Abstract
Primary pineal malignant melanoma is a rare entity, with only thirteen cases reported in the world literature to date. We report a case of a 70-year-old man, who consulted with gait disturbance of six months duration, associated in the last month with dizziness, visual abnormalities and diplopia. No other additional melanocytic lesions were found elsewhere. The magnetic resonance showed a 25 mm expansive mass in the pineal gland that was associated with hydrocephaly, ventricular and transependimary oedema. The lesion was partially excised by a supracerebellar infratentorial approach. The histological examination revealed a melanoma. The patient received radiation therapy, but died of disease 16 weeks later. We herein review the literature on this rare tumour and comment on its clinical, radiological and histopathological features and differential diagnosis.
Highlights
Primary pineal malignant melanoma is a rare entity
Most of the melanomas arising in the central nervous system (CNS) are metastatic and primary malignant melanoma represents an exceedingly rare malignant primary tumour of CNS, representing less than 1% of primary tumour in this location
Primary malignant melanoma represents
Summary
Primary pineal malignant melanoma is a rare entity. The initial symptoms are related to tectal compression and imaging findings can be variable, so frequently diagnosis is made only after surgery when tissue material for pathological exam is obtained. Magnetic resonance imaging (MRI) showed a 25 mm mass in the pineal gland, hyperintense in FLAIR sequences and isointense in T2 compared to the encephalic parenchyma (Figure 1). Many cells contain abundant intracytoplasmic granules of melanin pigment (Figure 4), and they display a strong immunoreactivity for melanin markers, namely HMB-45 (Figure 5), MelanA (Figure 6) and S100 protein. The CT scans did not reveal any mass other than the pineal. In the absence of clinical skin, mucous and ocular melanic lesions and radiologic evidence of no extracranial disease, diagnosis of primary malignant melanoma of the pineal gland was made
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