The pineal gland tumors account for 0.4% to 1.0% of all adult intracranial lesions. While most of the focus is on pineal parenchymal and germ cell tumors, a spectrum of rare pathologies also exist, including meningiomas, glioblastoma multiforme and various mesenchymal tumors. These are seldom reported, making definitive diagnosis and standard management difficult. They often present with non-specific signs of mass effect such as headache, vomiting and visual disturbance, increasing ambiguity in differentials for a clinician.

This search yielded 190 articles among which 142 studies met the inclusion criteria. The mean age at presentation was 37.73 years (range 1.25 to 78 years) with a notable male predominance (65.2%). Histological diversity was extreme; the most frequent rare entities were Meningioma, Glioblastoma, Primary Pineal Malignant Melanoma and Papillary Tumor of the Pineal Region. Clinically, the most common symptoms related to mass effect included headache (n=74) and signs of raised intracranial pressure (n=43). Surgical resection was the foundation of management. In 12 cases, acute hydrocephalus was addressed by a VP shunt or ventriculostomy prior to definitive tumor surgery. Prognostically, 41 cases demonstrated a positive long-term outcome (defined as no recurrence or progressive clinical improvement).

Rare tumors of the pineal region are biologically and clinically diverse, demanding aggressive and tailored management.