Abstract
Cutaneous angioleiomyomas (ALMs) are uncommon benign tumours of the skin which derive from the smooth muscle layer of dermal blood vessels. They usually present as tender nodules in the fifth or sixth decade of life, predominantly in the legs of females. These tumours rarely present on the head and neck, especially the ear. Head and neck ALMs differ from their more common leg counterparts in that they are painless. Additionally, they do not manifest with a female predominance. Herein, a new case of a painless auricular ALM in a 63-year-old man is reported.
Keywords: dermatology; ear, nose and throat/otolaryngology; pathology
Background
Cutaneous angioleiomyomas (ALMs), also known as vascular leiomyomas, are a type of benign subcutaneous or deep dermal tumour derived from vascular smooth muscle. They are typically distributed over the lower legs of an adult female and appear flesh-coloured and well-circumscribed.1 They are commonly recognised clinically since they are painful but are often misdiagnosed clinically as epidermoid cysts. Almost 10% of ALMs will develop on the head or neck (including the ear), and these are more difficult to clinically diagnose because they are usually painless.2 3 Less than 3% of ALMs will occur on the ear, making the case of discussion, an auricular ALM, a rare tumour on a rare location.2
Case presentation
A 63-year-old man developed a painless nodule on the right helix. This gradually developed over 1 year prior to presenting to the office. Physical examination showed an erythematous, smooth, well-demarcated and mobile nodule located over the superior aspect of the right helix which measured 7 mm (figure 1). The nodule resembled an epidermoid cyst that was non-tender. The patient was otherwise healthy and his family history was unremarkable.
Figure 1.
A smooth, well-demarcated and mobile nodule located over the right helix.
Investigations
The auricular nodule was excised under local anaesthesia and sent for pathological examination with a suspected diagnosis of epidermoid cyst. Microscopic examination revealed a circumscribed nodule which consisted of numerous interlacing bundles of smooth muscle fibres consisting of cells with thin, blunt-ended nuclei. Numerous blood vessels with thick muscular walls were appreciated, and the smooth muscle bundles extended tangentially from the periphery of these vessels (figure 2). Smooth muscle actin was strongly positive (figure 3), and the endothelial cells stained positive for CD31 (figure 4). Additionally, S-100 was positive only in the intra-tumour nerves and MART was negative. This was diagnostic of an angioleiomyoma.
Figure 2.
((10×) H&E stain. Blunt-ended spindle cells with eosinophilic cytoplasm are seen circumscribing flattened vascular channels.
Figure 3.
(4×) Smooth muscle actin (SMA) stain. A large, well-circumscribed collection of SMA-positive cells in the dermis.
Figure 4.
(4×) CD31 stain. Vascular channels within the tumour are CD31 positive.
Differential diagnosis
Haemangioma versus epidermoid cyst.
Treatment
Since the auricular ALM was completely excised prior to its histological diagnosis, no further management was necessary.
Outcome and follow-up
Four months post excision, the patient denies any reoccurrence of the nodule.
Discussion
Cutaneous ALMs are uncommon, and ALMs that occur on the ear are especially uncommon. Over a period of 16 years (1999–2015) in a dermatopathology lab in France, only 36 cases of ALMs were diagnosed. Of those cases, two were on the ear.4 In a larger study of 562 ALMs, auricular ALMs made up 2.8% of the total cases.2 Auricular ALMs, like other ALMs of the head and neck, are usually painless,3 5–11 with rare exceptions having been reported.12 Although cutaneous ALMs rarely occur on the ear, they should be included in the differential of soft tissue nodules over this anatomy. ALMs definitively diagnosed via histological examination of a biopsy or excision specimen as their clinical appearance in non-specific.
Learning points.
Cutaneous angioleiomyomas (ALMs) are a rare benign tumour that often occur on the legs of an adult woman
Less than 3% of ALMs occur on the ear.
ALMs of the head/neck (including the ear) are usually painless, in contrast to their painful counterpart on the leg.
Auricular ALMs may clinically mimic an epidermoid cyst.
Cutaneous ALMs, although very rare, should be on the differential for soft tissue nodules of the ear.
Footnotes
Contributors: AJN (submission author herein) compiled the work of the authors and coordinated the article’s completion. I contributed to formulating the majority of the text for the article, including the discussion section. I gained consent from the patient. DM constructed the literature review of the article topic and wrote the background section of the article. SE interpreted/diagnosed the pathology presented herein, and she yielded the figures and captions, and her expertise in pathology was used to solidify the pathology details of the article. JB saw the patient in office initially and performed the surgery on the pathology presented herein. He was our chief editor of the text.
Funding: The authors have not given a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests: None declared.
Patient consent: Obtained.
Provenance and peer review: Not commissioned; externally peer reviewed.
References
- 1.Patterson J. Weedon’s Skin Pathology. 4th ed London UK: Churchill Livingstone, 2016. [Google Scholar]
- 2.Hachisuga T, Hashimoto H, Enjoji M. Angioleiomyoma. A clinicopathologic reappraisal of 562 cases. Cancer 1984;54:126–30. 10.1002/1097-0142(19840701)54:1<126::AID-CNCR2820540125>3.0.CO;2-F [DOI] [PubMed] [Google Scholar]
- 3.Yoon TM, Yang HC, Choi YD, et al. Vascular leiomyoma in the head and neck region: 11 years experience in one institution. Clin Exp Otorhinolaryngol 2013;6:171 10.3342/ceo.2013.6.3.171 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Kanitakis J. Angioleiomyoma of the auricle: An unusual tumor on a rare location. Case Rep Otolaryngol 2017;2017:1–3. 10.1155/2017/8289710 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Kim H-U, Mun J-H, Park S-H, et al. Angioleiomyoma on the Helix of the Ear. J Dermatol 2004;31:858–9. 10.1111/j.1346-8138.2004.tb00618.x [DOI] [PubMed] [Google Scholar]
- 6.Akkash L, Nilles M, Mayser P. [Solitary blue nodule on the ear helix]. Hautarzt 2008;59:54-7 10.1007/s00105-007-1342-9 [DOI] [PubMed] [Google Scholar]
- 7.Cıtıl R, Ciralik H, Gül A, et al. Auricular angioleiomyoma: a case report. Turk Patoloji Derg 2011;27:268 10.5146/tjpath.2011.01089 [DOI] [PubMed] [Google Scholar]
- 8.Chen YA, Chuang WY, Hsueh S, et al. Solitary nodule on the auricle. Int J Dermatol 2012;51:1427–8. 10.1111/j.1365-4632.2012.05628.x [DOI] [PubMed] [Google Scholar]
- 9.Kim H-I, Roh S-G, Lee N-H, et al. Angioleiomyoma of the Auricle. Arch Plast Surg 2013;40:68 10.5999/aps.2013.40.1.68 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 10.Wirth GA, Sundine MJ, Kong AP, et al. Auricular angioleiomyoma: a case report and review of the literature. Ear Nose Throat J 2007;86:281-3. [PubMed] [Google Scholar]
- 11.Oh JH, Park JM, Choi H, et al. Two cases of angioleiomyoma of the auricle. J Int Adv Otol 2015;11:95-6. [DOI] [PubMed] [Google Scholar]
- 12.Palleschi GM, Cristofaro G, Maio V. Angioleiomyoma of the auricle: a rare finding. G Ital Dermatol Venereol 2013;148:537-8. [PubMed] [Google Scholar]