* Orthopaedic Department, Hospital Sant Pau, Spain.
** Pathologic Anatomy Department, Hospital Sant Pau, Spain.
*** Radiology Department, Hospital Sant Pau, Spain.

Address for Correspondence
Dr. Ignacio R. Proubasta
Servicio de Cirugía Ortopédica y Traumatología. Hospital Sant Pau.
Barcelona (Spain).
Email:  iproubasta@hsp.santpau.es

This article reports an unusual case of agressive behaviour of a giant-cell  tumor of the tendon sheath involving the thumb that was initially diagnosed as a synovial sarcoma. This clinical form of presentation of GCTTS is extraordinarily rare and perhaps can support the possibility of neoplastic origin.

J.Orthopaedics 2006;3(4)e17

Introduction:

Giant-cell tumor of the tendon sheath (GCTTS) is the second most common tumor of the hand after ganglia 1. Before 1941 the lesion had been described under a variety of names, most of which had the suffic – oma, implying a neoplasm 2, although the most widely held theory is that the disease is an inflammatory reaction of the synovium 3. In recent years, however, the discussion continues since more recent reports, including research into the D.N.A of the cells 4 and immunohistochemically study 5 suggests it to be a neoplastic process.

This article reports an unusual case of agressive behaviour of a giant-cell tumor of the tendon sheath involving the thumb that was initially diagnosed as a synovial sarcoma. This clinical form of presentation of GCTTS is extraordinarily rare and perhaps can support the possibility of neoplastic origin.

Case report

A 76-year-old man presented with a long-standing soft-tissue mass at the volar aspect of his right proximal phalange of the thumb that had recently become increase in size. Physical examination revealed a firm, fixed and diffuse subcutaneous mass, causing limited flexion at the both metacarpophalangeal and interphalangeal joints, but without loss of sensibility.  There was no significant tenderness, and no other nodules were identified. A giant-cell tumor of the profundus flexor tendon sheat was initially suspected and, following radiography when no abnormalities were seen (Fig. 1A), a magnetic resonance imaging (MRI) study was obtained for further evaluation. MRI revealed a diffuse soft-tissue mass arising from the flexor tendon sheat of the finger with numerous little zones of low signal intensity by the presence of hemosiderin,  all  of then  suggestive  of GCTTS (Fig. 1B).

   Figure 1: A) Radiograph showing soft-tissue swelling on the volar surface of the metacarpal bone of the thumb. B) MRI shows hypointense lesion in the volar aspect of the proximal phalange of the thumb, attaching to the flexor tendon. Laboratory data showed no abnormalities. However, in the interval between the first examination and surgery (two weeks) the patient returned to the hospital due to the fast increase of the mass with ulceration of the same over the skin (Fig. 2). Figure 2: Clinical picture of the mass. A) Frontal view. B) Lateral view. Observe the ulceration of the skin and the protrusion of the mass, resembling a malignant tumour.

  Due to the aggressive evolution of the mass and the possibility of being in the presence of synovial sarcoma, a both incisional and tru-cut biopsies were performed. However, histologic examination of the biopsy confirmed the presence of a GCTTS without evidence of malignancy. The patient then underwent resection of this mass through a volar zig-zag approach. Upon dissection through the subcutaneous layer, an infiltrative mass of brownish-yellow tissue was noted around and firmly fixed of the flexor sheat but without connection to the metacarpophalangeal and/or interphalangeal joints and without any osseous involvement of proximal phalange. After the dissection and meticulous liberation of the collateral nerves, the mass was resected until no further tissue was visualized. Due to the non-vascularized skin flaps, the surgical wound it itself open. Later and after the clinical follow-up showed a correct granulation tissue without recurrence of the mass, a free skin graft of the palmar wrist fold was performed. Six months later, the patient recovered with complete relief of symptoms and without evidence of recurrent disease or regrowth of the residual lesion investigated by MR imaging.

