Very late orbital recurrence of choroidal melanoma four decades post enucleation

Abstract

Orbital recurrence of malignant choroidal melanoma is quite uncommon, occurring in about 3% of patients undergoing enucleation for large tumors. Orbital recurrences after more than 10 years from enucleation are even rarer. In literature, only few reports described orbital recurrence that occurred between 10 and 40 years after primary diagnosis. Herein we report a very late choroidal melanoma recurrence, 40 years post enucleation, of a 52 year-old female who had undergone left enucleation at the age of 12. She presented to our clinic for consultation in 2017, complaining of difficulty to contain the prosthesis, as well as, presence of small pigmented palpable nodules inferiorly in her anophthalmic socket. The patient was not aware of the medical condition which had lead to the enucleation. We requested her medical reports and detailed history through her family. We realized after reviewing her charts 40 years back, that her enucleation was due to malignant choroidal melanoma (CMM). Full screening was performed. After magnetic resonance imaging (MRI), that showed the presence of nodular masses in the anophthalmic socket, an excisional biopsy was performed. Histopathology confirmed the diagnosis of CMM (epithelioid and spindle cell type), supporting the hypothesis that residual melanoma cells may remain clinically dormant for long periods, even for decades. A literature review was performed in order to review similar cases and to understand and discuss multiple factors, which may explain this extremely delayed recurrence. To the best of our knowledge, this is the third case to be reported in the literature.

Local recurrence of uveal melanoma and concomitant brain metastases associated with an activating telomerase promoter mutation seven years after secondary enucleation

“…Local recurrence of uveal melanoma is most frequently of the mixed cell type, 7 , 9 , 12 as seen in our patient. The advent of tumor genetic profiling has enabled molecular characterization of uveal melanomas and the potential derivation of insights into their pathophysiology.…”

“… 11 The presence of extrascleral extension does not seem to correlate with the time from enucleation to local recurrence, 7 which can vary widely from 3.5 months 7 to 40 years. 12 It is worth noting that our patient had no histologic evidence of extrascleral extension or optic nerve invasion at time of enucleation. Similarly, Sanke et al reported two patients with local recurrence of uveal melanoma after enucleation who did not have histological evidence of extrascleral extension.…”