orthop Sci 6:414-8,2001.

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 9:581-4,2004.

Hulse N, Watson SJ: Giant cell tumour of tendon sheath in a child: a case report. Hand Surg 10:97-100,2005.

Ikeda K, Osamura N, Tomita K: Giant cell tumour in the tendon sheath of the hand; importance of the type of lesion. Scan J Plast Reconstr Surg Hand Surg 41:136-42,2007.
Iyer VK, Kapila K, Verma K: Fine-needle aspiration cytology of giant cell tumor of tendon sheath. Diagn Cytopathol 29:105-10,2003.
Jelinek JS, Kransdorf MJ, Shmookler BM, Aboulafia AA, Malawer MM: Giant cell tumor of the tendon sheath: MR findings in nine cases. AJR 162:919-22,1994.
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 51A:76-86,1969.

Kandamany N, Mahaffey PJ: Diffuse-type giant cell tumour of the tendon sheath of the proximal forearm. J Plast Reconstr Aesthet Surg 62:556-7,2009.
Khanduja V, Dannawi Z, Ng L, El-Zebdeh M: Giant-cell tumor of the tendon sheath arising from the patellar tendon. J Knee Surg 17:28-9,2004.
Kitagawa Y, Ito H, Amano Y, Sawaizumi T, Takeuchi T: MR imaging for preoperative diagnosis and assessment of local tumor extent on localized giant cell tumor of tendon sheath. Skeletal Radiol 32:633-8,2003.

Kitagawa Y, Ito H, Yokoyama M, Sawaizumi T, Maeda S: The effect of cellular proliferative activity on recurrence and local tmour extent of localized giant cell tumour of tendon sheath. J Hand 29B:604-7,2004.
Kotwall PP, Gupta V, Malhotra R: Giant-cell tumour of the tendon sheath. J Bone Joint Surg 82B:571-3,2000.
Kruithof E, Baeten D, Dierckxsens P, Veys EM, De Keyser F: Tenosynovitis with acute onset: unexpected giant cell tumour of the tendon sheath. Ann Rheum Dis 61:847,2002.

Kuo CL, Yang SW, Chou YJ, Wong CY: Giant cell tumor of the EDL tendon sheath; an unusual cause of hallux valgus. Foot Ankle Int 29:534-7,2008.
Llauger J, Palmer J, Rosón N, Cremades R, Bagué S: Pigmented villonodular synovitis and giant cell tumors of the tendon sheath: radiologic and pathologic features. AJR 172:1087-91,1998.
Looi KP, Low CK, Yap YM: Pigmented villonodular synovitis of the hand in the Asian population. Hand Surg 4:81-5,1999.

Lorea P, Van De Walle H, Kinnen L, Ledoux P, Moemans JP, Van Den Heule B: Giant cell tumours of the tendon sheath: lack of correlation between nm23-H1 expression and recurrence. J Hand Surg 29B:67-70,2004.

Lu CT, Chen HC, Coskunfirat OK: Immediate toe transfer following index finger amputation for extensive giant cell tumor of the tendon sheath with infraosseous invation. Chang Gung Med J 27:312-7,2004.

Lu KH: Arthroscopic excision of juxta-articular giant-cell tumor arising from the patellar tendon sheath. Arthroscopy 20:eE35-8,2004.
Mackie GC: Pigmented villonodular synovitis and giant cell tumor of the tendon sheath: scintigraphic findings in 10 cases. Clin Nucl Med 28:881-5,2003.

Maheshwari AV, Muro-Cacho CA, Pitcher JD Jr: Pigmented villonodular bursitis/diffuse giant cell tumor of the pes anserine bursa: a report of two cases and review of literature. Knee 14:402-7,2007.
McClain EJ, Wissinger HA: The acute carpal tunnel syndrome: nine case reports. J Trauma 16:75-8,1976.

Middleton WD, Patel, V, Teefey SA, Boyer MI: Giant cell tumors of the tendon sheath: analysis of sonographic findings. AJR 183:337-9,2004.
Monaghan H, Salter DM, Al-Nafussi A: Giant cell tumour of tendon sheath (localised nodular tenosynovitis): clinicopathological features of 71 cases. J Clin Pathol 54:404-7,2001.
Moore JR, Weiland AJ, Curtis RM: Localized nodular tenosynovitis: experience with 115 cases. J Hand Surg 9A:412-7,1984.

Muramatsu K, Mine T, Ichihara K: Atypical tenosynovial giant cell tumor of the extensor hallucis longus tendon. J Am Podiatr Med Assoc 96:359-61,2006.
Nakashima M, Uchida T, Tsukazaki T, Hamanaka Y, Fukuda E, Ito M, Sekine I: Expression of tyrosine kinase receptors Tie-1 and Tie-2 in giant cell tumor of the tendon sheath: a possible role in synovial proliferation. Pathol Res Pract 197:101-7,2001.

Napier R, McCormack J: Giant cell tumour of the tendon sheath---an unusual cause for locking of the knee joint. Ulster Med J 77:130:2009.
Nilsson M, Hoglund M, Panagopoulos I, Sciot R, Dal Cin P, Debiec-Rychter M, Mertens F, Mandahl N: Molecular cytogenetic mapping of recurrent chromosomal breakpoints in tenosynovial giant cell tumors. Virchows Arch 441:475-80l,2002.

Occhipinti E, Heinrich SD, Craver R: Giant cell tumor of tendon sheath arising in the toe. Fetal Pediatr Pathol 23:171-9,2004.
O'Sullivan B, Cummings B, Catton C, Bell R, Davis A, Fornasier V, Goldberg R: Outcome following radiation treatment for high-risk pigmented villonodular synovitis. Int J Radiat Oncol Biol Phys 32:777-86,1995.

