A patient who had the contents of her right eye surgically removed at 14 returned to a Shenzhen hospital nine years later with chronic tearing and intermittent bloody discharge. Imaging found a mass sitting above her orbital implant. When surgeons opened the socket, they found two.
Both were malignant melanoma. Neither had spread from anywhere else. And the likeliest clue to where they came from had been visible on her scalp her entire life.
The case, published in January 2026 in Frontiers in Medicine by clinicians at Shenzhen Eye Hospital and affiliated institutions, is described by its authors as the first documented instance of primary orbital melanoma following evisceration for a congenital corneal staphyloma, and the only reported case of two such tumors found in one orbit at first diagnosis.
An Operation That Removes the Eye's Contents but Not Its Shell
In July 2012, the patient was referred for a right eye that had been blind since birth and kept enlarging. Her left eye tested at 20/20. The right eye had no light perception, a fully opaque lens, and a globe wall thickened enough that ultrasound could not distinguish retina from choroid.
Surgeons performed an evisceration, which removes the internal contents of the eye while leaving the outer scleral shell in place, and inserted a porous polyethylene implant. Pathology confirmed corneoscleral staphyloma and proliferative vitreoretinopathy. No tumor cells were present.
She also had multiple blue nevi. The largest, roughly 4.5 centimeters across, sat on her right parietal scalp, with more than a dozen smaller ones scattered across her lower back and right thigh. Blue nevi are benign skin growths made of pigment cells trapped deep in the dermis during fetal development. Their color is an optical effect, not a different pigment.
What Surgeons Actually Pulled Out of the Socket
MRI in 2021 showed a 1.9 by 1.5 by 1.5 centimeter oval mass above and behind the implant, bright on T1 and dark on T2, the signature pattern melanin produces. The inferior rectus muscle looked thickened.
Surgery yielded two masses. Below the implant sat a strip-shaped lesion measuring 41 by 23 by 8 millimeters, partly black and partly flesh colored, unencapsulated, firm. Above it sat a 20 by 18 by 17 millimeter sphere that was black throughout.
Pathology confirmed malignant melanoma in both, with tumor cells staining strongly for S-100, Melan-A, and HMB-45. A proliferation marker came back at roughly 5 percent, and comprehensive next-generation sequencing found no pathogenic variants tied to her presentation. PET-CT showed no metastasis.
The two tumors likely had different origins, the authors argue. Pathology showed the lower one contained melanocytic cells mixed with muscle tissue, consistent with an inferior rectus muscle that had undergone malignant transformation. The upper one appeared to arise from pigment cell nests in the orbit itself.
A Cancer So Rare Most Ophthalmologists Never See One
Primary orbital melanoma accounts for about 1 percent of orbital tumors. The 2026 report cites fewer than 100 documented cases worldwide. An earlier tertiary center series in Frontiers in Oncology put the published total at roughly 50 as of 2017, which gives a sense of how thin the literature remains. That series followed 13 patients and reported that five died of the disease, a reminder that the tumor is dangerous as well as rare. A national database analysis has since drawn on US registry data to describe how patients present.
Between 39 and 90 percent of reported cases have been associated with blue nevi or congenital melanosis, according to a comprehensive review in Orbit. Melanocytes and blue nevus cells both descend from neural crest cells that migrate through the embryo, and some get stranded along the way. That shared lineage is why the authors treat her scalp and back nevi as a plausible marker of underlying risk rather than a coincidence.
They stop short of proving it. No biopsy of the scalp or lumbar nevi was performed. Surgeons had planned to excise the scalp lesion years earlier but abandoned it because of its size, and after the melanoma diagnosis the patient and her family declined a biopsy. No orbital imaging was done before the 2012 evisceration, which the authors call a regrettable gap.
The Surgical Argument Buried in the Conclusion
The recommendation that emerges is technical but consequential. Evisceration leaves the scleral shell and can leave residual uveal tissue behind, meaning pigment cells stay in the orbit. Enucleation removes the entire globe.
The authors argue that for corneal staphyloma of unknown cause, enucleation may be preferable, on the reasoning that leaving pigment-producing cells in place could matter. They also note that surgery itself might theoretically drive malignant change through chronic inflammation or altered local conditions, and that a prior report described a blue nevus developing in an artificial eye socket five years after enucleation.
None of that is settled. A single case cannot establish that evisceration raises melanoma risk, and millions of eviscerations have been performed without this outcome. Anyone with an ocular prosthesis who develops new discharge, swelling, or visible pigment should see an ophthalmologist rather than assume the worst.
She was treated with tumor excision, implant removal, and 67.5 Gray of adjuvant radiation in 27 fractions. Three years later, there was no recurrence, though eyelid atrophy and closure of the conjunctival sac later required reconstructive surgery in August 2024. The work was funded by the Sanming Project of Medicine in Shenzhen, and the authors declared no competing interests.
Key Questions Answered
What is primary orbital melanoma?
A melanoma that arises within the tissues behind the eye rather than spreading there from the skin, the uvea, or elsewhere. It makes up roughly 1 percent of orbital tumors, with fewer than 100 cases documented.
How old was the patient?
She was 14 when her right eye was eviscerated in 2012 and roughly 23 when she returned in 2021 with tearing and bloody discharge.
What is a blue nevus, and does it become cancer?
Blue nevi are benign skin lesions formed by pigment cells trapped deep in the dermis. They are usually harmless. Between 39 and 90 percent of reported orbital melanomas have been associated with blue nevi or congenital melanosis, which is why researchers view them as a possible risk marker.
Did the orbital implant cause the tumors?
No evidence links implants to this cancer. The authors raise it as a question worth considering, citing one earlier case of a benign blue nevus appearing in an artificial socket, but they do not claim a causal role.
Why do the authors prefer enucleation over evisceration here?
Evisceration leaves the scleral shell and possibly residual pigment-producing tissue in the orbit. Enucleation removes the whole globe. The suggestion applies specifically to corneal staphyloma of unknown cause.
Should anyone with an artificial eye be worried?
This is an exceptionally rare event, and one case cannot define a risk level. New discharge, swelling, pigment, or implant movement should be evaluated by an ophthalmologist as a matter of routine care.
