https://doi.org/10.1177/2515841419844087 25158414191810.1177/2515841419844087 Ther Adv Ophthalmol 2019, Vol 11: 1–8 DOI: 10.1177/ 2515841419844087 © The Author(s), 2019. Article reuse guidelines: sagepub.com/journals- permissions Therapeutic Advances in Ophthalmology journals.sagepub.com/home/oed 1 Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). Introduction Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant sudoriferous gland tumor that is related to its more common, benign counterpart, syringocystadenoma papilliferum (SCAP). Since the original description of SCAP in 1917, 1 only 43 cases of SCACP have been described in the litera- ture. To date, only one has appeared in the eyelid. 2 SCACP is thought to develop from SCAP, nevus sebaceous, and linear nevus verrucosus lesions. 3 However, due to the rarity of this tumor, little is known regarding its etiology and origin. 3 In this study, we report the first case of SCACP with orbital involvement. Interestingly, it recurred following exenteration. An informed written con- sent was obtained from the patient for the publi- cation of medical data and images. Case report A 63-year-old man presented with a lesion on the right upper eyelid that had been present for 7 years. The lesion was nodular, measuring 5.0 cm × 7.0 cm, ulcerated, indurated, and erythema- tous. It involved the lower eyelid (Figure 1). The patient had no light perception with the right eye and had a visual acuity of 20/20 on the left. Due to the presence of the tumor over the right eye, his intraocular pressure could not be measured, and it was found to be 18 mmHg on the left. The left orbital examination did not reveal any abnormalities. A full examination of his local lymph nodes and lacrimal duct did not reveal any abnormalities. He explained that he did not have any previous therapy for this lesion. He was otherwise systemically well with no relevant family history. He did not have any history of trauma and informed us that he was a farmer by occupation. A computed tomography (CT) scan of the orbit revealed right anterior orbital invasion with no bony or lacrimal gland involvement (Figure 2). A subsequent incisional biopsy revealed squamous cell invaginations extending from the epidermis into the dermis. The invaginations and papillary projections were lined with a bilayer epithelium: the luminal layer was composed of columnar cells with decapitation secretion and the outer layer was composed of small cuboidal cells. These cells had significant nuclear pleomorphism, prominent nucleoli, and increased mitotic activity (Figure 3). Immunohistochemical staining demonstrated positivity for epithelial membrane antigen (EMA), Syringocystadenocarcinoma papilliferum with orbital invasion: a case report with literature review Carla Pagano Boza, Joaquin Gonzalez-Barlatay, Shoaib Ugradar , Melina Pol and Eduardo Jorge Premoli Abstract: We present a case of Syringocystadenocarcinoma papilliferum that originated in the eyelid and extended into the orbit. These tumors are very rare and have the potential to metastasize. A literature review of all the previous cases has been compiled from the Medline, EMBASE, and PubMed databases. We found that the majority of cases present on the head and neck and up to 17% of cases showed metastatic progression. This is the first case to show orbital involvement and highlights the need to remain vigilant with such lesions, as they have a tendency to become aggressive. Keywords: eyelid, orbit, Syringocystadenocarcinoma papilliferum Received: 19 October 2018; revised manuscript accepted: 21 March 2019. Correspondence to: Carla Pagano Boza, Oculoplastics Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina. Email: CPagano87@gmail.com Carla Pagano Boza Joaquin Gonzalez- Barlatay Oculoplastics Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina Shoaib Ugradar Division of Orbital and Ophthalmic Plastic Surgery, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA, USA Melina Pol Pathology Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina Eduardo Jorge Premoli Oculoplastics Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina 844087OED 0 0 10.1177/2515841419844087Therapeutic Advances in OphthalmologyC Pagano Boza, J Gonzalez-Barlatay case-report 2019 Case Report