Background: Primary meningeal melanocytoma is a rare neurological disorder. Although it may occur at the base of the brain, it is extremely rare at the anterior cranial fossa.
Case presentation: A 27-year-old man presented with headache and diplopia at our department. Fundoscopy showed left optic nerve atrophy and right papilledema consistent with Foster-Kennedy syndrome. Neurological exams were otherwise normal. A left frontal irregular space-occupying lesion was seen on magnetic resonance imaging (MRI), and enhancement was shown on contrast-enhanced computed tomography (CT) scan. CT angiography (CTA) revealed vascular compression around the lesion. Prior to surgery, meningioma was diagnosed and gross tumor removal was performed. On postoperative pathohistological exam, the tumor proved to be a meningeal melanocytoma, WHO grade I. No skin melanoma was found. After surgery, the patient received radiation therapy. No tumor was seen on follow-up MR images six months after surgery. The patient was well after two and a half years, and there was no tumor recurrence on the follow-up CT.
Conclusions: This case of primary meningeal melanocytoma located at the anterior cranial fossa is very rare. Although primary meningeal melanocytoma is benign, it may behave aggressively. Complete surgical resection is curative for most cases. Radiation therapy is important to prevent relapse of the tumor, especially in cases of incomplete surgical resection.