e144 © 2012 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Case Reports Ophthal Plast Reconstr Surg, Vol. 28, No. 6, 2012 Orbital Invasion by Squamous Cell Carcinoma Arising in Multiple Epidermoid Cysts Gherusa Helena Milbratz, M.D.*, Felipe Placeres Borges, M.D.*, Murilo Bicudo Cintra, M.D.†, Gyl Eanes Barros Silva, M.D.‡, and Antonio Augusto Velasco e Cruz, M.D.* *Department of Ophthalmology, †Department of Radiology, and ‡Department of Pathology, School of Medicine of Ribeirão Preto, University of São Paulo, São Paulo, Brazil Accepted for publication January 9, 2012. The authors have no financial or conflict of interest to disclose. Address correspondence and reprint requests to Antonio Augusto Velasco e Cruz, M.D., Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, Hospital das Clínicas-Campus, Av Bandeirantes 3900, 14049-900, Ribeirão Preto, São Paulo, Brazil. E-mail: aavecruz@fmrp.usp.br DOI: 10.1097/IOP.0b013e31824a48d4 superficial skin connection (Fig. 1). His left eye was exotropic with limitation of elevation and adduction. Orbital MRI disclosed a lesion in the topography of the supraorbital nerve extending from the superior orbital rim level to the cavernous sinus (Fig. 1). Biopsy of the orbital, frontal, and temporal regions was performed under general anesthesia. Several cysts in both regions were removed for pathological analysis. During biopsy, it was observed that the supraorbital nerve was grossly infil- trated. Microscopic examination revealed normal epidermoid cysts admixed with some cysts showing degeneration for squa- mous cell carcinoma. Perineural invasion was clearly seen (Fig. 2). The authors believe that their patient is the first case of perineural spreading to the orbit of this peculiar form of epi- thelial cancer. Squamous cell carcinoma arising in epidermal cysts are especially dangerous because these tumors may lack superficial connections and are thus interpreted as benign lesions. 4 Perineural spread is a common route of dissemination of epithelial malignancies of the head and neck to the central nervous system. 5 The frontal region is especially prone to this devastating complication due to the rich supply provided by the supraorbital nerve. Tumor spread through the supraorbital nerve is usually asymptomatic until the cancer reaches the cavernous sinus and infiltrates extraocular motor nerves. The left orbit of the patient was exenterated. Surgical resection extended into the supraorbital region and temporal fossa to remove all subepider- mal cysts. The authors were able to perform a 1-stage recon- struction using a combination of temporalis muscle rotation and a subcutaneous cervicofacial flap advancement. Currently, the patient is undergoing chemo- and radiotherapy. REFERENCES 1. MacKee PH. Tumors of the epidermal appendages. In: MacKee PH, Brenn T, eds. Pathology of the Skin with Clinical Correlations. 2nd edn. London: Mosby-Wolfe, 1996:15.2–15.9. 2. López-Ríos F, Rodríguez-Peralto JL, Castaño E, et al. Squamous cell carcinoma arising in a cutaneous epidermal cyst: case report and literature review. Am J Dermatopathol 1999;21:174–7. 3. Morgan MB, Stevens GL, Somach S, et al. Carcinoma arising in epidermoid cyst: a case series and aetiological investigation of hu- man papillomavirus. Br J Dermatol 2001;145:505–6. 4. Folpe AL, Reisenauer AK, Mentzel T, et al. Proliferating trichil- emmal tumors: clinicopathologic evaluation is a guide to biologic behavior. J Cutan Pathol 2003;30:492–8. 5. Goepfert H, Weber RS. Perineural invasion of skin cancer. In: Miller MJ, Goepfert H, eds. Basal and Squamous Cell Skin Cancers of the Head and Neck. Media, PA: Williams & Wilkins, 1996:133–9. Abstract: Epidermal or epidermoid cysts usually are benign, solitary-growing masses located in the mid- or lower dermis. They are believed to derive from pilosebaceous units and are lined with an epidermis-like epithelium including a granular cell layer. 1 The occurrence of multiple epidermal cysts on the scalp of nonsyndromic patients is extremely rare. Although the presence of squamous cell carcinoma in the wall of an isolated epidermoid cysts is well documented in the dermatological literature, 2,3 the authors are not aware of any article in the English literature describing orbital invasion by a carcinoma developed in isolated or multiple epidermoid cysts. CASE DESCRIPTION A 60-year-old man sought treatment for diplopia and mul- tiple subepidermal cysts on the left frontotemporal region. The patient was previously healthy, with no history of immunosup- pressive disease or human papilloma virus infection. He reported that the first cysts appeared on the temporal region 4 months ago. He noticed that the number of cysts increased and 3 months before consultation diplopia ensued. A left lateral tarsorrhaphy had been performed elsewhere 1 month ago due to orbicularis paresis. On examination, his visual acuity was 20/25 in the right eye and 20/40 in the left. There were 11 cysts 0.5 to 2.0 cm in diameter on the left frontotemporal region without any signs of FIG. 1. Left, Clinical aspect of the patient. Notice the temporal cysts and left-eye exotropia. Coronal slices of T1-weighted MRI with fat suppression: Center, Supraorbital nerve enlargement with peripheral enhancement (arrow). Right, Peripheral enhancement in the cavernous sinus (arrow).