Feb 6, 1985 - tinguish this pathology from a subungual melanoma or haemangioma. The age at presentation is gener- ally over 20 and no familialtraits have ...
Journal of the Royal Society of Medicine Volume 80 October 1987
647
Case reports
Multiple glomus tumours
Case presented to Section
of Surgery, 6 February 1985
J C Shotton MB FRCS T I Davidson MRCP FRCS G Westbury FRCP FRCS Academic Surgical Unit, Royal Marsden Hospital, London SW3 Keywords: glomangioma; glomus tumours, multiple
Multiple glomus tumours are extremely rare and differ from the relatively more common solitary glomus tumours in clinical presentation and histological features. We now report a case of multiple glomus tumours and review the features of these uncommon skin lesions. Case report A 28-year-old Caucasian woman presented with multiple blue lesions, 7 in all, located on the right thigh, left elbow, left shoulder, left buttock, right forearm, left thenar eminence and ulnar border of right hand. The thigh, forearm and thenar lesions had become
painful. Excision of the right forearm lesions had been performed four months previously but had recurred in the scar. At the age of 14 a similar lesion had been removed from the infraclavicular region. There was no family history of note. Clinically the lesions were blue, oval, slightly raised and between 3 and 8 mm in size. On palpation they were soft and did not blanch on pressure. The thenar, buttock and forearm lesions were moderately tender. Excision of the symptomatic lesions showed features typical of glomus tumour, glomangioma variant histology. Discussion Glomus tumours are usually regarded as hamartomatous malformations of the normal glomus body'. The normal glomus body is a small, richly innervated arteriovenous anastomosis situated mainly in the extremities concerned with thermoregulation. It has an afferent arteriole which runs into a thick-walled and tortuous arteriovenous anastomosis known as the Saquet-Hoyer canal (Figure 1). It is here that the glomus cells, diagnostic of both the glomus body and tumour, are found. These -are rounded epithelialappearing cells in close apposition to each other, with clear or slightly eosinophilic cytoplasm (Figure 2). As the ambient temperature falls, the SaquetHoyer canal dilates, diverting blood away from the dermal circulation and conserving temperature.
Solitary glomus tumours are almost invariably subungual in the finger and present with the classical and virtually diagnostic triad of symptoms comprising lancinating limb pain out of all proportion to the small size of the lesion; exquisite tenderness to even
-Sucquet-Hoyer canal - Collecting venule h- Afferent arteriole -Afferent artery Figure 1. Schematic representation of aglomus body
the lightest touch; and exacerbation of symptoms on exposure to cold2. These symptoms readily distinguish this pathology from a subungual melanoma or haemangioma. The age at presentation is generally over 20 and no familial traits have been reported. Very occasionally solitary glomus tumours occur at other sites including bone3, stomach', and nose4. Multiple glomus tumours, in contrast, are rarely subungual, showing no predilection for any particular region of the body and are often asymptomatic4. The age at presentation is earlier and familial traits are described5. An autosomal dominant mode of inheritance with incomplete penetrance is suggested6.
