tenosynovial giant cell tumor
Image Gallery
Definition: Tenosynovial giant cell tumor (TGCT) or Giant cell tumor of tendon sheath (GCTTS)is the most common benign tumor of synovium and tendon sheath. Immunophenotypical evidence suggests it is of synovial cell origin. There is controversy regarding the underlying nature of this lesion, specifically whether it is a neoplastic or nonneoplastic (ie, reactive or hyperplastic) process.
Synopsis
sharply circumscribed
lobulation by fibrous septa
round or polygonal histiocytic or fibrohistiocytic cell
giant cells (multinucleated histiocytes) usually present
+/- osteoclastic type giant cells
foamy histiocytes
hemosiderin laden histiocytes
Types
localized TGCT (nodular tenosynovitis)
diffuse TGCT (pigmented villonodular synovitis)
Immunochemistry
histiocytic cells
multinucleate cells
- CD68+
- LCA+
Differential diagnosis
foreign body granuloma
necrobiotic granuloma
tendinous xanthoma
fibroma of the tendon sheath
epithelioid sarcoma
biphasic synovial sarcoma
Cytogenetics
1p11 to 1p13.2 structural rearrangements (8780753, 12447678)
- t(1;1)(q21;p11) (1q21 and 1p11) (15548367)
- t(1;2)(p11;q35-36) (1p11 and 2q35-q36) (8033128)
- t(1;2)(p13;q37) (15768862)
- t(1;5)(p11;q22) (1p11 amd 5q22) (8033128)
- t(1;19)(p11;p12) (1p11 and 19p12) (7685623)
- t(1;22)(p13;q12) (1p13 and 22q12) (15548367)
- t(1;2)(pter->p22::q24->pter) (7631554)
- t(1;14)(qter->p13::q13->ter) (7631554)
1q21 structural rearrangements
2q33-2q35-2q36-2q37 structural rearrangements
t(2;3) (12028654)
t(8;12) (12028654)
16q24 structural rearrangements
- translocation partner
- 2q35-37
- 5q22-31
- 8q21-22
- 11q11-12
other translocations
CGH (15766598)
| Gains | 16p | 16q | 22q |
Molecular biology
Prognosis
benign
local recurence rate: 10%
References
Brandal P, Bjerkehagen B, Heim S. Molecular cytogenetic characterization of tenosynovial giant cell tumors. Neoplasia. 2004 Sep-Oct;6(5):578-83. PMID: 15548367 (Full text free)
Occhipinti E, Heinrich SD, Craver R.Giant cell tumor of tendon sheath arising in the toe.Fetal Pediatr Pathol. 2004 Mar-Jun;23(2-3):171-9. PMID: 15768862
Nilsson M, Höglund 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. 2002 Nov;441(5):475-80. PMID: 12447678
Ferrer J, Namiq A, Carda C, López-Ginés 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. 2002 Jan-Feb;26(1):15-21. PMID: 12028654
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. 2002 Nov;441(5):475-80. PMID: 12447678
Rowlands CG, Roland B, Hwang WS, Sevick RJ. Diffuse-variant tenosynovial giant cell tumor: a rare and aggressive lesion. Hum Pathol. 1994 Apr;25(4):423-5. PMID: 8163276