Case Reports Primary Transitional Cell Carcinoma of the Conjunctiva Ashraf A. Khan, F.R.C. OPHTH*, James R. Cameron, F.R.C. OPHTH*, Antonia Torgersen, F.R.C. PATH†, Fiona Roberts, F.R.C. PATH‡, and Jan Kerr, F.R.C. OPHTH* Abstract: A 77-year-old male presented with a large papillomatous conjunctival lesion on his lower eyelid. Biopsy and extensive systemic investigation revealed this to be a primary conjunctival transitional cell carcinoma. Patient preference and coexisting medical problems dictated conservative management with surgical debulking, topical mitomicin, and radiotherapy. Local control has been maintained for 4 years to date.
T
ransitional cell carcinoma (TCC) is a nonkeratinizing variant of squamous cell carcinoma. TCC comprises 2–11% of paranasal sinus tumors1,2 and there are 38 published reports of TCC arising in the lacrimal sac.3 Primary TCC of the conjunctiva has not to the authors knowledge previously been reported in the English literature, with only 1 previous French language report.4 TCC of the bladder and urological tract, although morphologically similar, is considered a separate entity.
FIG. 1. Appearance of right lower eyelid on presentation revealing florid papillomatous lesion.
CASE REPORT The authors report the case of a 77-year-old male who presented in 2010 with a 6-month history of right lower eyelid swelling and pain. His past medical history included treated pulmonary TB, alcohol excess with recurrent falls and head trauma, CVA and bilateral femoral fractures. He was wheelchair bound with a profound kyphosis, fixed flexion deformity of the hips, and a long-term catheter in situ. On examination his best corrected visual acuity was 6/18 OD, and 6/12 OS. Examination of the eyelid revealed a florid papillomatous swelling with prominent vessels, involving the temporal palpebral conjunctiva and extending deeply into the lower fornix (Fig. 1). The medial palpebral conjunctiva and lacrimal puncta were normal. A biopsy of the lesion was performed. Histology revealed a papillary tumor with signs of inverted growth pattern and areas of frank invasion consistent with TCC (Fig. 2). Initially, metastatic disease was suspected and he was referred for urological review. CT scan of head, chest, and abdomen revealed no other tumors and no evidence was found of a primary bladder TCC. A working diagnosis of primary TCC of the conjunctiva was reached. The patient was strongly in favor of conservative management for this. Radiotherapy was not thought to be possible because of difficulties with positioning and therefore debulking surgery was undertaken with postoperative mitomycin 0.04% drops. The tumor was found to be adherent to the globe and extending posteriorly. Clinically, much of the surface of the *Department of Ophthalmology, Princess Alexandra Eye Pavilion, Edinburgh; †Department of Neuropathology, Western General Hospital, Edinburgh; and ‡Department of Pathology, Southern General Hospital, Glasgow, United Kingdom. Accepted for publication January 17, 2015. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Ashraf A. Khan, F.R.C. Ophth, Department of Ophthalmology, Princess Alexandra Eye Pavilion, Edinburgh, EH3 9HA, UK. E-mail:
[email protected] DOI: 10.1097/IOP.0000000000000464
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2015
FIG. 2. Papillary tumor with inverted growth pattern and surrounding inflammation (H&E stain).
tumor was soft and friable, with fibrovascular material in the deeper, adherent part. After a very good initial response to mitomycin the tumor recurred. Exenteration was offered to the patient but he opted for further debulking surgery and a second course of mitomycin 0.04% drops in the knowledge that local recurrence was likely. Two years on from his initial presentation following further local recurrence but with no signs of metastatic disease, radiotherapy was reconsidered. Challenges posed by positioning were overcome and 5 fractions of radiotherapy delivering an anterior 6 MV photon field was performed. A dramatic response was observed with complete resolution of the tumor over 8 weeks. Eighteen months following radiotherapy, the treated area remained disease free; however, changes were noted at the medial end of the eyelid, outwith the original area of treatment. A second course of radiotherapy was undertaken with a single fraction of 100 kV photons to the right lower eyelid. There was subsequent improvement of the clinical appearance over 1 month which has been maintained for 3 months.
DISCUSSION Conjunctival mucosa is stratified columnar epithelium with goblet cells. There are areas of transitional epithelium at the eyelid margin and at the limbus.
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Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2015
Case Reports
Primary TCC of the conjunctiva appears to be extremely rare with only 1 previous case report; however, it is possible that the incidence is under-reported. Basaloid squamous tumors of the conjunctiva may have previously been classed as squamous cell carcinoma rather than TCC accounting for a shortfall in the histological diagnosis of TCC. Histologically, the tumor is characterized by a papillary appearance with an irregular stratified epithelium, invasive growth with conspicuous mitotic activity, and marked cellular pleomorphism.5 The only previous reported case of primary TCC of the conjunctiva was treated surgically and no follow-up data was given.4 Primary TCC, more common in the sinonasal tract, may also occur in the lacrimal sac although there are relatively few reported cases compared with other epithelial malignancies of the lacrimal system.6–10 Unfortunately, TCC of the lacrimal sac, despite extensive resection and radiotherapy, has a 50% recurrence rate11 and a 5-year mortality rate as high as 100%.12 Clinically, the patient’s tumor did not involve the lacrimal sac or punctum. Orbital metastasis from primary bladder TCC has previously been observed.13 In the patient, there was no evidence of a primary tumor of his urological tract. The patient had local regrowth of tumor 4 months after debulking surgery. Topical mitomycin C produced a rapid reduction in tumor bulk but again regrowth occurred within 6 months. There has been an excellent response to radiotherapy so far and the patient remains content with his decision to pursue conservative treatment. Primary TCC of the conjunctiva is extremely rare. It is aggressive and local recurrence is likely without complete excision. In the patient, the tumor was highly responsive to radiotherapy and this may represent an alternative treatment option in appropriate patients.
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© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.