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Intraepithelial sebaceous neoplasia invading the lacrimal gland
  1. Department of Pathology and Ophthalmology, Erasmus University, Room Ee 993, PO Box 1738, 3000 DR Rotterdam, Netherlands

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    Editor,—Intraepithelial sebaceous neoplasia of the ocular surface without an underlying invasive carcinoma is considered to be an incipient carcinoma. These lesions can remain in situ for prolonged periods without undergoing transformation to an invasive carcinoma.1 The diagnosis of intraepithelial sebaceous neoplasia is established by map biopsies, determining the extent of intraepithelial spread, excluding an occult invasive carcinoma. This is a report of a case with extensive intraepithelial sebaceous neoplasia, with invasive growth in the lacrimal gland.


    A 77-year-old woman presented with a long term history of a chronic irritated right eye. On clinical examination the tarsal and bulbar conjunctival surface was irregular with vascular ingrowth. Full thickness biopsies from the upper and lower eyelid revealed an intraepithelial sebaceous carcinoma of the Bowenoid type. Clinically, the major part of the conjunctiva was affected and it was decided to perform an exenteration.


    Multicentric localisation of sebaceous carcinoma was found in Meibomian glands, glands of Zeis, and a diffuse intraepithelial sebaceous carcinoma (Fig 1), affecting the conjunctiva of the upper and lower eyelid. The contiguous large, atypical cells had a finely vacuolated cytoplasm with large nuclei, prominent nucleoli and a high mitotic rate. The intraepithelial extension involved more than 90% of the bulbar and tarsal conjunctiva, with extension into the epithelium of the eyelid margin, and the corneal epithelium. An intense secondary inflammation of the conjunctival stroma was noted. Although several areas of microinvasion were found, an underlying nodular invasive sebaceous carcinoma was not present. However, the intraepithelial carcinoma extended into the lacrimal duct (inset, Fig 2), and an invasive sebaceous carcinoma was found in the lacrimal gland (Fig 2), with chronic inflammation and atrophy of the surrounding lacrimal tissue. The lacrimal gland tumour measured 3 mm. Stains on intracellular mucins were negative. There was no wet tissue left to perform reliable staining on intracellular lipid. Lipid on paraffin embedded tissue is difficult to perform and interpret on conjunctival epithelium. On immunohistochemistry the cells were positive with antibodies against carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA), and negative for S100 protein and HMB-45. This findings exclude (amelanotic) primary acquired melanosis and sweat gland differentiation, and are consistent with sebaceous differentiation.1

    Figure 1

    Diffuse intraepithelial sebaceous carcinoma in the eyelid. The contiguous large, atypical cells have a finely vacuolated cytoplasm with large nuclei, prominent nucleoli, and a high mitotic rate (haematoxylin and eosin × 335).

    Figure 2

    Invasive sebaceous carcinoma (black arrow) in the lacrimal gland, with chronic atrophic inflammation of the surrounding lacrimal tissue (open arrow) (haematoxylin and eosin × 176). Inset: intraepithelial sebaceous carcinoma spreading along the lacrimal duct (haematoxylin and eosin × 133).


    Ocular sebaceous carcinoma originate from the Meibomian glands, glands of Zeis, or sebaceous glands of the caruncle or eyelid skin.2 Intraepithelial spread to conjunctiva, cornea or skin of the eyelids in a manner similar to that of mammary and extramammary Paget’s disease is frequently observed. Between 44% and 80% of ocular sebaceous carcinoma are associated with intraepithelial neoplasia34 and a high mortality.4

    In contrast, intraepithelial sebaceous neoplasia withoutan underlying nodular invasive sebaceous carcinoma has been described less frequently,156 and is associated with a prolonged clinical course.6 These cases usually spread within the epithelium over 3–10 years, causing symptoms and signs of chronic blepharoconjunctivitis. Two patterns of intraepithelial spread have been recognised: (1) pagetoid spread, consisting of large individual neoplastic cells scattered throughout all layers of the epithelium; and (2) bowenoid spread, consisting of contiguous large atypical cells that partially or fully replace the epithelium. The diffuse intraepithelial neoplasia may have resulted from an intraepithelial migration from these affected glands, or from in situ development within the conjunctival epithelium.1 The treatment of intraepithelial spread is controversial.78 When intraepithelial spread is extensive, or the involved eye has a poor visual prognosis, an orbital exenteration may be indicated.1 Intraepithelial tumour spread into the introitus of the lower lacrimal canaliculus has been described.6 A lacrimal gland origin of the sebaceous carcinoma9 seems unlikely considering the minimal size of the invasive tumour. Our patient represents a unique case of diffuse intraepithelial sebaceous neoplasia with invasive growth into the lacrimal gland. This case stresses the importance of including the lacrimal system in the surgical resection.


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