Spontaneous globe rupture from an intraocular melanotic schwannoma

Melanotic schwannoma (MS) is a rare neoplasm composed of Schwann cells with melanosomes in various maturation stages. While MS is typically observed in spinal nerve roots or peripheral nerves, their involvement in intraocular structures is uncommon. Here, we present a case of spontaneous globe rupture as the presenting feature of intraocular extension of a MS.

Enucleation was performed to remove the lesion. Confirmatory light microscopy and immunohistochemistry were used to properly diagnose and differentiate from conventional schwannoma and uveal malignant melanoma.

The ocular tumor in our patient showed melanin pigmentation with positive melanocytic markers. The tumor revealed spindle and epithelioid cells proliferating in fascicles, which has previously been described for MS. No choroidal invasion, moderate nuclear pleomorphism, and a low mitotic rate were observed supporting a diagnosis of MS in our patient.

With reports of MS with high recurrence and metastatic spread rates, there have been some suggestions that MS should be considered an aggressive malignancy. This case highlights a dramatic presentation of a rare ocular malignancy with some features of aggressiveness. Our case report endorses the importance of close monitoring for local recurrence or metastasis to prevent a poor outcome.