FESSH prep Hand tumours Classical triad and subungual site, cell of origin, cause of recurrence, extradigital presentation, association with neurofibromatosis type 1
Glomus tumour of the hand
Regarding glomus tumours of the hand:
1.Glomus tumours arise from Schwann cells of the terminal branches of the digital nerves.
2.The classical triad of a subungual glomus tumour is severe pain, localised tenderness and cold sensitivity.
3.Recurrence after excision of a glomus tumour is mainly caused by incomplete excision.
4.Extradigital glomus tumours, such as those in the forearm, usually present with the full classical triad, including cold intolerance.
5.Glomus tumours of the fingers, sometimes multiple, are part of the tumour spectrum of neurofibromatosis type 1.
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Answer key and explanations — open after answering
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1. Glomus tumours arise from Schwann cells of the terminal branches of the digital nerves.
False
Glomus tumours are usually benign vascular hamartomas whose cells resemble the smooth muscle cells of the normal glomus body, a thermoregulatory shunt concentrated in the fingers and toes. Painful nerve lesions such as neuroma belong in the differential diagnosis, not in the histogenesis.
- Chou T, Pan SC, Shieh SJ, Lee JW, Chiu HY, Ho CL. Glomus Tumor: Twenty-Year Experience and Literature Review. Ann Plast Surg. 2016;76 Suppl 1:S35-40. PMID 26808758
- Brems H, Park C, Maertens O, Pemov A, Messiaen L, Upadhyaya M, et al.. Glomus tumors in neurofibromatosis type 1: genetic, functional, and clinical evidence of a novel association. Cancer Res. 2009;69(18):7393-401. PMID 19738042
- Morey VM, Garg B, Kotwal PP. Glomus tumours of the hand: Review of literature. J Clin Orthop Trauma. 2016;7(4):286-291. PMID 27857505
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2. The classical triad of a subungual glomus tumour is severe pain, localised tenderness and cold sensitivity.
True
Morey's review describes the classical triad of localised tenderness, severe pain and cold sensitivity, often with bluish or pinkish-red discolouration of the nail plate; Love's pin test and Hildreth's test support the clinical diagnosis. Chou's review notes that digital glomus tumours most commonly arise subungually and show a strong female predominance.
- Morey VM, Garg B, Kotwal PP. Glomus tumours of the hand: Review of literature. J Clin Orthop Trauma. 2016;7(4):286-291. PMID 27857505
- Chou T, Pan SC, Shieh SJ, Lee JW, Chiu HY, Ho CL. Glomus Tumor: Twenty-Year Experience and Literature Review. Ann Plast Surg. 2016;76 Suppl 1:S35-40. PMID 26808758
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3. Recurrence after excision of a glomus tumour is mainly caused by incomplete excision.
True
Complete surgical excision usually gives complete relief of symptoms. Chou's review attributes recurrence largely to incomplete excision but recommends repeat imaging when symptoms return, to rule out a new or a malignant lesion.
- Chou T, Pan SC, Shieh SJ, Lee JW, Chiu HY, Ho CL. Glomus Tumor: Twenty-Year Experience and Literature Review. Ann Plast Surg. 2016;76 Suppl 1:S35-40. PMID 26808758
- Morey VM, Garg B, Kotwal PP. Glomus tumours of the hand: Review of literature. J Clin Orthop Trauma. 2016;7(4):286-291. PMID 27857505
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4. Extradigital glomus tumours, such as those in the forearm, usually present with the full classical triad, including cold intolerance.
False
In Schiefer's 20-year series of 56 extradigital glomus tumours, 48 patients presented with pain and localised tenderness but only one with cold intolerance. Symptoms had lasted on average more than seven years, most patients had been misdiagnosed, and MRI was the most useful investigation for localising the lesion.
- Schiefer TK, Parker WL, Anakwenze OA, Amadio PC, Inwards CY, Spinner RJ. Extradigital glomus tumors: a 20-year experience. Mayo Clin Proc. 2006;81(10):1337-44. PMID 17036559
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5. Glomus tumours of the fingers, sometimes multiple, are part of the tumour spectrum of neurofibromatosis type 1.
True
Brems reported 11 individuals with neurofibromatosis type 1 (NF1) who had 20 finger glomus tumours and one in a toe, five of them with multiple tumours, and found germline and somatic NF1 mutations in the tumours. The authors concluded that finger and toe glomus tumours should be considered part of the NF1 tumour spectrum.
- Brems H, Park C, Maertens O, Pemov A, Messiaen L, Upadhyaya M, et al.. Glomus tumors in neurofibromatosis type 1: genetic, functional, and clinical evidence of a novel association. Cancer Res. 2009;69(18):7393-401. PMID 19738042