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Pagetoid spread of sebaceous cell carcinoma to the cornea

Published:September 27, 2012DOI:https://doi.org/10.1016/j.jcjo.2012.04.017
      Sebaceous cell carcinoma (SebCC) is a malignancy that most commonly presents in the eyelid.
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • et al.
      Sebaceous carcinoma of the ocular region: A review.
      Pagetoid spread to the conjunctiva has been well documented,
      • Shields J.A.
      • Demirci H.
      • Marr B.P.
      • et al.
      Sebaceous carcinoma of the ocular region: A review.
      • Akpek E.K.
      • Polcharoen W.
      • Chan R.
      • Foster C.S.
      Ocular surface neoplasia masquerading as chronic blepharoconjunctivitis.
      • Shields C.L.
      • Shields J.A.
      Tumors of the conjunctiva and cornea.
      but spread to the cornea is less common.
      • Lin A.Y.
      • Bhorade A.M.
      • Sugar J.
      • et al.
      Neoplastic corneal pannus in sebaceous cell carcinoma.
      We report a 64-year-old female who was diagnosed with pagetoid spread of SebCC to her right cornea. She initially presented with a 3-year history of red eyes and trichiasis. After multiple epilations, she was referred to the oculoplastics service for electrolysis. On examination, her visual acuity was 20/70 in the right eye and counting fingers in the left eye (the vision was limited due to a central stromal scar and a dense cataract). She was being scheduled for cataract surgery in her right eye. Slit-lamp examination revealed bilateral, asymmetric lid findings of blepharitis and aberrant lashes on the right upper lid (RUL) (Fig. 1). Pertinent negatives included no thickening or ulceration of her lids, no lid masses, absence of an Arlt line or symblepharon, and no history of usage of glaucoma drops or topical antivirals or of trachoma or foreign travel. Even with electrolysis over several months, the patient developed worsening trichiasis. Given the lack of improvement despite medical management and the irregularity of the RUL margin, a full-thickness RUL biopsy was performed, and it returned positive for SebCC. Map biopsies were then taken in the superior, inferior palpebral, and superior and inferior bulbar conjunctiva (medial, central, and lateral positions). All superior palpebral and conjunctival biopsies were positive for SebCC. Surgical excision of the following was performed: the superior bulbar conjunctiva from 9 to 3 o'clock, the entire superior palpebral conjunctiva, and the RUL with entire tarsus. Multiple margins, including the limbus, were clear. Despite negative limbal margins intraoperatively, because the 12 to 3 o'clock peripheral cornea appeared suspicious (Fig. 2A), mitomycin C 0.04% was applied to that corneal limbal area for 2 minutes intraoperatively and then irrigated with normal saline. In addition, double freeze-thaw cryotherapy was applied to the scleral base. Metastatic investigations were negative. After several weeks, a pseudopterygium appeared from 12 to 5 o'clock. Increasing numbers of white plaques were also observed (Fig. 2B). Incisional biopsy of the pseudopterygium and scrapings of the white plaques using a 64 blade were performed. The pseudopterygium biopsy was negative, but the corneal plaque scrapings were positive for SebCC. Treatment options were discussed, including the option of exenteration. The patient opted for a more conservative approach and underwent a 4-week course of topical mitomycin-C 0.04% 4 times daily, 1 week on, 1 week off.
      Figure thumbnail gr1
      Fig. 1A 64-year-old female who presented with findings of ocular rosacea, meibomian gland dysfunction, blepharitis, and trichiasis (right eye).
      Figure thumbnail gr2
      Fig. 2(A) Pseudopterygium from 12-3 o'clock and few elevated white plaques adherent to the corneal surface, for which corneal scrapings were positive for sebaceous cell carcinoma (2 o'clock midperipheral cornea at the leading edge of the pseudopterygium). (B) Significant spread of elevated corneal plaques from 12 to 6 o'clock while awaiting results of the pseudopterygium biopsy and corneal scrapings.
      Pagetoid spread of SebCC to the conjunctival epithelium has been reported with corneal involvement in up to 18% of patients, appearing as superficial punctate keratitis, a corneal ulcer, or superior limbic keratoconjunctivitis.
      • Shields C.L.
      • Shields J.A.
      Tumors of the conjunctiva and cornea.
      • Lin A.Y.
      • Bhorade A.M.
      • Sugar J.
      • et al.
      Neoplastic corneal pannus in sebaceous cell carcinoma.
      • Chao A.N.
      • Shields C.L.
      • Krema H.
      • Shields J.A.
      Outcome of patients with periocular sebaceous gland carcinoma with and without conjunctival intraepithelial invasion.
      To our knowledge, this is the first report of SebCC with pagetoid spread over the cornea that presented as multiple elevated white plaques. Corneal scrapings may be performed in such cases. In any patient with a history of SebCC with corneal involvement, incisional cataract surgery should be avoided because disruption of the Bowman membrane may seed carcinomatous cells into the eye.

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