Auricular angioleiomyoma: a case report and review of the literature.

Abstract

A 29-year-old woman presented with an unusual lesion on the right auricular antihelix. The mass was purple and painful, and it had been present for 17 years. Preoperatively, the presumptive diagnosis was a venous malformation. The mass was resected, and a staged reconstruction was performed. Microscopic analysis of the specimen revealed that the lesion was an angioleiomyoma. Most cases of angioleiomyoma involve the extremities; few have been described in the head and neck region, and very few of those have been reported on the ear. Among those auricular angioleiomyomas that have been reported, most were distinctly painless. We report a new, atypical case of this unusual tumor.

Introduction

Angioleiomyomas are benign tumors of vascular smooth-muscle-cell origin that typically occur in middle-aged patients. Most of these tumors appear as a painful solitary lesion on a lower extremity. Angioleiomyomas of the head and neck are uncommon, but when they do occur, they are generally painless. Very few of these lesions arise on the auricle.

In this article, we describe an unusual presentation of a cavernous-type auricular angioleiomyoma. In addition to the uncommon location of the tumor, this case was atypical in that the lesion was distinctly purple and it was painful.

Case report

A 29-year-old woman presented to the plastic surgery clinic at the University of California-Irvine Medical Center with a chief complaint of a slowly growing lesion on the antihelix of her right ear. Her primary concern was the lesion's appearance, but she also said that it was frequently painful. Most of the time she felt a chronic, dull ache in the area, but she would often experience episodes of sharp pain with pressure, especially when she slept on that ear. She also noted that the lesion often felt worse when she was under stressful conditions and during hot weather; the mass would tend to shrink slightly during cool weather. She had first noted the lesion 17 years earlier, when she was 12 years old. Her history was significant only for current tobacco use and an earlier case of depression.

Physical examination revealed the presence of a raised, well-circumscribed, easily compressible, 1.0 x 1.5-cm purple lesion on the antihelix of the right ear (figure 1, A). The size of the lesion increased slightly during Valsalva's maneuver. No thrill or bruit in the lesion was noted. The presumptive diagnosis was a venous malformation.

[FIGURE 1 OMITTED]

The patient underwent local resection of the lesion and a planned staged reconstruction; a posterior auricular tissue flap was created during the first stage of the reconstruction, and an interval flap division and inset was performed 2 weeks later. The patient tolerated both procedures well, and she experienced no complications. Her postoperative follow-up was unremarkable, and at 7 months, the cosmetic result was excellent (figure 1, B).

The excised specimen was made up of a skin ellipse with an underlying 1.0 x 1.0 x 0.6-cm reddish-brown nodule. Microscopically, the nodule was made up of a well-circumscribed proliferation of smooth-muscle bundles that surrounded somewhat dilated, blood-filled vascular spaces (figure 2). The smooth muscle did not form well-defined vascular walls; this characteristic identified the lesion as an angioleiomyoma of the cavernous type. (1,2)

[FIGURE 2 OMITTED]

Discussion

By definition, angioleiomyoma is a benign tumor derived from the smooth muscle of blood vessels. The first report of a leiomyoma arising from the wall of a blood vessel was published by Aufrecht in 1868. (3) The current classification of leiomyomas is based on the description of these lesions published by Babes in 1885. (4) More specific traits of angioleiomyomas were reported by Stout (5) in 1937, and many of these characteristics are still used for diagnosis today.

The origin of angioleiomyomas is unclear. Many authors have argued that they arise from veins. (1,3,5-7) Others believe that they are actually hamartomas, based on the presence of mature lipocytes (2,7,8) Duhig and Ayer, in their investigation of 61 angioleiomyomas, considered them to be vascular hamartomas. (8) They suggested that angioleiomyomas are produced by a proliferation of smooth muscle within a hemangioma, and that further proliferation would produce a simple leiomyoma.

Histopathology. Morimoto studied 241 cases of angioleiomyoma and separated these tumors into two groups: the larger extremity tumors and the smaller head and neck tumors. (1) He further classified them into three histologic types--capillary (or solid), cavernous, and venous:

* Tumors of the capillary type are made up of smooth-muscle bundles that surround and involve these vessels. These bundles are closely compacted, and they intersect with one another, demonstrating vascular channels that are large in number but usually very small (almost slit-like) in appearance.

* Tumors of the cavernous type are made up of dilated vascular channels. They have less total smooth-muscle volume and less distinct boundaries between the vessel walls and the perivascular smooth-muscle bundles.

* Venous-type tumors have less compact smooth-muscle bundles and very distinct smooth-muscle vascular walls.

