Superficial Angiomyxoma: A Case Report Elizabeth M. Aradine, DO,* Albert E. Rivera, DO,** Mark Teague, MD***

*Intern, Preliminary Medicine, St. Vincent Charity Medical Center, Cleveland, OH **Dermatologist and Mohs Surgeon, Southeastern Skin Cancer & Dermatology, Madison, AL ***Dermatopathologist, Pathology Associates, Huntsville, AL

Abstract Superficial angiomyxoma is a benign proliferation of highly vascular myxoid cells. Herein, we report a case of a solitary, superficial angiomyxoma of the nasal dorsum in a patient without Carney complex. In the literature, there are 28 reports of superficial angiomyxoma found on the head and neck. Two of the case reports describe lesions on the nasal dorsum, each treated with a different method. We present another case of superficial angiomyxoma on the nasal dorsum and compare our patient’s treatment and outcome -- wide local excision with disease-free survival for 11 months at time of writing -- with the other reported cases.

enlarged over time. A second complaint was Introduction Figure 2 Superficial (or cutaneous) angiomyxoma, a benign another large “mole,” also on the left side of his proliferation of highly vascular myxoid cells, can nose, that had been present for years. This lesion appear as a solitary lesion or as multiple lesions had not changed in size in the past few years but associated with Carney complex.1-3,5 Superficial was mildly painful and was also irritated by his angiomyxomas more commonly occur in men eyeglasses. and have a predilection for the head, neck, and Physical exam showed two flesh-colored to 2,3 trunk. The majority of superficial angiomyxomas slightly pigmented papules on the left nasal root (75%) do not have epithelial components, and and left nasal side wall (Figure 1). The lesion these have a lower recurrence rate than those with clinically appeared as a benign dermal nevus. epithelial components.2,3 Current recommended treatment is wide local excision; however, it has Differential a 20% to 30% local recurrence rate.3 Superficial The clinical differential diagnosis of the patient’s angiomyxomas are not to be confused with benign-appearing papule includes benign nevus aggressive angiomyxomas, which are deeper and, less likely, basal cell carcinoma. with high vascular proliferation, more common occurrence in women and a predilection Testing for the vulvar region.6,7 Here, we present the case The patient underwent a shave biopsy and shave of a 55-year-old male with a solitary superficial removal of the respective lesions due to history angiomyxoma of the nasal dorsum without and examination. evidence of Carney complex. The patient was Diagnosis treated with wide local excision and remains Pathology of the lesion revealed a multinodular disease free at 11 months. Figure 3 growth of myxoid areas that contained scattered spindled and epithelioid cells (Figures 2, 3). Some Case Report scattered small blood vessels were seen but were Presentation not a predominant feature. Inflammatory cells A 55-year-old male presented to our clinic with such as lymphocytes, mast cells, and neutrophils a painless, enlarging papule on the left side of were present with occasional multinucleate cells his nose. It had been growing for two years and and minimal atypia. The mitotic activity was was frequently irritated by his eyeglasses. Per his sparse. Immunohistochemistry stain was negative report, the lesion started as a “pimple” and had for S100, actin and calponin and positive for Figure 1 CD34. Although there was little development of the vascular component, the histologic findings Figures 2 and 3. H&E, 40x/200x: Pathology were most compatible with a benign superficial showing multinodular growth of myxoid areas angiomyxoma. (Of note, one differentiating factor containing scattered spindled and epithelioid between aggressive angiomyxomas and superficial cells. angiomyxomas is that the former are - positive on pathology staining.) The other lesion Our patient presented with a benign-appearing was found to be a benign nevus. papule with the clinical differential diagnosis of benign nevus and, less likely, basal cell carcinoma. Treatment Reported clinical differential diagnoses Recommended treatment was wide local considered in other cases of angiomyxomas on excision, which the patient underwent without different locations of the body have included complication shortly after his visit. The patient verruca vulgaris, , and tumor or lesion has been disease-free, with no signs or symptoms not specified.1,11,12 The pathological differential of recurrence, for 11 months at the time of writing diagnosis of our lesion includes neurothekeoma, of this article. , and plexiform fibrohistiocytic tumor. Other reports have given pathological differentials including, but not limited to, cutaneous myxoid cysts, focal cutaneous mucinosis, myxoid neurofibroma, and .2,3 Discussion