Oyemade GA, Abioye AA: A clinicopathologic review of benign giant cell tumors of tendon sheaths in Ibadan, Nigeria. Am J Surg 134:392-5,1977.

Park JW: Multiple separated giant cell tumors of the tendon sheath in a thumb. J Am Acad Dermatol 54:540-2,2006.

Parker WL, Beckenbaugh RR, Amrami KK: Caclifying aponeurotic fibroma of the hand; radiologic differentiation from giant cell tumors of the tendon sheath. J Hand Surg 31A:1024-8,2008.
Peh WC, Shek TW, Ip WY: Growing wrist mass. Ann Rheum Dis 60:550-3,2001.
Peh WC, Wong Y, Shek TW, Ip WY: Giant cell tumour of the tendon sheath of the hand: a pictorial essay. Australas Radiol 45:274-80,2001.
Phalen GS, McCormack LJ, Gazale WJ: Giant-cell tumor of tendon sheath (benign synovioma) in the hand. Evaluation of 56 cases. Clin Orthop 15:140-51,1959.
Rao AS, Vigorata VJ: Pigmented villonodular synovitis (giant cell tumour of the tendon sheath and synovial membrane). J Bone Joint Surg 66A:76-94,1984.
Reilly KE, Stern PJ, Dale JA: Recurrent giant cell tumors of the tendon sheath. J Hand Surg 24A:1298-1302,1999.
Relwani J, Factor D, Khan F, Dutta A: Giant cell tumour of the patellar tendon sheath--an unusual cause of anterior knee pain: a case report. Knee 10:145-8,2003.

Roberts CC, Liu PT, Chew FS: Imaging evaluation of tendon sheath disease: self-assessment module. AJR 188(3 Suppl):S10-12,2007.
Rodrigues C, Desai S, Chinoy R: Giant cell tumor of the tendon sheath: a retrospective study of 28 cases. J Surg Oncol 68:100-3,1998.
Rosa MA, Galli M, Fadda G, Maggiano N, Gambino GF: Proliferating cell nuclear antigen labelling index in localised pigmented villonodular synovitis and its relationship to the size of nodules. Int Orthop 24:197-201,2000.
Rowlands CG, Roland B, Hwang WS, Sevick RJ: Diffuse-variant tenosynovial giant cell tumor: a rare and aggressive lesion. Hum Pathol 25:423-5,1994.
Savage RC, Mustafa EB: Giant cell tumors of tendon sheath (localized nodular tenosynovitis). Ann Plastic Surg 13:205- ,1984.
Scott SJ, Jenkinson MD: Giant-cell tumour of the tendon sheath. J Bone Joint Surg 82B:1206,2000.
Sherry CS, Harms SE: MR evaluation of giant cell tumors of tendon sheath. J Magn Reson Imaging 7:195- ,1989.
Skaliczki G, Mady F: Giant cell tumor of the tendon sheath of the toe imitating macrodactyly: case report. Foot Ankle Int 24:868-70,2003.
Uriburu IJF, Levy VD: Intraosseous growth of giant cell tumors of the tendon sheath (localized nodular tenosynovitis) of the digits: report of 15 cases. J Hand Surg 23A:732-6,1998.
Ushijimo M, Hashimoto H, Tsvneyoshi M, Enjoji M: Giant cell tumor of the tendon sheath (nodular tenosynovitis): a study of 207 cases to compare the large joint group with the common digit group. Cancer 57:875-84,1986.
Vardakas DG, Musgrave DS, Goebel F, Sotereanos DG: A large, multiply recurrent tenosynovial giant cell tumor of the hand treated with resection and radiation therapy. Am J Orthop 33:137-40,2004.

Vasconez HC, Nisanci M, Lee EY: Giant cell tumour of the flexor tendon sheath of the foot. J Plast Reconstr Aesthet Surg 61:815-8,2008.

Walsh EF, Mechrefe A, Akelman E, Schiller AL: Giant cell tumor of tendon sheath. Am J Orthop (Belle Mead NJ) 23:116-21,2005.

Wang Y, Tang J, Luo Y: The value of sonography in diagnosing giant cell tumors of tendon sheath. J Ultrasound Med 26:1333-40,2007.

Ward CM, Lueck NE, Steyers CM: Acute carpal tunnel syndrome caused by diffuse giant cell tumor of tendon sheath: a case report. Iowa Orthop J 27:99-103,2007.

Weinstock RE: Giant cell tumor of tendon sheath origin (benign synovioma)--a case report. J Am Podiatry Assoc 52:903-5,1962.
Weinzweig J, Watson HK: Recurrent giant cell tumor over a 23-year period. Ann Plast Surg 39:659-61,1997.

Wissmeyer M, Kohl S, Juengling FD, Stauffer E, Siebenrock KA, Krause TM: FDG uptake in giant cell tumor of the tendon sheath in a patient restaged for gastrointestinal stroma tumor (GIST). Clin Nucl Med 34:193-8,2009.

Wittels M, Ghandur-Mnaymneh L, Mnaymneh W: Giant cell tumor of tendon sheath developing at the site of tendon laceration. Clin Orthop 169:207-10,1982.
Wright CJE: Benign giant cell synovioma: an investigation of 85 cases. Br J Surg 38:257-71,1951.

Youngleson J, Malkin C: Hypertrophic pigmented villonodular tumour of the tendon sheath. A case report. S Afr J Surg 3:109-16,1965.
Zook EG: Extensive giant cell tumor of the finger: a case history. J Hand Surg 2A:267-8,1977.

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