Discussion :

GCTTS is usually a firm, nodular, well-defined tumour-like mass occurring commonly on the palmar aspect of the fingers and hand 6. The diagnosis of these lesions should be include physical examination and radiological study. GCTTS commonly manifest as a solitary, painless, palpable mass adherent to the extensor or flexor surface of tendons with a history of size progression. In general, palpation tenderness or pain with movement of the joint are infrequent symptoms. Radiographs show a slightly opaque soft tissue mass, although in some cases were associated with erosions of the adjacent bones 7,8. These erosions have well-defined sclerotic margins and are a sign not of malignancy but of direct extension and pressure effect. Other imaging studies include RMI and sonography. On MRI the lesions are isointense to muscle on T1-weighted imaging and hyperintense to the very low signal seen with ganglion cyst. In some cases they may be slightly hyperintense to muscle because of the deposition of hemosiderin, which also may bloombing on gradient echo imaging, as occurred in our case. On T2-weighted imaging the lesions may be low, intermediate, or high signal, similar to pigmented villonodular synovitis 9. With respect to sonographic findings, GCTTS of the hand typically appear as solid, homogeneous hypoechoic masses with detectable internal vascularity that are associated with the flexor tendons of the fingers 10. Finally, when the diagnosis is controversial, fine needle aspiration cytology o tru-cut biopsies have been found to correlate well with the final diagnosis 11,12.

  When the clinical and radiological diagnosis was stablished, the treatment  of choice is the complete excision. However it is important to distinguish between focal and diffuse forms of GCTTS, because both forms are different rates of recurrence. In this sense and according to Al-Qattan 1, these lesions were classified into two main types, depending on whether the entire tumour was, or was not, surrounded by one pseudocapsule as assessed by the surgeon during surgery. In Type I the entire tumour is surrounded by one pseudo-capsule and in consequence easily to perform a complete excision without recurrences, while in Type II the entire tumour is not surrounded by one pseudo-capsule and for this reason, more difficult to perform a complete excision and, and consequently with a high rate of recurrence 1. In addition, each type was then sub-classified according to the thickness of the capsule, lobulation of the tumour, the presence of satellite lesions, and the diffuse or multicentric nature of the tumour; these factors were also assessed by the surgeon  (Table I).

I. The entire tumour is surrounded by one pseudo-capsule

1. Single nodule with a thick whitish capsule.
2. Single nodule with a thin capsule.
3. Multi-lobulated lesion surrounded by a common pseudo-capsule.

II. The entire tumor is not surrounded by one pseudo-capsule

1. One main nodule (with a pseudo-capsule) accompanied by separate satellite lesions within the same anatomical area.
2. Diffuse type with multiple granular-like lesions with no pseudo-capsule.
3. Multicentric type with separate discrete lesions in the same digit.

Table I: Classification of the giant-cell tumours of tendon sheat (Al-Qattan, 2001).

  In our patient, the tumour was classified as a Type II b, that is, a diffuse tumour type with multiple granular-like lesions with no pseudo-capsule. This type of tumour variant presents a high rate of recurrence after excision 1. In this context, some authors have noted a higher recurrence rate for tumours with increased cellularity and mitotic activity on histological examination, although we could not find a correlation between these histological features and recurrence 13,14,15. Recent research shown that giant cell tumours which are nm 23 negative are more aggressive and are associated with a high recurrence   rate 4. Others significant risk factors for recurrence included presence of adjacent degenerative joint disease, location at the distal interphalangeal joint of the finger or interphalangeal joint of the thumb, although the most important factor for recurrence was the poor surgical technique/incomplete excision of the lesion 1,16,17,18. In these cases in which complete excision may not be possible, radiotherapy may have a role as a prophylaxis against recurrence 17. However, in our case no radiotherapy was employed due to presence of skin graft and the probable possibility of necrosis.

  Regarding the underlying nature of this lesion, specifically whether it is neoplastic or nonneoplastic process, more controversy exist. Since the first description by Chassaignac in1852 , which he called cancer of the tendon  sheat 19, for many years any tumor containing giant cells was considered to be a sarcoma 20.  It was not even 1941 in which Jaffe and coworkers 3 suggested that fibrous xanthoma of synovium and pigmented villonodular synovitis are closely related conditions which are not true neoplasms, but reactions to injury. Since then, many authors indicated that the GCTTS is a nonneoplastic proliferation, if one accepts that a population of cells forming a tumorous mass must show clonality to be classified as a neoplasm 21, while others supports a neoplastic origin 22,23. In general, the natural course of the tumor is bening 24,25 although in our case, the aggressive behaviour lesion it could be labeled as neoplastic.

