Angioleiomyoma of the Sinonasal Tract: Analysis of 16 Cases and Review of the Literature

Abstract

Angioleiomyoma (ALM; synonyms: angiomyoma, vascular leiomyoma) is an uncommon benign tumor of skin and subcutaneous tissue. Most arise in the extremities (90 %). Head and neck ALMs are uncommon (~10 % of all ALMs) and those arising beneath the sinonasal tract mucosa are very rare (<1 %) with 38 cases reported so far. We herein analyzed 16 cases identified from our routine and consultation files. Patients included seven females and nine males aged 25-82 years (mean 58; median 62). Symptoms were intermittent nasal obstruction, sinusitis, recurrent epistaxis, and a slow-growing mass. Fifteen lesions originated within different regions of the nasal cavity and one lesion was detected incidentally in an ethmoid sinus sample. Size range was 6-25 mm (mean 11). Histologically, all lesions were well circumscribed but non-encapsulated and most (12/16) were of the compact solid type superficially mimicking conventional leiomyoma but contained numerous compressed muscular veins. The remainder were of venous (2) and cavernous (2) type. Variable amounts of mature fat were observed in four cases (25 %). Atypia, necrosis, and mitotic activity were absent. Immunohistochemistry showed consistent expression of smooth muscle actin (12/12), h-caldesmon (9/9), muscle-specific actin (4/4), variable expression of desmin (11/14) and CD56 (4/6), and absence of HMB45 expression (0/11). The covering mucosa was ulcerated in 6 cases and showed squamous metaplasia in one case. There were no recurrences after local excision. Submucosal sinonasal ALMs are rare benign tumors similar to their reported cutaneous counterparts with frequent adipocytic differentiation. They should be distinguished from renal-type angiomyolipoma. Simple excision is curative.

Angioleiomyoma of Nasal Septum: Case Report and Literature Review

“…Our patient's specimen was classified as angioleiomyoma by histopathologic examination. The vascular component of this smooth muscle derived tumor may also be classified as solid, venous, or cavernous type based on histological findings [1,27]; this was not specified in our patient.…”

“…Roughly 10% of these tumors are located in the head and neck region and presenting sites include the nose, auricle, lips, and neck [1,21]. This slow growing, benign tumor may present as a solitary mass that may or may not be painful.…”

“…This slow growing, benign tumor may present as a solitary mass that may or may not be painful. Other typical presenting symptoms may include nasal obstruction, epistaxis, facial pain, recurrent sinusitis, and can be associated with a septal deviation [1]. Angioleiomyoma is not often included in the clinician's differential diagnosis of an obstructive nasal mass.…”

“…Angioleiomyoma is not often included in the clinician's differential diagnosis of an obstructive nasal mass. The differential diagnosis includes both benign and malignant tumors of the nasal cavity including inverted papilloma, nasal angiofibroma, hemangioma, hemangiopericytoma, solitary fibrous tumor, desmoid fibromatosis, peripheral nerve sheath tumors, and sinonasal sarcomas including leiomyosarcoma and rhabdomyosarcoma [1,22].…”

“…Immunohistology will reveal expression of alpha smooth muscle actin (SMA) and possibly h-caldesmon, muscle specific actin, desmin, S100, or myoglobin [1,3].…”