Australasian Journal of Dermatology (2016) 57, e8–e10

doi: 10.1111/ajd.12245


Angiomyxoma of the nasal dorsum treated by Mohs surgery Steven L Kahn,1 Mark E Juhl,2 Michael Sidiropoulos,3 Joan Guitart,3 Sasha Antonijevic1 and Aleksandar L Krunic1,3 1

Department of Dermatology, 2College of Medicine, University of Illinois, and 3Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA

ABSTRACT Cutaneous angiomyxomas are myofibroblastic neoplasms with locally aggressive behaviour and a high risk of recurrence. We describe a case of a solitary cutaneous angiomyxoma presenting on the nasal dorsum of a 28-year-old man, excised with Mohs surgery using permanent section control and repaired with an advancement flap. Histology showed myxoid nests of bland CD34-positive and vimentinpositive stellate and spindled cells in the deep dermis and abundant thin-walled blood vessels. An echocardiogram, performed to rule out the possibility of a cardiac myxoma with cutaneous embolisation, was normal. Key words: angiomyxoma, Mohs surgery, myxoma, superficial angiomyxoma.

INTRODUCTION Superficial angiomyxomas of the skin are rare tumours that can be locally aggressive and recur if they are not completely removed.1 The appearance of a solitary cutaneous angiomyxoma warrants an examination to rule out the presence of both Carney complex and paradoxical metastasis from an isolated cardiac myxoma.1–3 We describe a case of a solitary cutaneous angiomyxoma presenting on the nasal dorsum of a 28-year-old man, which was excised with Mohs surgery using permanent section control and repaired

with an advancement flap. The use of Mohs micrographic surgery with permanent section control allows for the complete removal of the lesion with the maximal preservation of healthy skin, which is important in cosmetically sensitive areas. Hence, it decreases the risk of recurrence and locally aggressive behaviour.

CASE REPORT A 28-year-old man with no significant medical history presented with a solitary nodule that had been on the left nasal dorsum for the previous 5–6 months. The lesion was tender and occasionally bled after the patient attempted to express the contents. The tumour was a 1.2 × 1.1 cm in size cystic nodule with yellow to clear drainage from the centre and surrounding erythema (Fig. 1). A biopsy revealed an ulcerated epidermis with the proliferation of stellate and round to spindled cells with relatively uniform nuclei and scant cytoplasm in the deep dermis, extending into the subcutaneous fat and skeletal muscle, embedded in myxoid stroma (Fig. 2a–c). The myxoid material stained homogenously with Alcian blue (pH 2.5). The cells stained positively with CD34 and vimentin but were negative for other markers, including S100, Melan-A, microphthalmia transcription factor and periodic acid–Schiff, as well as gram and acid-fast bacilli stains. Mitoses were rare, and significant cellular pleomorphism was not identified. Mohs surgery with two stages using permanent section control was performed. The initial debulking of the tumour was processed vertically, followed by the horizontal sectioning of the excised peripheral ring. This enabled the assessment of the entire peripheral margin of the tumour. The surgery resulted in a 1.7 × 1.5 cm defect, which was closed with an advancement flap, with no signs of recurrence at 18 months follow up.

CONCLUSION Correspondence: Dr Steven L. Kahn, Department of Dermatology, University of Illinois, 808 S. Wood Street, Room 380, Chicago, IL 60612, Email [email protected] Steven L. Kahn, MD. Mark E. Juhl, BA. Michael Sidiropoulos, MD. Joan Guitart, MD. Sasha Antonijevic, RN. Aleksandar L. Krunic, MD. Conflict of interest: none Submitted 10 June 2014; accepted 26 July 2014.

Defined by Stout in 1948, a myxoma is ‘a true neoplasm composed of stellate cells set in a loose mucoid stroma Abbreviation: SAM

superficial angiomyxoma

© 2014 The Australasian College of Dermatologists

Angiomyxoma of the nasal dorsum

Figure 1 Cystic nodule with surrounding erythema on the left nasal dorsum.

through which course delicate reticulin fibers in various directions’.1 Superficial angiomyxoma (SAM), also called cutaneous angiomyxoma, is a rare benign yet locally aggressive myxoid-type neoplasm first described by Allen and colleagues1 and named angiomyxoma to stress its vascular component. Soft tissue myxomas consist of five entities: intramuscular myxomas, juxta-articular myxomas, superficial angiomyxomas, aggressive angiomyxomas and myxoid neurothekeomas.4 SAM are distinguished from aggressive (recurrent, infiltrative, deep) angiomyxomas, which affect the genital region, are more common in females and are positive for steroid hormone receptors.5 Clinically, SAM presents as a skin nodule or polypoid lesion less than 5 cm in size and is located most commonly on the trunk or lower limbs, followed by the head and neck, and least commonly on the upper limbs.1 In a retrospective review, only 28 cases of SAM have been described in the


