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.