Mateusz Gładysz FESSH prep

FESSH prep Hand tumours Localised tenosynovial giant cell tumour: presentation, MRI features and recurrence; enchondroma of the hand: site, curettage and IDH mutations

Giant cell tumour of tendon sheath and enchondroma of the hand

Last reviewed v1.0

FESSH-041Hand tumours●○○ basic

Regarding giant cell tumour of tendon sheath and enchondroma of the hand:

  1. 1.Giant cell tumour of tendon sheath is the second most common soft-tissue tumour of the hand after the ganglion cyst and typically presents as a firm, non-tender, slowly enlarging nodule, most often on the volar aspect of a finger.

  2. 2.On MRI, giant cell tumour of tendon sheath characteristically shows low signal intensity on both T1- and T2-weighted sequences because of haemosiderin and dense collagen, and enhances markedly after gadolinium.

  3. 3.Local recurrence after marginal excision of giant cell tumour of tendon sheath is rare, occurring in fewer than 2 per cent of cases.

  4. 4.Enchondromas of the hand most commonly arise in the distal phalanx.

  5. 5.Somatic mutations of IDH1 or IDH2 are found in most enchondromas of Ollier disease and Maffucci syndrome and in a substantial proportion of solitary central cartilage tumours, with IDH1 mutations far outnumbering IDH2.

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Question ID
FESSH-041
First published
Last reviewed
Version
v1.0
Answer key and explanations — open after answering

  1. 1. Giant cell tumour of tendon sheath is the second most common soft-tissue tumour of the hand after the ganglion cyst and typically presents as a firm, non-tender, slowly enlarging nodule, most often on the volar aspect of a finger.

    True

    Ozben's series describes giant cell tumour of tendon sheath as the second most common soft tissue tumour of the hand region after ganglion cyst, and Adams' 65-case series found that it most commonly presented as a firm, non-tender mass in the dominant hand, palmar in 49%, with the index finger (37%) and the distal interphalangeal joint level (35%) the commonest sites. Fotiadis' systematic review of 605 patients found pain in only 15.7% and a female predominance (1:1.47); the current nomenclature is tenosynovial giant cell tumour, localised type.

    • Ozben H, Coskun T. Giant cell tumor of tendon sheath in the hand: analysis of risk factors for recurrence in 50 cases. BMC Musculoskelet Disord. 2019;20(1):457. PMID 31638958
    • Adams EL, Yoder EM, Kasdan ML. Giant cell tumor of the tendon sheath: experience with 65 cases. Eplasty. 2012;12:e50. PMID 23185646
    • Fotiadis E, Papadopoulos A, Svarnas T, Akritopoulos P, Sachinis NP, Chalidis BE. Giant cell tumour of tendon sheath of the digits. A systematic review. Hand (N Y). 2011;6(3):244-9. PMID 22942846

  2. 2. On MRI, giant cell tumour of tendon sheath characteristically shows low signal intensity on both T1- and T2-weighted sequences because of haemosiderin and dense collagen, and enhances markedly after gadolinium.

    True

    Jelinek found all nine lesions hypointense on T1-weighted images and most hypointense or isointense to muscle on T2, noting that decreased signal on both sequences is an uncommon appearance for an extra-articular soft-tissue mass and suggests the diagnosis. De Schepper's radiological–histological correlation attributed the high CT attenuation and the low T2 signal to dense collagen and haemosiderin-laden macrophages, and the marked gadolinium enhancement to a high density of proliferative capillaries; the same series showed that the tumour can erode adjacent bone and mimic an intrinsic osseous lesion. Some lesions are intermediate or mildly hyperintense on T2, so the sign is suggestive rather than absolute.

    • Jelinek JS, Kransdorf MJ, Shmookler BM, Aboulafia AA, Malawer MM. Giant cell tumor of the tendon sheath: MR findings in nine cases. AJR Am J Roentgenol. 1994;162(4):919-22. PMID 8141018
    • De Schepper AM, Hogendoorn PC, Bloem JL. Giant cell tumors of the tendon sheath may present radiologically as intrinsic osseous lesions. Eur Radiol. 2006;17(2):499-502. PMID 16807700

  3. 3. Local recurrence after marginal excision of giant cell tumour of tendon sheath is rare, occurring in fewer than 2 per cent of cases.

