A Rare Case of Primary Pineal Melanoma with Metastasis in an African American Patient
Micah Sy1, Paul Wright1
1Nuvance Health
Objective:
We present a case report of a primary pineal melanoma (PPM) with local metastasis in an African American patient.
Background:
Primary pineal melanomas present a diagnostic and treatment challenge, largely in part due to the paucity of data available and low incidence among the population. PPM is thought to arise from leptomeningeal melanocytes which are derived from the neural crest. These melanocytes are thought to migrate throughout the CNS; however, are most abundant in the anterior and lateral spinal cord and ventrolateral to the medulla. To date, there have only been 27 cases reported in the literature since 1899.
Design/Methods:

A 41-year-old African-American female was diagnosed with a PPM after she presented with lightheadedness, headache, blurred vision, and Parinaud syndrome. MRI brain demonstrated a pineal mass with adjacent hemorrhage and intraventricular extension. She underwent a left paramedian suboccipital craniotomy for gross total resection of the mass, third ventriculocisternostomy, and right frontal ventriculoperitoneal shunt. Pathology confirmed PPM with the tumor positive for HMB-45. She completed six weeks of intensity-modulated radiation therapy and immunotherapy with pembrolizumab then nivolimab/ipilumumab.

Serial MRIs demonstrated progression involving the cerebrum and cerebellum. There was however, clinical improvement with immunotherapy and stereotactic radiosurgery for the intracranial tumor extension. Despite a waxing and waning clinical picture and multiple hospital admissions with two further surgical evacuations, the patient survived >82 weeks from initial symptoms. At the time of death, MRI spine confirmed metastatic disease to the spinal cord.

Results:
NA
Conclusions:
We present a rare case of metastatic primary pineal melanoma in an African American female. While more studies are needed to establish a standard protocol for treatment, the evidence today supports surgical resection with adjuvant medical therapy as the best management plan.
10.1212/WNL.0000000000206519