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 Table of Contents  
Year : 2017  |  Volume : 9  |  Issue : 2  |  Page : 115-119

Tenosynovial giant cell tumor of subtalar joint: A case report with review of literature

Department of Orthopedics, Veer Chandra Singh Garhwali Government Medical Science and Research Institute, Srinagar, Pauri Garhwal, Uttarakhand, India

Date of Web Publication14-Dec-2017

Correspondence Address:
Dr. Vipan Kumar
Department of Orthopedics, VCSGMSRI, Srinagar, Uttarakhand
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jotr.jotr_45_15

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Tenosynovial giant cell tumor (TSGCT) are benign neoplasm affecting synovial lining of joints, tendon, and bursae. These tumor have been broadly divided into localized and diffuse type. Localized include TSGCT of tendon sheath and localized pigmented villonodular synovitis (PVNS) located mainly in hand whereas diffuse encompass conventional PVNS and diffuse-type GCT involving one or more joints. In this article, here, we present a case of TSGCT in subtalar joint which has been never reported till date along with review of literature, dilemma in diagnosis, differential diagnosis, and management of this tumor.

Keywords: Benign, excision, giant cell tumor, tenosynovial

How to cite this article:
Krishna D, Kumar V, Chand S, Bhatt V, Yarky A. Tenosynovial giant cell tumor of subtalar joint: A case report with review of literature. J Orthop Traumatol Rehabil 2017;9:115-9

How to cite this URL:
Krishna D, Kumar V, Chand S, Bhatt V, Yarky A. Tenosynovial giant cell tumor of subtalar joint: A case report with review of literature. J Orthop Traumatol Rehabil [serial online] 2017 [cited 2021 Mar 2];9:115-9. Available from: https://www.jotr.in/text.asp?2017/9/2/115/220770

  Introduction Top

Localized tenosynovial giant cell tumor (TSGCT) was described by Chassaignac in 1852, as nodular swelling in flexor tendon of finger.[1] Later, Jaffe et al., in 1941, first coined the term pigmented villonodular synovitis (PVNS) a condition affecting synovial membrane of joints, tendon sheaths, or bursae.[2] TSGCT has been classified into diffuse and localized as described by Lucus 2012. Localized is further subdivided into GCT of tendon sheath (GCTTS) and localized PVNS. Localized PVNS is similar to GCTTS with knee being common location appearing as polypoidal mass arising from synovium. Diffuse type is further subdivided into conventional PVNS and extra-articular diffuse GCT (DGCT).[3]

TSGCT harbor a consistent chromosomal translocation, t(1;2)(p13; q37), which fuses colony-stimulating factor 1 (CSF 1) coding sequences to the promoter of collagen Type VI alpha-3 gene; as a result, the tumor cell overexpresses CSF 1 a chemoattractant for macrophages.[4] Increased M-CSF expression by proliferating cells leads to increased macrophages that expressed macrophage CSF which ultimately leads to the formation of tumor mass.[5] This pathway is being intensely explored and studied for the management of recurrent or unresectable tenosynovial GCT.

Patient usually presents with pain, effusion, and limited range of motion. X-ray usually are normal; magnetic resonance imaging (MRI) definitely helps in diagnosis and the extent of involvement of soft tissue by the tumor. It is recommended that any variety tenosynovial tumor formal histological examination of the lesion and resection should be undertaken. Although good results have been published for arthroscopic treatment particularly of the knee, an open approach allows a more complete resection and potentially reduces the risk of recurrence.

