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A Painful Infraorbital Mass

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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C. Angioleiomyoma

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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Article Information

Corresponding Author: Mohamad F. Bazerbashi, MD, Department of Neuroradiology, Michigan Medicine, 1500 E Medical Center Dr, Ann Arbor, MI 48109-5030 (mbazer2@gmail.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.

References
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Hisaoka  M, Quade  B. Angioleiomyoma. In: Fletcher  CDM, Bridge  JA, Hogendoorn  PCW, Mertens  F, eds.  World Health Organization Classification of Tumours of Soft Tissue and Bone. 4th ed. Lyon, France: IARC Press; 2013:120-121.
2.
Hachisuga  T, Hashimoto  H, Enjoji  M.  Angioleiomyoma: a clinicopathologic reappraisal of 562 cases.  Cancer. 1984;54(1):126-130.PubMedGoogle ScholarCrossref
3.
Morimoto  N.  Angiomyoma: a clinicopathologic study.  Med J Kagoshima Univ. 1973;24:663-683.Google Scholar
4.
Agaimy  A, Michal  M, Thompson  LDR, Michal  M.  Angioleiomyoma of the sinonasal tract: analysis of 16 cases and review of the literature.  Head Neck Pathol. 2015;9(4):463-473. doi:10.1007/s12105-015-0636-yPubMedGoogle ScholarCrossref
5.
Liu  Y, Li  B, Li  L, Liu  Y, Wang  C, Zha  L.  Angioleiomyomas in the head and neck: a retrospective clinical and immunohistochemical analysis.  Oncol Lett. 2014;8(1):241-247.PubMedGoogle ScholarCrossref
6.
Ramesh  P, Annapureddy  SR, Khan  F, Sutaria  PD.  Angioleiomyoma: a clinical, pathological and radiological review.  Int J Clin Pract. 2004;58(6):587-591.PubMedGoogle ScholarCrossref
7.
Wang  CP, Chang  YL, Sheen  TS.  Vascular leiomyoma of the head and neck.  Laryngoscope. 2004;114(4):661-665.PubMedGoogle ScholarCrossref
8.
Yoon  TM, Yang  HC, Choi  Y-D, Lee  DH, Lee  JK, Lim  SC.  Vascular leiomyoma in the head and neck region: 11 years experience in one institution.  Clin Exp Otorhinolaryngol. 2013;6(3):171-175. doi:10.3342/ceo.2013.6.3.171PubMedGoogle ScholarCrossref
9.
Yoo  HJ, Choi  JA, Chung  JH,  et al.  Angioleiomyoma in soft tissue of extremities: MRI findings.  AJR Am J Roentgenol. 2009;192(6):W291-4.PubMedGoogle ScholarCrossref
10.
Kumar  N.  Infra-orbital nerve schwannoma: report and review.  J Nat Sci Biol Med. 2015;6(1):267-271. doi:10.4103/0976-9668.149229PubMedGoogle ScholarCrossref
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