Purpose

Patients with hereditary retinoblastoma (Rb) are predisposed to secondary cancers. Secondary malignancies are a leading cause of morbidity and mortality in such patients in contrast to other childhood cancers, which typically lead to mortality through primary tumour recurrence or progression. Sebaceous carcinoma (SC) is an uncommon associated secondary malignant neoplasm in survivors of hereditary Rb. We report a case of orbital SC in an octogenarian following bilateral Rb in infancy and discuss the approach to the diagnosis and management.

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Methods

Case report

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Results

An 82-year-old Caucasian female with a background of bilateral enucleation for Rb at 6 months of age presented to the oculoplastic service with a 6 month history of an enlarging mass within the left anophthalmic socket, precluding the use of her prosthetic eye. Further medical history included left breast adenocarcinoma managed with wide local excision with sentinel node biopsy and letrozole 2 years prior, with axillary node clearance declined by the patient. Examination and MRI identified a 20x14x16mm lesion within the left socket without intraconal fat or extraocular muscle involvement. Intraoperatively, the orbital mass was friable and gelatinous and it was found unattached to the socket but attached to the central upper eyelid. Orbital and upper eyelid biopsy confirmed moderately to poorly differentiated high grade ocular SC extending to all margins with strong androgen receptor positivity in all nuclei, with no evidence of metastatic breast carcinoma or residual Rb. Subsequent positron emission tomography, MRI and neck ultrasound did not identify residual disease or metastasis. Following Head and Neck Oncology Multidisciplinary Team discussion, wide local excision and reconstruction was recommended.

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Conclusion

In this report we highlight the association between SC and hereditary Rb. SC may mimic chronic meibomian gland disease and is frequently diagnosed late; in this case concealed within the anophthalmic socket. Surveillance for such secondary ocular malignancies in hereditary Rb is advisable at each eye clinic review.

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Additional Authors

First name	Last name	Base Hospital / Institution
Allan	Nghiem	Maidstone and Tunbridge Wells NHS Trust

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