Objective: To report the rare occurrence of orbitopalpebral and intracranial melanoma in patients with periorbital cellular blue nevus.
Design: A case series.
Intervention: The clinicopathologic records of two patients with periorbital cellular blue nevus were reviewed.
Results: In case 1, a 32-year-old woman with congenital blue discoloration of her eyelids had gradual increase in nodularity of the eyelid tissue develop over the prior 20 years. The lesion enlarged to the point that the eyelids were almost completely closed from the massive tumor. Results from a biopsy disclosed orbitopalpebral melanoma arising from cellular blue nevus. Because of the diffuse infiltrative pattern of the lesion, she was treated by surgical debulking of the mass. She had a separate focus of ipsilateral intracranial melanoma develop at 40 years of age and eventually died of meningeal melanoma. In case 2, a 29-year-old man with blue discoloration of his left lower eyelid since 10 years of age had an anterior orbital mass develop. He underwent excisional biopsy, the results of which showed an orbitopalpebral melanoma arising from the periorbital cellular blue nevus. Orbital melanoma recurrence and ipsilateral lymph node metastasis were found 9 years later and treated with resection and chemotherapy. He was free of recurrence or metastasis 2 years later.
Conclusions: Patients with periorbital cellular blue nevus may present in the preteen years with a bluish eyelid discoloration. They are at risk of having orbitopalpebral and intracranial malignant melanoma develop.