    False

    Recurrence is the principal problem of this tumour. Fotiadis' systematic review of ten studies (605 patients) found recurrence in 14.8%, significantly more often in Al-Qattan type II (multiple or non-encapsulated) tumours; Reilly reported 27% at an average of 2 years 3 months, with recurrences up to 10 years after excision, and Adams cites a literature range of 0–44%. Ozben's series using loupe magnification and meticulous removal of satellite nodules reported 6%, and incomplete excision is the one universally accepted risk factor; bone erosion and digital location are inconsistently associated. Treatment of recurrence is repeat marginal excision.

    • Fotiadis E, Papadopoulos A, Svarnas T, Akritopoulos P, Sachinis NP, Chalidis BE. Giant cell tumour of tendon sheath of the digits. A systematic review. Hand (N Y). 2011;6(3):244-9. PMID 22942846
    • Reilly KE, Stern PJ, Dale JA. Recurrent giant cell tumors of the tendon sheath. J Hand Surg Am. 1999;24(6):1298-302. PMID 10584957
    • Ozben H, Coskun T. Giant cell tumor of tendon sheath in the hand: analysis of risk factors for recurrence in 50 cases. BMC Musculoskelet Disord. 2019;20(1):457. PMID 31638958
    • Al-Qattan MM. Giant cell tumours of tendon sheath: classification and recurrence rate. J Hand Surg Br. 2001;26(1):72-5. PMID 11162024

  4. 4. Enchondromas of the hand most commonly arise in the distal phalanx.

    False

    Gaulke's analysis of 357 solitary hand enchondromas (327 from the literature plus 30 own cases) found 283 in the phalanges, 70 in the metacarpals and only four in the carpus, with highly significant preferences for the proximal phalanges and the little-finger ray; the proximal phalanx of the little finger was the single most commonly involved bone. Yasuda's series of ten cases comprised six proximal phalanges, two middle phalanges and two metacarpals. Distal phalangeal enchondromas are uncommon. Enchondroma is the most common primary bone tumour of the hand and often presents as a pathological fracture.

    • Gaulke R. The distribution of solitary enchondromata at the hand. J Hand Surg Br. 2002;27(5):444-5. PMID 12367543
    • Yasuda M, Masada K, Takeuchi E. Treatment of enchondroma of the hand with injectable calcium phosphate bone cement. J Hand Surg Am. 2006;31(1):98-102. PMID 16443112

  5. 5. Somatic mutations of IDH1 or IDH2 are found in most enchondromas of Ollier disease and Maffucci syndrome and in a substantial proportion of solitary central cartilage tumours, with IDH1 mutations far outnumbering IDH2.

    True

    Amary found heterozygous somatic IDH1/IDH2 mutations, which produce the oncometabolite 2-hydroxyglutarate, in at least 56% of central and periosteal cartilaginous tumours from enchondroma through to dedifferentiated chondrosarcoma, in patients with solitary and with multiple lesions, with an IDH1:IDH2 ratio of 10.6:1 and no germline mutations; peripheral chondrosarcomas and osteochondromas carried none. Pansuriya identified somatic mosaic IDH1 (98%) or IDH2 (2%) mutations in 81% of patients with Ollier disease and 77% with Maffucci syndrome — in 87% of their enchondromas — and in 40% of solitary central cartilaginous tumours, explaining the non-hereditary, asymmetric distribution of the enchondromatoses.

    • Amary MF, Bacsi K, Maggiani F, Damato S, Halai D, Berisha F, et al.. IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours. J Pathol. 2011;224(3):334-43. PMID 21598255
    • Pansuriya TC, van Eijk R, d'Adamo P, van Ruler MA, Kuijjer ML, Oosting J, et al.. Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome. Nat Genet. 2011;43(12):1256-61. PMID 22057234