  Case Report Top

Our case is 23-year-old female presented with a complaint of swelling over dorsolateral aspect of the foot for 1 year. She was treated elsewhere by local steroid and aspiration before with no regression of swelling. On local examination, swelling was soft to hard in consistency with no fluctuation, translucency [Figure 1].
Figure 1: A 23-year-old female with swelling over dorsolateral aspect of foot for 1 year

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Movements at ankle and subtalar joint were not restricted. Plain radiograph of foot and ankle showed no significant abnormality [Figure 2].
Figure 2: Oblique X-ray of patient showing no obvious bony abnormality

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Patient was advised MRI but could not afford it; hence, excisional biopsy under spinal anesthesia was planned for the patient. A well-circumscribed mass of dull yellow color with brown pigmentation and irregular margin just below the flexor tendon and muscle mass was found [Figure 3].
Figure 3: Intraoperative photo showing no involvement of extensor tendon

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In transverse section of mass, brown pigmentation present in yellow cheese like bone base [Figure 4] and [Figure 5].
Figure 4: Cheesy white tumor seen with areas of brownish pigmentation and part of involved synovium of subtalar joint

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Figure 5: Tenosynovial tumor arising from subtalar joint with corrugated outer surface and no involvement of extensor tendon

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We thoroughly inspected the origin and surrounding tissue for the remaining part of mass and removed the part of synovium of the subtalar joint. Wound was closed in layers, and compressive bandage with below knee plaster of Paris slab was applied for 1 week. The excised was send for biopsy in two different laboratories having similar report of TSGCT [Figure 6].
Figure 6: Histopathology slide of tenosynovial tumor showing sheets of proliferating cells with macrophages containing hemosiderin and tumor giant cells

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The patient was on regular follow-up in every 3 months for 1 year with last follow-up at 12 months; there was no pain or tenderness present and no sign of recurrence was seen. The patient was advised follow-up every 6 months.

  Discussion Top

GCTTS is the second most common soft tissue tumor of the hand, second only to ganglion.[3] GCTTS involves the volar surface of the fingers more often than the dorsal surface. It is generally indolent and is successfully treated by simple excision. However, it recurs approximately 25% of the time.[6] Patients typically present with a painless mass in the digits or large joints. The lesions are usually well circumscribed and localized and infrequently erode or infiltrate the nearby bone. MRI features T1- and T2-weighted images, which may present with a homogeneous low signal intensity in GCTTS. After intravenous gadolinium administration, marked enhancement is often noted.[7],[8]

Localized PVNS is similar to GCTTS. It is well circumscribed, sometimes pedunculated or sessile polypoidal mass arising from the synovium, with knee being the most common site.

PVNS is rare, with an estimated annual incidence of 1.8 patients per million. It usually affects young adults (average age, 35 years) but has a wide age range and is slightly more common in women. It usually affects large joints, especially the knee,[7] which accounts for 75%–80% of cases presenting with long-standing, painful mass with hemarthrosis and limited range of motion. McMaster et al. indicated the chondrocancellous junction through which the PVNS invaded the bone.[9] Low signal intensity is noted in T1- and T2-weighted sequences with enhancement after gadolinium injection and without enhancement after gadolinium in areas of fibrosis on MRI scan.[7],[10]

Diffuse-type GCT (Dt-GCT) has a similar age distribution, location, and symptoms as PVNS does. The most common sites are the knee, ankle, wrist, and foot. Dt-GCTs form large, firm to spongelike, often clefted masses. Microscopically, they are identical to other forms of TSGCT in its cell population with high recurrence rate following excision surgery, 25% with intra-articular, and 25%–50% with extra-articular disease.[10]

Grossly, tenosynovial tumor is red-brown to mottled orange-yellow. Synovium of diffuse type converted into tangled type with folds and finger projection whereas localized tumors are well circumscribed. All variants are heavily infiltrated by macrophages containing hemosiderin and lipid-filled vacuoles or coalesce into multinucleated giant cell.[11] Synovial membrane shows extensive intimal thickening with villous hypertrophy and hemosiderin deposition. A translocation involving chromosome 1p13, a locus that includes M-CSF gene, has been noted in local and diffuse forms of GCT.[12]

Local lesion tends to involve specific region of synovium whereas diffuse lesion is considered to involve extensive synovium and joint. Inflammatory synovitis is one of the differential diagnoses and can be ruled out on the basis of X-rays showing the preservation of the articular joint space until relatively late in the disease and the absence of periarticular osteopenia. Rheumatoid arthritis and hemophilia can be ruled out on the basis of history. Amyloid arthropathy on the basis of laboratory test and magnetic resonance (MR) finding. Pathognomonic MRI findings of synovial hemangioma consist of a lobulated intra-articular mass with marked hyperintensity on T2-weighted MRI reflecting blood within vascular spaces in combination with septae and serpiginous vascular structures.

