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A white man in his 70s presented with a gradually enlarging right supraorbital mass that had been neglected for 4 years. His comorbidities included congestive heart failure, atrial fibrillation, coronary artery disease, chronic obstructive pulmonary disease, hypertension, and a 50-pack-year history of tobacco abuse. Physical examination revealed a firm, nonmobile, nontender, bulging mass situated over the right frontal sinus, which expanded to the upper right eyelid. The mass covered the globe completely, resulting in total vision obstruction (Figure, A). However, the patient’s vision remained intact when the mass was elevated off the eye with great effort. His right pupil was round and reactive to light without afferent pupillary defect. No palpable lymphadenopathy was noted. A non–contrast-enhanced computed tomography (CT) scan demonstrated a large, right supraorbital mass with bony destruction of the anterior table of the right frontal sinus (Figure, B). It extended into the superior aspect of the orbit, displacing the right globe inferiorly. Air was seen within the mass (Figure, C). A fine-needle aspiration biopsy yielded inconclusive results. After medical clearance, the patient was taken to the operating room for a wedge excisional biopsy with decompression of the right supraorbital mass under monitored anesthesia care. Histopathological investigation revealed large nests of neoplastic epithelioid cells surrounded by fibrotic tissue and necroinflammatory debris. The neoplastic cells contained round nuclei with single prominent nucleoli and abundant clear to vacuolated cytoplasm (Figure, D).
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D. Sebaceous carcinoma
Sebaceous carcinoma (SC) is an aggressive, malignant epithelial tumor with a variable presentation and occasional multicentric origin due to the widespread distribution of sebaceous glands in the skin.1- 3 Sebaceous carcinoma encompasses 0.67% of all tumors of the eyelid, and is the third most common cancer of the eyelid (3%) behind basal cell carcinoma (86%) and squamous cell carcinoma (7%).2,4- 6 The majority of sebaceous carcinomas occur in the intratarsal Meibomian and Zeis glands (38.7%), with other locations noted in the literature including the trunk, scalp, neck, upper limbs, lower limbs, and genitalia.5- 8 An SC typically presents as a painless, firm, and rounded mass with a yellow hue due to its lipid content.2 It can also present as treatment unresponsive unilateral blepharitis or as a pedunculated lesion with keratinization and ulceration similar to a cutaneous horn or basal cell carcinoma.7 One clinical feature that would suggest SC over its benign mimics is a diffuse loss of cilia, which is typically seen in malignant lesions.1,7 Other lesions to add to the differential diagnosis include basal cell carcinoma, cutaneous horn, blepharitis, squamous cell carcinoma, Pott puffy tumor, chalazion, hordeolum, and sebaceous adenoma. Sebaceous carcinoma tends to be locally aggressive and has been known to invade the orbit and cause orbital displacement.1 Invasion of the paranasal sinuses has been noted only once to our knowledge, involving the maxillary sinus and ethmoids.2 To the our knowledge, there have been no reports of an aggressive SC that has invaded the frontal sinus alone, as seen in this case. As seen in the Figure, B, this SC invaded the anterior wall of the frontal sinus with noted air pockets, and because of its location, appeared initially similar to a Pott puffy tumor. To distinguish SC from Pott puffy tumor, a biopsy with corresponding histological analysis was required (Figure, D). Final pathological findings revealed large pleomorphic cells with brisk mitotic activity, vacuolated cytoplasm, and zones of necrosis, which are consistent with SC.1,2 Extensive necrotic areas were noted without normal epithelial or stromal tissues, suggesting that the tumor’s rapid growth outpaced its vascular supply. Oil-red-O staining can be adjunctively used to demonstrate the large lipid components of these cells.1,2
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Corresponding Author: Cang T. Nguyen, DO, Bon Secours Mercy Health, Department of Otolaryngology, St Elizabeth Boardman Hospital, 8401 Market St, Boardman, OH 44512 (firstname.lastname@example.org).
Published Online: March 7, 2019. doi:10.1001/jamaoto.2019.0016
Conflict of Interest Disclosures: None reported.
Additional Contributions: The authors would like to thank Kyle Quillin, DO, and Stephen Reynolds, DO, for the clinical photographs. They received no compensation for their photographs.
Additional Contributions: We thank the patient’s family for granting permission to publish this information.
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