Clinical characteristics. Morimoto's database was expanded to 562 cases, and patterns were reported by Hachisuga et al. (2) They found a male-to-female ratio of 1:1.7 and an average age at diagnosis of 47 years, with 67% of all cases occurring during the fourth, fifth, and sixth decades of life. Capillary-type tumors occurred in women more than three times as often as in men, while cavernous-type tumors appeared in men more than four times as often as in women; venous-type tumors were somewhat more common in men than in women. Lower-extremity tumors occurred twice as often in women, while head/neck and upper-extremity presentations were more common in men.

It is important to note that of the 562 tumors studied by Hachisuga et al, 500 occurred in the extremities (89.0%), including 375 in the lower extremities (66.7%); of the remainder, 48 occurred in the head and neck (8.5%) and 14 in the trunk (2.5%). (2) Of the 48 head and neck tumors, 14 occurred in the ear, 11 in the lip, 6 in the nose, 5 in the nasal cavity, 3 in the face, 2 in the mandible, and 1 each in the cheek, temporal region, zygomatic region, maxillary sinus, hard palate, larynx, and parotid gland. Pain was the most common subjective complaint, occurring primarily in patients with capillary-type tumors and in patients with tumors of the extremities; pain was uncommon in patients with head and neck tumors.

MacDonald and Sanderson studied 18 solitary tumors, 16 of which occurred in women. (6) Of these, 13 occurred near or below the knee, 3 were near the elbow, and 1 each occurred on the lower lip and nose. Neither of the facial lesions was painful.

The mechanism of pain production is unknown. Possible explanations include pressure on neurofibrils or a contraction of vessels that gives rise to local ischemia. (6) Some authors have postulated that the pain is caused by the compression of the somatic nerves that accompany the blood vessels within the tumor. (9)

Auricular angioleiomyomas are rare. Reported sites on the ear include the helix, pinna, and lobule. (10-13)

It is important to consider angioleiomyoma in the differential diagnosis of a purple, vascular-appearing lesion because this tumor can develop into its malignant counterpart, leiomyosarcoma.

References

(1.) Morimoto N. Angiomyoma (vascular leiomyoma): A clinicopathologic study. Medical Journal of Kagoshima University 1973;24: 663-83.

(2.) Hachisuga T, Hashimoto H. Enjoji M. Angioleiomyoma. A clinicopathologic reappraisal of 562 cases. Cancer 1984;54:126-30.

(3.) Aufrecht E. Ein Myom der Vena Saphena. Virchow's Archly fur pathologische Anatomie und Physiologie und fur klinische Medizin 1868;44:133.

(4.) Babes V. The myoma of the skin. In: Ziemssen BH, ed. Handbook of Diseases of the Skin. New York: William Wood and Co.; 1885:607.

(5.) Stout AP. Solitary cutaneous and subcutaneous leiomyoma. Am J Cancer 1937;29:435-69.

(6.) MacDonald DM, Sanderson KV. Angioleiomyoma of the skin. Br J Dermatol 1974;91:161-8.

(7.) Magner D, Hill DR Encapsulated angiomyoma of the skin and subcutaneous tissues. Am J Clin Pathol 1961;35:137-41.

(8.) Duhig JT, Ayer JR Vascular leiomyoma. A study of sixtyone cases. Arch Pathol 1959;68:424-30.

(9.) Montgomery H, Winkelmann RK. Smooth-muscle tumors of the skin. AMA Arch Derm 1959:79:32-40.

(10.) Inoue F, Matsumoto K. Vascular leiomyoma of the auricle. Arch Dermatol 1983;119:445-6.

(11.) Agrawal R, Singh PA, Mishra V. Angioleiomyoma pinna--a case report. Journal of Nepal Medical Association 2000;39(135). Available in pdf format at http://www.jnma.com.np/viewarticle. php?id=60 (access verified Feb. 1,2007).

(12.) Choe KS, Sclafani AR McCormick SA. Angioleiolnyoma of the auricle: A rare tumor. Otolaryngol Head Neck Surg 2001:125: 109-10.

(13.) Wang MC, Shiao AS. Auricle angioleiomyoma. Zhonghua Yi Xue Za Zhi (Taipei) 2002;65:180-2.

Garrett A. Wirth, MD; Michael J. Sundine, MD; Allen P. Kong, MD; Philip M. Carpenter, MD

From the Aesthetic and Plastic Surgery Institute (Dr. Wirth, Dr. Sundine, and Dr. Kong) and the Department of Pathology (Dr. Carpenter), University of California-Irvine Medical Center.

Reprint requests: Michael J. Sundine, MD, Aesthetic and Plastic Surgery Institute, University of California-Irvine, Manchester Pavilion, 200 S. Manchester Ave., Suite 650, Orange, CA 92868-3298. Phone: [phone]; fax: [phone]; e-mail [e-mail]