Desmoid-type fibromatosis is usually centered in an intermuscular location with irregular infiltrative margins with extension along fascial planes (fascial tail sign).[13] Tubercular tenosynovitis compound ganglion present with dumbbell-shaped, fluctuant swelling and painful movement of involved tendon.

Multiple treatment options are available with surgical option the most favored one. Arthroscopic synovectomy is a viable option at the cost of high recurrence rate with decreased postoperative pain and stiffness of joint.[14] Either partial or complete arthroscopic synovectomy can be performed, dependent on disease extension. Lesion localized to knee complete synovectomy should be preferred over partial because of high recurrence rate.[14],[15],[16] In Arthroscopic synovectomy for diffuse lesion, there is high possibility of leaving tumor cells behind thus increasing high recurrence rate.

Open synovectomy gives lesser recurrence rate and complete removal of tumor is possible.[13] Open approach can be considered for all joints; local disease has low recurrence rate hence both open or arthroscopic approach can be applied, with still higher weightage for open synovectomy and removal of tumor. Chiari et al., Brien et al. presented with zero percent recurrence for foot and ankle TSGCT when treated with open synovectomy.[17],[18] For diffuse lesions about the knee, (two-stage) open synovectomy with a separate anterior and posterior approach is reported to be a safe surgical treatment option.[19],[20]

Intra-articular radioactive isotope such as 90-yttrium into the joint can be safe adjuvant after subtotal or total synovectomy with variable result on recurrence.[21],[22] Ozturk et al. performed open partial synovectomy with instillation of 90-yttrium in knee joint reported zero recurrence in four patients.[21] Chin et al. reported 17% recurrence rate with complete synovectomy of knee with dysprosium-165 instillation in thirty patients.[23] Clinical impact of local dose of radioactive isotope on surrounding normal tissue still needs to investigated along with evaluation of result of treatment.

External beam radiation can be a primary treatment in unresectable disease or an adjuvant treatment in incompletely resectable or recurrent DGCT.[24],[25],[26] Moderate dose external beam radiation with total of 35 Gy offers a high chance of local control with avoidance of long-term radiotherapy-induced toxicity, and radiotherapy is not advocated in the joints of hand and foot.

Systemic therapy in from imatinib, emactuzumab is a novel treatment approach, as these drugs prevent overexpression of CSF 1 which is commonly seen with tenosynovial tumors.[27],[28] Most common side effects reported were nausea, fluid retention, and skin changes. In 2008, the first case report of the activity of imatinib in recurrent M-CSFR-dependent Dt-GCT was published. The patient was treated with imatinib 400 mg/day, and a complete response was induced after 5 months of therapy. Following discontinuation of imatinib, the disease recurred. Reintroduction induced a secondary complete remission.[29] We here present in tabulated form of results of all treatment options available [Table 1].
Table 1: Results of various treatment options available for management of tumor

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  Conclusion Top

This case is reported as rare as it has been never documented arising from subtalar joint. On reviewing the literature, we believe that localized GCT should be treated with open synovectomy whereas DGCT preferred approach should be open synovectomy with complete removal of tumor and regular follow as there are high chances of recurrence. In unresectable disease, it is recommended that radical resection and joint reconstruction should be done followed by radiotherapy. Systemic therapy such as imatinib can be considered for unresectable tumor where radiotherapy cannot be administered. In our case, complete removal of tumor with regular follow-up up to 12 months no recurrence with good result was achieved.[36]

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Conflicts of interest

There are no conflicts of interest.

