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A man in his 40s with a 2-year history of recurrent sinus infections presented after 2 months of progressive vertical diplopia, blurry vision, and several weeks of medial right eye pain. He also noted more recent hypoesthesia of the right nare, anterior cheek, and right upper lip. On examination, the patient had corrected visual acuity of 20/30 on the right and 20/20 on the left without visual field deficits, and a mild afferent pupillary defect on the right. He had normal intraocular pressure and pupillary response. There was partial anesthesia of the upper lip and an abnormal Schirmer test result on the right eye. Findings from the otoscopic and rhinoscopic evaluations were normal, and the oral cavity and oropharynx were clear. A coronal noncontrast CT (Figure, A) showed a 3.7 × 2.3 × 3.4-cm soft-tissue mass expanding the bony margins of the right infraorbital nerve canal with extraconal extension into the right orbital floor, causing upward mass effect on the right inferior rectus muscle. There was inferior extension into the right maxillary sinus. Axial postcontrast fat-saturated T1-weighted MRI (Figure, B) showed heterogeneous enhancement. The patient underwent combined orbitotomy, endonasal, and sublabial approaches for partial resection of the mass, leaving that which was noted to be intimately entwined within the right infraorbital nerve fascicles. Histologic sections demonstrated a benign-appearing smooth muscle proliferation admixed with thick-walled vessels and benign adipose tissue (Figure, C). Immunohistochemical stains demonstrated the spindled cells to be positive for smooth muscle actin and negative for S100 and HMB45.
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Angioleiomyoma (ALM; synonyms: vascular leiomyoma, angiomyoma) is an uncommon, benign, slow-growing solitary tumor composed of well-differentiated smooth muscle cells arranged around vascular channels.1,2 Three common histologic subtypes have been described, including solid, cavernous, and venous type ALMs.3 About 3% of all ALMs also feature mature fat cells, which are most commonly associated with the venous histologic subtype and are located in the head and neck area; these are termed angioleiomyomas with adipocyte differentiation.2,4
ALMs typically present during the fourth to sixth decades, with tumors usually seen in the skin and subcutaneous soft tissues but also in fascial tissues and bone.1,2,5,6 About 90% are found in the extremities, particularly in the lower limbs; however, about 10% can be found in the head and neck.2,5
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Corresponding Author: Mohamad F. Bazerbashi, MD, Department of Neuroradiology, Michigan Medicine, 1500 E Medical Center Dr, Ann Arbor, MI 48109-5030 (email@example.com).
Published Online: April 12, 2018. doi:10.1001/jamaoto.2018.0178
Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported.
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