Reference :

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2. Flandry F, Hughston J. Current concepts review: pigmented villonodular synovitis. J Bone Joint Surg 1987; 69 A: 942-9.

3. Jaffe HL, Lichtenstein L, Sutro CJ. Pigmented Villonodular Synovitis, Bursitis, and Tenosynovitis. A Discussion of the Synovial and Bursal Equivalents of the Tenosynovial Lesion Commonly Denoted as Xantoma, Xanthogranuloma, Giant Cell Tumor or Myeloplaxoma of the Tendon Sheat, with some Consideration of This Tendon Sheat Lesion Itsel. Arch Pathol 1941; 31: 731-65.

4. Abdul-Karim FW, El-Naggar AK, Joyce MJ, Makkley JT, Carter JR. Diffusse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: a clinicopathological and flow cytometric DNA analysis. Human Pathology 1992; 23: 729-35

5. .Ferrer J, Namiq A, Carda C, López-Gines C, Tawfik O, Llombart-Bosch A. Diffuse type of giant-cell tumor of tendon sheat: an ultrastuctural study of two cases with cytogenetic support. Ultrastruct Pathol 2002; 26: 15-21.

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7. Uriburu IJF, Levy VD. Intraosseous growth of giant cell tumors of the tendon sheat (localized nodular tenosynovitis) of the digits: report of 15 cases. J Hand Surg 1998; 23 A: 732-6.

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13. Rodrigues C, desai S, Chinoy R. Giant cell tumor of the tendon sheat: a retrospective study of 28 cases. J Surg Oncol 1998; 68: 100-3.

14. Byers PD, Cotton RE, deacon OW. The diagnosis and treatment of pigmented villonodular synovitis. J Bone Joint Surg 1968; 50 B: 290-305.

15. Rao As, Vigorita Vj. Pigmented villonodular synovitis (giant cell tumor of the tendon sheat and synovial membrane). J Bone Joint Surg 1984; 66 A: 76-94.

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18. Reilly KE, Stern PJ, Dale A. Recurrent giant cell tumors of the tendon sheat. J Hand Surg 1999; 24 A: 1298-302.

19. Chaissagnac M. Cancer de la gaine des tendons. Gaz hosp civ milit 1852 ; 47 : 185-6.

20. Heurtaux MA. Myélome des gaines tendineuses. Arch Gen Med 1891 ; 167 : 40-54.

21. Vogrincic GS, O’Connell JX, Gilks CB. Giant cell tumor of tendon sheat is a polyclonal cellular proliferation. Hum Pathol 1997; 28: 815-9.

22. Nielsen AL, Kiaer T. Malignant cell tumor of synovium and locally destructive pigmented villonodular synovitis: ultrastructural and immunohistochemical study and review of the literature. Hum Pathol 1989; 20: 765-71.

23. Walsh EF, Mechrefe A, Akelman E, Schiller AL. Giant cell tumor of tendon sheat. Am J Orthop 2005; 34: 116-21.

24. Uriburu IJF, Levy VD. Intra-osseous growth of giant cell tumors of the tendon sheat (localized modular tenosynovitis) of the digits: report of 15 cases. J Hand Surg 1998; 23 A: 732-6.

25. shijima M, Hashimoto H, Tsuneyoshi M, Enjoji M. Giant cell tumour of the tendon sheat (nodular tenosynovitis): a study of 207 cases to compare the large joint group with the common digit group. Cancer 1986; 57: 875-84.

This is a peer reviewed paper 

Please cite as : I R Proubasta: Aggressive behaviour of a giant-cell tumor of the flexor tendon sheath of the thumb.

J.Orthopaedics 2006;3(4)e17

URL: http://www.jortho.org/2006/3/4/e17

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