  References Top

Chassaignac M. Cancer de la gaine des tendons. Gaz Hosp Civ Milit. 1852;47:185-6.  Back to cited text no. 1
Jaffe HL, Lichtenstein L, Sutro CJ. Pigmented villonodular synovitis, bursitis and tenosynovitis. A discussion of synovial and bursal equivalents of the tenosynovial lesion commonly denoted as xanthorna, xanthogranuloma, giant cell tumor or myeloplaxoma of the tendon sheath, with some consideration of this tendon sheath lesion itself. Arch Pathol 1941;31:731-65.  Back to cited text no. 2
Lucas DR. Tenosynovial giant cell tumor: Case report and review. Arch Pathol Lab Med 2012;136:901-6.  Back to cited text no. 3
Möller E, Mandahl N, Mertens F, Panagopoulos I. Molecular identification of COL6A3-CSF1 fusion transcripts in tenosynovial giant cell tumors. Genes Chromosomes Cancer 2008;47:21-5.  Back to cited text no. 4
West RB, Rubin BP, Miller MA, Subramanian S, Kaygusuz G, Montgomery K, et al. A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc Natl Acad Sci U S A 2006;103:690-5.  Back to cited text no. 5
Reilly KE, Stern PJ, Dale JA. Recurrent giant cell tumors of the tendon sheath. J Hand Surg Am 1999;24:1298-302.  Back to cited text no. 6
Murphey MD, Rhee JH, Lewis RB, Fanburg-Smith JC, Flemming DJ, Walker EA. Pigmented villonodular synovitis: Radiologic-pathologic correlation. Radiographics 2008;28:1493-518.  Back to cited text no. 7
Ushijima M, Hashimoto H, Tsuneyoshi 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 1986;57:875-84.  Back to cited text no. 8
McMaster P. Pigmented villonodular synovitis with invasion of bone. J Bone Joint Surg 1960;42:1170-83.  Back to cited text no. 9
Schwartz HS, Unni KK, Pritchard DJ. Pigmented villonodular synovitis. A retrospective review of affected large joints. Clin Orthop Relat Res 1989;247:243-55.  Back to cited text no. 10
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Brandal P, Bjerkehagen B, Heim S. Molecular cytogenetic characterization of tenosynovial giant cell tumors. Neoplasia 2004;6:578-83.  Back to cited text no. 12
van der Heijden L, Gibbons CL, Hassan AB, Kroep JR, Gelderblom H, van Rijswijk CS, et al. A multidisciplinary approach to giant cell tumors of tendon sheath and synovium – A critical appraisal of literature and treatment proposal. J Surg Oncol 2013;107:433-45.  Back to cited text no. 13
Zvijac JE, Lau AC, Hechtman KS, Uribe JW, Tjin-A-Tsoi EW. Arthroscopic treatment of pigmented villonodular synovitis of the knee. Arthroscopy 1999;15:613-7.  Back to cited text no. 14
Ogilvie-Harris DJ, McLean J, Zarnett ME. Pigmented villonodular synovitis of the knee. The results of total arthroscopic synovectomy, partial, arthroscopic synovectomy, and arthroscopic local excision. J Bone Joint Surg Am 1992;74:119-23.  Back to cited text no. 15
De Ponti A, Sansone V, Malcherè M. Result of arthroscopic treatment of pigmented villonodular synovitis of the knee. Arthroscopy 2003;19:602-7.  Back to cited text no. 16
Chiari C, Pirich C, Brannath W, Kotz R, Trieb K. What affects the recurrence and clinical outcome of pigmented villonodular synovitis? Clin Orthop Relat Res 2006;450:172-8.  Back to cited text no. 17
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O'Sullivan B, Cummings B, Catton C, Bell R, Davis A, Fornasier V, et al. Outcome following radiation treatment for high-risk pigmented villonodular synovitis. Int J Radiat Oncol Biol Phys 1995;32:777-86.  Back to cited text no. 24
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

  [Table 1]


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