head and neck area, with a slight male predominance and mean age at presentation of 36.45 years.6 Diagnoses of SAM have also been made in association with adnexal tumours, including pilomatricomas and trichofolliculomas.6 Histologically, SAM demonstrates a well-defined proliferation of spindle, stellate and oval cells within a myxoid stroma.6 SAM characteristically has a scattered distribution of thin-walled blood vessels. Inflammatory cells, including interstitial neutrophils, which are absent in other myxomatous lesions such as myxoid neurothekeomas, may be present in SAM.6 Furthermore, myxoid neurothekeomas tend to demonstrate positive staining for S100 and epithelial membrane antigen.4 Another entity to be distinguished from SAM is cutaneous focal mucinosis by its well-defined dermal mucin accumulation, epidermal hyperplasia and thin collagen fibres without the presence of a reticulin network, elastic fibres or prominent vascular component, and a tendency towards local recurrence.1,2,7 Most reported cases of SAM, including immunohistological stains, have stained positive with vimentin and negative with desmin. Variable positivity has been shown for CD34, S100, smooth muscle actin, muscle-specific actin, factor XIIIa and CD68.8 There is a known association of angiomyxomas with Carney complex,2 recently linked to the modification of function of the catalytic subunits of cyclic AMP-dependent protein kinase A.9 This autosomal dominant syndrome, which includes the presence of cardiac and cutaneous myxomas, lentigines and endocrine hyperactivity,1,2 should be ruled out in any patient presenting with solitary SAM since it could be a forme fruste of the condition. This was ruled out in our patient due to the lack of lentigines, endocrine hyperactivity and cardiac myxomas. In addition, embolic angiomyxomatous tumours should also be considered, as cases of solitary myxomatous skin nodules of the head and thoracic region have been described in association with atrial myxomas even many years after tumour resection.3 Apart from a mandatory cardiac ultrasound to rule out atrial myxoma in any patient with SAM, one should also consider the high propensity for local recurrence, which was reported in up to 38% of cases.1 We decided to perform Mohs surgery using permanent section control based on the excellent report of Sage and colleagues,10 who

Figure 2 (a, b) Large ulcer with underlying collections of fibroblast-like cells in the deep dermis with a myxoid and vascularised stroma (H&E original magnification ×2.5 and ×40). (c) Spindle-shaped, fibroblast-like cells with thin-walled blood vessels and focal microhaemorrhages (H&E original magnification ×20). © 2014 The Australasian College of Dermatologists

SL Kahn et al.


successfully treated neurothekeomas and other benign tumours presenting in a similar fashion. The presence of SAM in cosmetically sensitive areas with a high tendency for recurrence, as in our patient, should make another possible indication for microscopically controlled excision due to the required strict margin control, maximal preservation of the healthy tissue and high success rate for complete tumour removal.

4. 5.






Allen PW, Dymock RB, MacCormac LB. Superficial angiomyxomas with and without epithelial components. Report of 30 tumors in 28 patients. Am. J. Surg. Pathol. 1988; 12: 519–30. Carney JA, Headington JT, Su WP. Cutaneous myxomas. A major component of myxomas, spotty pigmentation, and endocrine overactivity. Arch. Dermatol. 1986; 122: 790–8. Terada Y, Wanibuchi Y, Noguchi M et al. Metastatic atrial myxoma to the skin at 15 years after surgical resection. Ann. Thorac. Surg. 2000; 69: 283–4.

© 2014 The Australasian College of Dermatologists

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Allen PW. Myxoma is not a single entity: a review of the concept of myxoma. Ann. Diagn. Pathol. 2000; 4: 99–123. Rocco F, Cozzi G, Spinelli MG et al. Massive recurring angiomyxoma of the scrotum in a obese man. Rare Tumors 2011; 3: 98–9. Rosado Rodriguez P, de Vicente JC, de Villalain L et al. Superficial angiomyxoma of the parotid region and review of the literature. Acta Otorrinolaringol. Esp. 2012; 63: 147– 9. Wilk M, Schmoeckel C. Cutaneous focal mucinosis – a histopathological and immunohistochemical analysis of 11 cases. J. Cutan. Pathol. 1994; 21: 446–52. Satter EK. Solitary superficial angiomyxoma: an infrequent but distinct soft tissue tumor. J. Cutan. Pathol. 2009; 36: 56– 9. Forlino A, Vetro A, Garavelli L et al. PRKACB and Carney complex. N. Engl. J. Med. 2014; 370: 1065–7. Sage RJ, Lopiccolo MC, Laungani AG et al. Mohs micrographic surgery for the treatment of cellular neurothekeoma and review of its use in surgical management of benign tumors. Dermatol. Surg. 2010; 36: 1214–18.

Angiomyxoma of the nasal dorsum treated by Mohs surgery.

Cutaneous angiomyxomas are myofibroblastic neoplasms with locally aggressive behaviour and a high risk of recurrence. We describe a case of a solitary...
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