Proton Therapy of a Conjunctival Carcinoma in the Anophthalmic Socket, 41 Years after Enucleation for a Sporadic Retinoblastoma. A Case Report and Review of the Literature

Précis

We document here an aggressive carcinoma arising in the conjunctiva of an anophthalmic socket, 41 years after enucleation for a sporadic, non-hereditary unilateral retinoblastoma. This tumor was treated for the first time with proton beam irradiation of the orbit. Investigations revealed an extensive metastatic spread to the loco-regional lymph nodes, requiring complementary parotidectomy, chemotherapy, and radiation therapy.

The incidence of conjunctival carcinoma arising in the bulbar conjunctiva peaks in Africa (3.4/100 000/year) and has been linked to factors contributing to its development, such as UV light exposure, as well as HIV or HPV infections [1]. On the contrary, carcinomas arising in the conjunctiva of anophthalmic sockets are extremely rare and, so far, only 23 cases have been documented, usually occurring a long time after enucleation. We document here the clinicopathological findings of a conjunctival carcinoma identified in the anophthalmic socket of a patient enucleated 41 years ago for a sporadic, unilateral retinoblastoma.

Publication History

Received: 25 September 2020

Accepted: 25 January 2021

Article published online:
30 April 2021

• References

• 1 Gichuhi S, Sagoo MS, Weiss HA. et al. Epidemiology of ocular surface squamous neoplasia in Africa. Trop Med Int Health 2013; 18: 1424-1443 doi:10.1111/tmi.12203
• 2 Nguyen J, Ivan D, Esmaeli B. Conjunctival squamous cell carcinoma in the anophthalmic socket. Ophthalmic Plast Reconstr Surg 2008; 24: 98-101 doi:10.1097/IOP.0b013e31816381fa
• 3 Gaier ED, Jakobiec FA, Stagner AM. et al. PV16-Positive Invasive Conjunctival Squamous Cell Carcinoma in an Anophthalmic Socket. Ophthalmic Plast Reconstr Surg 2017; 33: S2-S4 doi:10.1097/IOP.0000000000000521
• 4 Campanella PC, Goldberg SH, Erlichman K. et al. Squamous cell tumors and ocular prostheses. Ophthalmic Plast Reconstr Surg 1998; 14: 45-49 doi:10.1097/00002341-199801000-00010
• 5 Whittaker KW, Trivedi D, Bridger J. et al. Ocular surface squamous neoplasia: report of an unusual case and review of the literature. Orbit 2002; 21: 209-215 doi:10.1076/orbi.21.3.209.7173
• 6 Endo T, Hata J, Togashi S. et al. Conjunctival squamous cell carcinoma of the orbit 40 years after enucleation. Ophthalmic Plast Reconstr Surg 2006; 22: 299-301 doi:10.1097/01.iop.0000222521.92518.ee
• 7 Chaudhry TA, Memon M, Ahmad K. Use of artificial eye and conjunctival squamous cell carcinoma. J Postgrad Med 2006; 52: 234-235
• 8 Hsu VJ, Agarwal MR. Re: “Conjunctival squamous cell carcinoma in the anophthalmic socket”. Ophthalmic Plast Reconstr Surg 2009; 25: 257-258 doi:10.1097/IOP.0b013e3181a41faf
• 9 Barrett RV, Meyer DR, Carlson JA. Conjunctival squamous cell carcinoma in situ in the anophthalmic socket. Ophthalmic Plast Reconstr Surg 2010; 26: 52-53 doi:10.1097/IOP.0b013e3181b8e3bc
• 10 Espana EM, Levine M, Schoenfield L. et al. Ocular surface squamous neoplasia in an anophthalmic socket 60 years after enucleation. Surv Ophthalmol 2011; 56: 539-543 doi:10.1016/j.survophthal.2011.06.005
• 11 Oka S, Tsuboi S, Morita C. et al. Two cases of conjunctival squamous cell carcinoma in the anophthalmic socket after treatment of retinoblastoma. J Dermatol 2018; 45: e346-e347 doi:10.1111/1346-8138.14490
• 12 Shields CL, Kancherla S, Bianciotto CG. et al. Ocular surface squamous neoplasia (squamous cell carcinoma) of the socket: management of extensive tumors with interferon. Ophthalmic Plast Reconstr Surg 2011; 27: 247-250 doi:10.1097/IOP.0b013e318203d605
• 13 Hayashi A, Komoto M, Matsumura T. et al. Conjunctival Squamous Cell Carcinoma due to Long-term Placement of Ocular Prosthesis. Plast Reconstr Surg Glob Open 2015; 3: e325 doi:10.1097/GOX.0000000000000299
• 14 McGrath LA, Salvi SM, Sandramouli S. et al. Squamous cell carcinoma in the anophthalmic socket: a series of four cases with HPV-16 profiling. Br J Ophthalmol 2018; DOI: 10.1136/bjophthalmol-2018-311916.
• 15 Jain RK, Mehta R, Badve S. Conjunctival squamous cell carcinoma due to ocular prostheses: a case report and review of literature. Pathol Oncol Res 2010; 16: 609-612 doi:10.1007/s12253-010-9251-0
• 16 Kim JH, Lee MJ, Choung HK. et al. Conjunctival cytologic features in anophthalmic patients wearing an ocular prosthesis. Ophthalmic Plast Reconstr Surg 2008; 24: 290-295 doi:10.1097/IOP.0b013e3181788dff
• 17 Manoharan S, Nagaraja V, Eslick GD. Ill-fitting dentures and oral cancer: a meta-analysis. Oral Oncol 2014; 50: 1058-1061 doi:10.1016/j.oraloncology.2014.08.002
• 18 Gichuhi S, Ohnuma S, Sagoo MS. et al. Pathophysiology of ocular surface squamous neoplasia. Exp Eye Res 2014; 129: 172-182 doi:10.1016/j.exer.2014.10.015
• 19 Marees T, Moll AC, Imhof SM. et al. Risk of second malignancies in survivors of retinoblastoma: more than 40 years of follow-up. J Natl Cancer Inst 2008; 100: 1771-1779 doi:10.1093/jnci/djn394
• 20 Cruzado-Sanchez D, Tellez WA, Villarreal-Aguilar B. et al. Conjunctival squamous cell carcinoma: prognostic factors for the recurrence and metastasis and clinicopathological characteristics at an oncological hospital in Peru. Br J Ophthalmol 2020; 104: 1010-1015 doi:10.1136/bjophthalmol-2019-314058
• 21 Miller CV, Wolf A, Klingenstein A. et al. Clinical outcome of advanced squamous cell carcinoma of the conjunctiva. Eye (Lond) 2014; 28: 962-967 doi:10.1038/eye.2014.79