Three Weeks in Florida

The patient underwent leptomeningeal biopsy. During surgery, the meninges were noted to have diffuse pigmentation. Histopathologic evaluation demonstrated proliferation of heavily pigmented cells with prominent nucleoli. Immunostains were diffusely positive for S-100 as well as melanocytic markers HMB-45, MART-1 and tyrosinase while negative for keratinocyte markers AE1/AE3, and PCK26. The Ki-67 staining showed foci of elevated labeling > 10%. Genetic testing was negative for BRAF p.V600E and BRAF p.V600K mutations. Full body dermatologic examination was negative for pigmented skin lesions. CT of the chest, abdomen, and pelvis were negative for tumors. PET scan was unattainable while inpatient. Ophthalmic examination demonstrated the patient was awake but confused and only partially aware of his surroundings. Visual acuity was 20/400 in each eye, visual fields were constricted to confrontation by finger counting. His extraocular movements appeared to be full with some exotropia. His pupils were noted to be sluggish and mid-dilated but no afferent defects were present. His anterior segment was unremarkable without scleral pigmentation; fundus examination was significant for grade 3 papilledema without any tumors present. He then underwent ventriculo-peritoneal shunt placement for persistent hydrocephalus. He was started on ipilimumab, a monoclonal antibody to increase immune response to melanoma cells. The patient was discharged to rehab where he self-extubated his tracheostomy and could not be re-intubated subsequently expiring. Autopsy was not feasible as death notification occurred over 36 hours.