Differentialdiagnosis: Multiple glomus tumours may clinically resemble certain pigmented skin lesions and some dermal or subcutaneous vascular anomalies. Of the former, junctional naevi may occasionally be multiple and, particularly if placed in thick skin, may appear blue. They are unlikely to be painful and are typically flat, unlike multiple glomus tumours which are usually slightly raised. Malignant melanoma is not commonly simultaneously multiple and usually can be differentiated from bengn lesions by the sinister features of rapid change of pigmentation, itching, bleeding or ulceration. The dysplastic naevus syndrome, a familial condition where multiple naevi occur, each having malignant potential, may enter the differential diagnosis, although these moles will often contain hair and have a natural history quite distinct from multiple glomus tumours. Multiple neurofibromas may acquire pigment and hence enter the differential diagnosis. Of the vascular anomalies, the cavernous haemangioma may be clinically identical when small, slightly raised, non-tender and blue. It is not often multiple and is generally asymptomatic in contrast to glomus tumours, which by virtue of their rich nerve supply may be painful even when small. Histopathological assessment may be required to differentiate between the two. A dermatofibroma (histiocytoma, sclerosing haemangioma), a benign lesion comprising
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Histology: Three different types of tumour are described, largely corresponding to the clinical variants. The classical 'painful' solitary glomus tumour is a predominantly cellular, relatively avascular tumour termed histologically 'glomus tumour proper'. Multiple glomus tumours, however, are altogether more vascular and are termed 'glomangioma variant histology'. They may be mistaken for haemangiomas at low power (Figure 2A) but diagnostic glomus cells can be seen at higher magnifications (Figure 2B). The third histological subtype, termed 'glomangiomyoma', is rare, with smooth muscle cells predominating. Clinical behaviour: Both solitary and multiple glomus tumours are regarded as benign. There are no convincing reports of malignant behaviour in these tumours8, and thus fear of malignant change does not dictate removal of all lesions and appropriate treatment is local excision for symptomatic, cosmetic or diagnostic reasons. Local recurrence after surgical removal is undoubtedly due to incomplete excision. References 1 Laymon CW, Peterson WC. Glomus tumours. A clinicopathological study with special reference to multiple lesions appearing during pregnancy. Arch Dermatol
1965;92:509 Figure 2. Photomicrographs of a multiple glomus tumour: A, low magnification (x 6.6); B, high magnification (x 132) to show glomus cells
fibrous tissue and blood vessels in variable proportions, uncommonly may take on a blue appearance if vascular elements predominate and are dilated; it may then simulate a glomus tumour. It is, however, usually pink or red, usually becoming paler with time, single and found on the lower limbs of females. It may be a reaction to minor trauma to the part7. Campbell de Morgan spots are unlikely to cause confusion, occurring typically in the middle-aged or elderly, being cherry red, non-tender and remaining less than 3 mm in size.
Case presented to Clinical Section, 10 October 1986
Non-functioning paraganglioma in wall of abdominal aortic aneurysm: a source of diagnostic confusion
I Hudson FRCS R K S Phillips MS FRCS E J Williams MS FRCS Department of Surgery, Wexham Park Hospital, Slough, Berkshire Keywords: paraganglioma, non-chromaffin; aortic aneurysm, abdominal
0141-0768/87/ 010648-02/$02.00/0 1987 The Royal Society of Medicine
Paragangliomas are rare tumours ofneuroectodermal origin. We report what appears to be the first case of such a tumour arising in association with an aortic aneurysm - a situation which caused diagnostic confusion and precipitated an emergency operation.
2 Masson P. Glomus neuromyo-arterial des regions tactiles et ses tumeurs. Lyon Chirurgical 1924;21:257 3 Siegel MW. Interosseous glomus tumour. A case report. Am JOrthop 1967;9:98 4 Apfelberg DB, Teasler JL. Unusual locations and manifestations of glomus tumours (glomangiomas). Am J Surg 1968;116:62 5 Beaton LI, Davies L. Glomus tumour. Report of three cases. Analyses of 271 recorded cases. Quarterly Bulletin of the Northwest University Medical School 1941;15:254 6 Conant MA, Wiesenfield SL. Multiple glomus tumours of the skin. Arch Dermatol 1941;103:481 7 Roberts AHN. The diagnosis and treatment of benign skin tumours. Surgery 198$;26:621-7 8 Enzinger FM, Wiess SW. Soft tissue tumours. New York: Mosby, 1983:450
(Accepted 14 July 1986. Mr Shotton is now at King's College Hospital, London SE5)
Case report
A 70-year-old man presented to his general practitioner with a one-year history of back pain radiating down both legs and was found on examination to have a pulsatile abdominal mass. Real-time ultrasound appearances suggested an aneurysm with either a double lumen or a collection of blood outside the aorta (Figure 1). Clinically there was no evidence of leakage or dissection, but in view ofthe possibility of impending rupture, emergency repair was undertaken. At laparotomy an aortic aneurysm involving also the common iliac arteries was found. There was no evidence of a leak but there was a marked anterior prominence which, on incising the aneurysm which was of inflammatory type, was found to be caused by a soft tissue mass 4.5 cm in diameter lying within the aortic wall. This was fully excised and a routine repair of the aneurysm performed using a bifurcated woven Dacron graft. Recovery was uneventful and
