Primary pineal malignant melanoma - illustrated review

Area of Science:

• Neuro-oncology
• Pathology
• Radiology

Background:

• Primary pineal melanoma is an exceptionally rare central nervous system (CNS) tumor.
• This review synthesizes current knowledge on its histogenesis, pathology, and diagnostic imaging.
• It also explores available therapeutic strategies for this aggressive malignancy.

Observation:

• A systematic literature search identified 16 cases of primary pineal melanoma.
• Patients presented at a median age of 50 with symptoms like headache, personality changes, and gait disturbance.
• Commonly observed spread patterns included leptomeningeal dissemination (70.6%) and ventricular ependymal spread (35.3%).

Findings:

• Surgery, radiotherapy, and chemotherapy were employed in 29.4%, 47.1%, and 23.5% of cases, respectively.
• The median overall survival was 56 weeks.
• Combined modality treatment, including surgery and radiation, demonstrated efficacy.

Implications:

• For localized primary pineal melanoma without leptomeningeal spread, combined maximal safe surgery and post-operative radiation is recommended.
• Chemotherapy may be reserved for recurrent disease, drawing parallels with other melanoma subtypes.
• Further research is needed to optimize treatment protocols for this rare tumor.