Angioleiomyoma in a Rare Location

Angioleiomyomas are benign smooth muscle tumors that arise from the tunica media of blood vessels.¹ They tend to present in women in their 4th to 6th decades of life and most commonly occur on the extremities, particularly the lower extremities, but can also appear on the trunk, head, and neck.² Angioleiomyomas are often painful, having been described as paroxysmal, drilling, and/or radiating in nature.³ The treatment of choice is typically surgical excision, with recurrence being rare.² Here, we present a rare case of a biopsy-proven angioleiomyoma of the right nasal ala.

CASE REPORT

A 65-year-old Caucasian female patient presented to our clinic for evaluation of a nodule on her nose that was intermittently painful and cosmetically bothersome to her. On physical examination, there was a 6-mm bluish papule to the right nasal tip. A punch excision was performed to rule out a malignant vascular lesion. Hematoxylin and eosin stain revealed a deep dermal nodule composed of vascular smooth muscle tissue with ectatic vascular spaces and without cytologic atypia (Figures 1 and 2). A Desmin stain was performed, which was positive, confirming the tumor was of muscular origin, and the lesion was signed out as an angioleiomyoma (Figure 3).

Figure 1. Hematoxylin and eosin stain showing deep dermal nodule composed of vascular smooth muscle tissue with ectatic vascular spaces.

Figure 2. Hematoxylin and eosin stain showing vascular smooth muscle cells without cytologic atypia.

Figure 3. Positive Desmin stain, confirming tumor is of muscular origin.

Treatments were initially nonsurgical in nature and included topical timolol, intralesional triamcinolone (Kenalog, Bristol-Myers Squibb), and intralesional onabotulinum toxin A (Botox, Allergan Aesthetics). Onabotulinum toxin A, thought to reduce pain by reducing local ischemia via inhibition of smooth muscle contraction in blood vessels,⁴ was administered every three to six months and was effective in temporarily reducing the pain, but did not slow the tumor’s growth. Over the next five years, the lesion increased in size to 28 mm and involved multiple cosmetic subunits, including the right nasal supratip, right nasal ala, and right supra-alar crease (Figures 4 and 5). The patient opted for more aggressive interventions, as her pain was worsening, and she was beginning to develop congestion on the right side.

Figure 4. Frontal view of nasal angioleiomyoma 5 years after initial presentation.

Figure 5. Nodule measuring 28 mm overlying right nasal supratip, ala, and supra-alar crease.

Surgery on the nose involving multiple subunits is inherently difficult, due to the complex anatomy and delicate functionality and relationship of the external and internal nose. During surgical planning, special consideration was given to the collapsed internal nasal valve, as an excision would require a flap and margin evaluation. The resulting scar tissue, if not appropriately repaired, could further compromise this valve, and worsen the patency of the nasal canal.

Our initial attempt using intralesional cryosurgery failed. As angioleiomyomas are cavernous lesions, we opted to pursue a minimally invasive technique, with the goal of inducing ischemia and necrosis in the tumor. We first placed a purse-string suture along the outermost periphery of the lesion using 5-0 polyglactin suture 910 (Vicryl Rapide, Ethicon) (Figure 6). Then, a needle-tipped Hyfrecator (ConMed) was used to cauterize the tumor until even destruction was noted (Figure 7). The patient returned for a wound check three weeks later, at which point uniform necrosis was noted along the surface of the lesion. Another purse-string suture was placed at that time, after which intralesional cryotherapy was performed. A locked, single-horizontal mattress suture was also placed in the center to ligate any residual feeding vessels.

Figure 6. Purse-string suture using 5-0 Vicryl Rapide applied to periphery of tumor.

Figure 7. Needle-tipped electrocautery administered to tumor until even destruction noted.

Six weeks after the initial procedure, full-thickness, uniform necrosis of the entire lesion was noted (Figure 8). The scab was removed, revealing a flat, heme-crusted surface (Figure 9). The area continued to heal over the next several months and was completely healed at seven months postop. Superior cosmetic results were obtained with only a small, residual stellate scar present to the anterior alar crease (Figure 10). The patient regained full patency of her right nostril and was pleased with the appearance and overall outcome of the procedure (Figure 11).

Figure 8. Six-week postoperative visit showing full-thickness, uniform necrosis.

Figure 9. Scab removed to reveal smooth, heme-crusted surface underneath.

Figure 10. Small stellate scar present to anterior alar crease at 7-month postoperative visit.

Figure 11. Normal patency of right nasal canal 7 months post surgery.

DISCUSSION

Given the cosmetically sensitive location of this tumor on the nose and the inherent difficulty excising lesions in this area, several nonsurgical treatments were initially employed, including the use of topical beta blockers, intralesional corticosteroids, and intralesional neurotoxins, all of which were unsuccessful in halting tumor growth. The lesion increased in size to the point that the patient had difficulty breathing out of the right side of her nose. A minimally invasive technique using serial purse-string sutures applied to the periphery of the tumor, combined with intralesional cryotherapy and electrocautery, led to full-thickness necrosis of the tumor over a period of six weeks. The procedure led to complete resolution of the tumor with minimal scarring and return of patency of the right nasal canal. The patient has not had recurrence of the tumor one year after treatment and is pleased with the overall functional and cosmetic outcomes of the procedure.

This case outlines the utility of serial purse-string suture application combined with intralesional destruction to treat angioleiomyomas in cosmetically sensitive areas that would be difficult to treat with excision alone. Use of this technique allowed for avoidance of complications that may arise from surgical excision which, depending on the repair method, may include excess scar tissue that further compromises the nasal valve, flap necrosis, and blunting of the alar-facial groove, among others. There was concern that the patient would have significant pain in the postoperative period while the tumor was undergoing necrosis. However, her pain was mild and controlled with only a few days of over-the-counter pain medication.

CONCLUSION

Serial purse-string suture application combined with intralesional destructive methods can be considered as a treatment for well-circumscribed smooth muscle tumors such as angioleiomyomas in areas not amenable to surgical excision only after a biopsy has been performed to rule out other muscular, vascular, lipomatous, or fibrohistiocytic malignant neoplasms. The technique results in superior cosmetic and functional outcomes.

The authors have no relevant disclosures.

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