Page 46 Superficial Angiomyxoma: A Case Report Wide excision of superficial angiomyxoma has could ultimately prove to be more appropriate. Correspondence: Elizabeth M. Aradine, DO, been the historical treatment of choice and can be Nevertheless, the efficacy and recurrence need 2351 E 22nd Street, Suite 342W, Cleveland, OH curative; however, this is not easily accomplished, further study in trials. Although the low incidence 44115; Ph: 216-861-6200; F: 216-363-2721; as studies show the local recurrence rate is 20% of this lesion can make sufficient statistical power [email protected] to 30%.1-3,5 Recurrence is typically dependent more difficult, it can be a worthwhile future on depth of invasion, presence of epithelial investigation. components, and adequacy of lesion excision.1-3,7,8 Most superficial angiomyxomas invade past the 2 References dermis and into subcutaneous tissue. Increased 1. Fetsch J, Laskin W, Tavassoli F. Superficial depth of invasion is directly related to increased Angiomyxoma (Cutaneous Myxoma): A aggressiveness and recurrence in multiple tumors. Clinicopathologic Study of 17 Cases Arising in While there are no studies directly looking at the Genital Region. International J Gyn Path. this relationship in the superficial angiomyxoma, 1997;16(4):325-34. it would be likely that the aggressiveness and recurrence of superficial angiomyxomas directly 2. Allen PW, Dymock RB, MacCormac LB. increase with depth as well; this could be a topic Superficial Angiomyxomas with and without of further research. Research does indicate that Epithelial Components. Am J Surg Pathol. lesions with presence of epithelial components 1988;12(7):519-30. have a higher tendency to recur, with a rate of 68% 3. Allen PW. Myxoma Is Not a Single Entity: A compared to 13% in lesions without epithelial Review of the Concept of Myxoma. Ann Diagn 2 components. The median recurrence time of Pathol. 2000 Apr;4(2):99-123. superficial angiomyxoma is 18 months;2 however, lesions have recurred after up to 20 years.1,3 For 4. Tardío J. CD-34 reactive tumors of the skin. An the aforementioned aggressive angiomyxomas, updated review of an ever-growing list of lesions. current recommended treatment is also wide J Cutan Pathol. 2008 Dec-2009 Jan;36:89-102. local excision, but these lesions are more locally 5. Wilk M, Schmoeckel C, Kaiser HW, Hepple aggressive and have an increased recurrence R, Kreysel HW. Cutaneous angiomyxoma: A rate of 36% to 72%.6,10 Although uncommon, benign neoplasm distinct from cutaneous focal metastases from aggressive angiomyxomas have mucinosis. J Am Acad Dermatol. 1995;33(2)Pt been reported.10 2:352-5. There are 28 reported cases of superficial 6. Satter E. Solitary superficial angiomyxoma: an angiomyxoma occurring in the head and neck infrequent but distinct tumor. J Cutan region.9 Two cases are reported with a superficial Pathol. 2009;36(Suppl.1):56-9. angiomyxoma in a location similar to ours. Each 7. Heymans O, Medot M, Hermanns-Le was treated with a different type of excision and T, Pierard G, Fissette J, Lahaye T. Recurrent resulted in different outcomes.7,8 One report Pleomorphic Solitary Angiomyxoma of the Face. described a superficial angiomyxoma on the Dermatology. 1999;198:195-7. nasal dorsum treated with wide local excision.7 It recurred multiple times in 26 years, with the 8. Kahn SL, Juhl ME, Sidiropoulos M, Guitart J, longest disease-free interval at eight years. The Antonijevic S, Krunic AL. Angiomyxoma of the latest treatment of that lesion was reported to nasal dorsum treated by Mohs surgery. Australas be a wide local excision with a 0.5 cm margin. J Dermatol. 2016 Feb;57(1):e8-e10. Epub 2014 Inadequate excision was the presumed cause of Oct 21. previous recurrences, although margins were 9. Rodriguez PR, de Vincente JC, de Villalain not reported from prior excisions. The patient L, Blanco V. [Superficial Angiomyxoma of the remained disease-free two years post the latest Parotid Region and Review of the Literature.] excision. The other reported case of superficial Acta Otorrinolaringol Esp [Internet]. 2012 angiomyxoma on the nasal dorsum was treated [cited 2015 Apr 30];63(2):147-9. Available by Mohs surgery.8 The patient was disease-free from: http://www.elsevier.es/en-revista- 18 months post excision. Both methods, wide acta-otorrinolaringologica-espanola-402- local excision and Mohs, appear to be options for articulo-superficial-angiomyxoma-of-the- treatment of superficial angiomyxoma. parotid-90134262. Spanish. 10. Kura MM, Jindal SR, Khemani UN. Conclusion Aggressive angiomyxoma of the vulva: An While our patient and both of the comparative uncommon entity. Indian Dermatol Online J. cases were relapse-free upon latest follow-up, 2012 May-Aug [cited 2015 March 9];3(2):128- extended follow-up is still required. Although 30. Available from: http://www.ncbi.nlm.nih. wide local excision is currently the treatment of gov/pmc/articles/PMC3481881/?report=reader choice for superficial angiomyxomas, there are no studies defining the optimal margins of wide 11. Misago N, Mori T, Yoshioka M, Narisawa Y. local excision for an acceptable cure rate. Since Digital superficial angiomyxoma. Clin and Exp this is a relatively rare tumor, the determination Dermatol. 2007;32:536-8. of a sufficient margin is left to the physician’s 12. Choi HJ, Kim YJ, Yim JH, Kim MY, Kim discretion. With its tissue-sparing benefits and HO, Park YM. Unusual presentation of solitary potential lower risk of recurrence (providing less cutaneous myxoma. JEADV. 2006;21:392-435. long-term morbidity from additional surgery as well as cost savings), Mohs micrographic surgery

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