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Resident PGY 3
Henry Ford Hospital
A 49-year-old female was referred to Neuro-Ophthalmology for evaluation of bilateral disc edema associated with headache. On initial presentation she was found to have a constricted visual field in her left eye and evidence an atrophic optic disc in the right eye and optic disc edema in the left eye. Evaluation with MRI showed no evidence of a mass lesion and lumbar puncture showed an elevated opening pressure leading to a presumed diagnosis of pseudotumor cerebral eye. The patient was started on treatment with an oral carbonic anhydrase inhibitor and encouraged to attempt weight loss. She was subsequently followed for 4 months with varying doses of oral carbonic anhydrase inhibitor and 62 having 15 lb of weight loss since her initial presentation, however she had no subsequent improvement of her optic disc edema. Two months later she developed new symptoms of nausea, vomiting, and difficulty with her gait which prompted a repeat MRI showing a leptomeningeal pattern of enhancement and nodularity throughout the basal cistern, cerebellum and bilateral temporal lobes with areas of abnormal enhancement in the basilar cisterns do demonstrate some increased signal on T1-weighted images without contrast. Repeat lumbar puncture again showed elevated opening pressure. These findings were concerning for a CNS melanoma and a MRI cervical/thorax/lumbar was performed showing an intrinsically T1 hyperintense, intramedullary mass centered at T2-T3 with larger intradural, extra-medullary component extending into the ventral CSF space of the upper thoracic spine suggestive of primary melanotic spinal cord melanoma. A laminectomy from C7 to T3 was performed and pathology revealed metastatic melanoma. A full dermatologic exam was performed revealing vision all sites consistent with melanoma, suggesting the diagnosis of a primary CNS melanoma. The patient was started on systemic chemotherapy per medical oncology and received 1 of fusion, unfortunately she had rapid decline in subsequently died 5 weeks after diagnosis. Primary CNS melanoma is uncommon and constitute approximately 1% of all melanoma cases and 0.07% of all brain tumors, median survival is generally only 10 weeks after there is leptomeningeal spread.
Leikert, Kevin; Mikkilineni, Shravani; and Skarf, Barry, "Primary CNS Melanoma" (2020). Case Reports. 59.