From the Departments of Ophthalmology (ST, BA, GP) and Pathology (NB), Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Samuray Tuncer, MD, Istanbul University, Istanbul Faculty of Medicine, Department of Ophthalmology, Ocular Oncology Service, Capa 34390, Istanbul, Turkey. E-mail: firstname.lastname@example.org
The lacrimal secretory apparatus is formed by the main lacrimal gland (orbital and palpebral lobe) and the accessory lacrimal glands (Krause and Wolfring).1–3 Lacrimal gland tissue located at any other site is considered a lacrimal gland choristoma.1
Lacrimal gland choristomas are rare.2 They have been reported in the eye and in various sites in ocular adnexa.1–8 The most common sites are the bulbar conjunctiva and the limbus.3 Orbital involvement is relatively uncommon.1,2 They have also been found rarely in the tarsal conjunctiva,4 under the nasal mucosa,5 in the eye,8 and on the eyelids.9
We report the clinical and pathologic features of a solitary conjunctival lacrimal gland choristoma in a 34-year-old woman.
A 34-year-old woman presented with a painless, pinkish epibulbar conjunctival mass on her left eye. She stated that the lesion had been present since birth and had gradually enlarged over the past 3 years. She denied any trauma or pain. There was no history of any rheumatologic, inflammatory, or autoimmune disease.
The ophthalmic examination showed that the right eye was entirely normal with 20/20 visual acuity. The visual acuity was 20/20 in the left eye and slit-lamp examination displayed an elevated, pinkish, limbal lesion at the 2-o’clock position, measuring 7 × 4 × 2 mm (Fig. 1). Just beneath this main lesion, there was also another small island of pinkish mass at the 3-o’clock position, measuring 1 × 1 × 1 mm (Fig. 1). The main lesion showed a 2-mm tongue-like projection over the peripheral cornea causing dellen formation. Dilated episcleral feeder vessels were present superior and temporal to the lesion.
Figure 1. Color Anterior Segment Photograph of the Left Eye Showing an Elevated, Pinkish Limbal Lesion. A Similar Small Island of Tissue Is Noted Beneath This Main Lesion. Dilated Episcleral Feeder Vessels Were Present Superior and Temporal to the Lesion.
We removed the conjunctival lesion by excisional biopsy under local anesthesia. The histopathological examination was consistent with conjunctival lacrimal gland choristoma (Fig. 2). There was no recurrence after 1 year of follow-up.
Figure 2. Histopathologic Examination Showed the Presence of Ectopic Lacrimal Gland Tissue (the Portion Between the Thick Arrows). The Thin Arrow Shows the Normal Conjunctival Epithelium (hematoxylin–Eosin, Original Magnification ×40).
The lacrimal gland begins to develop in the lower eyelid at the end of the second month of intrauterine life. It originates from five to eight epithelial buds derived from basal conjunctival cells. The gland first moves toward the lateral canthus and then into the upper eyelid. Orbital and palpebral parts are formed in the fifth month, but the full differentiation of the lacrimal gland occurs 3 to 4 years after birth.2 The accessory lacrimal glands are formed by the invagination of the conjunctiva. Any disarrangement in this normal process of migration may cause formation of a lacrimal gland choristoma.
In the literature, most lesions containing ectopic lacrimal gland tissue have been reported to occur in the temporal epibulbar conjunctiva.1,6 This supports the presumptions that the lacrimal gland choristomas originate from the palpebral lobe of the lacrimal gland.3,6 In our case, the tumor showed the same quadrantic (ie, superotemporal quadrant) location with the palpebral lobe of the lacrimal gland.
Lacrimal gland choristoma can be seen as a solitary mass or can be part of a complex choristoma in conjunction with a variety of mesodermal tissues such as nerve, muscle, and cartilage.6,7
The differential diagnosis of conjunctival lacrimal gland choristoma includes dermoids, lipodermoids, prolapse of the palpebral lobe of the lacrimal gland, herniated orbital fat, pyogenic granuloma, lymphoid infiltration, lymphangioma, and hemangioblastoma. The presence of a painless lesion since birth, epibulbar location, and pink color may help us in the differential diagnosis of a lacrimal gland choristoma. The exact final diagnosis can only be made histopathologically.
Lacrimal gland choristomas of the conjunctiva are extremely rare. This entity should be considered in the differential diagnosis of the congenital, limbal, and pinkish lesions of the conjunctiva.
- Green WR, Zimmerman LE. Ectopic lacrimal gland tissue: report of eight cases with orbital involvement. Arch Ophthalmol. 1967;78:318–327.
- Rush A, Leone CR Jr, . Ectopic lacrimal gland cyst of the orbit. Am J Ophthalmol. 1981;92:198–201.
- Alyahya GA, Bangsgaard R, Prause JU, Heegard S. Occurrence of lacrimal gland tissue outside lacrimal fossa: comparison of clinical and histopathological findings. Acta Ophthalmol Scand. 2005;83:100–103. doi:10.1111/j.1600-0420.2005.00365.x [CrossRef]
- Lee WR, Aitken DA, Kirkness CM. Diffuse hyperplasia of intratarsal ectopic lacrimal gland tissue. Arch Ophthalmol. 2002;120:1748–1752.
- Pe’er J, Ilsar M. Ectopic lacrimal gland under the nasal mucosa. Am J Ophthalmol. 1982;94:418–419.
- Pokorny KS, Hyman BM, Jakobiec FA, Perry HD, Caputo AR, Iwamoto T. Epibulbar choristomas containing lacrimal tissue: clinical distinction from dermoids and histologic evidence of an origin from the palpebral lobe. Ophthalmology. 1987;94:1249–1257.
- Duncan JL, Golabi M, Fredrick DR, et al. Complex limbal choristomas in linear nevus sebaceous syndrome. Ophthalmology. 1998;105:1459–1465. doi:10.1016/S0161-6420(98)98029-0 [CrossRef]
- Hunter WS. Aberrant intra-ocular lacrimal gland tissue. Br J Ophthalmol. 1960;44:619–625. doi:10.1136/bjo.44.10.619 [CrossRef]
- Cheng PH. Pleomorphic adenoma of lid. Chin Med J (Engl). 1964;83:49–51.