Primary tumors of the pineal gland occur infrequently with a preponderance of either
parenchymal tumors or germ cells tumors. Papillary tumor of the pineal region is a
rare neuroepithelial lesion that arises exclusively in the pineal region. They have
been designated as either Grade II or Grade III lesions as per the 2016 WHO classification
of central nervous system tumors. Clinically, they usually present with obstructive
hydrocephalus and visual disturbance. On imaging, these tumors are solid-cystic, heterogeneously
enhancing, and show T2 hyperintensity. Pathologically, they can closely resemble a
Grade I pineocytoma and immunohistochemistry is essential to differentiate the two.
No definite guidelines exist to confirm the ideal protocol of treatment. Evidence
regarding the role of radiation after surgery is limited to case reports and series.
Adjuvant therapy is usually recommended for tumors with subtotal excision, high proliferative/mitotic
index, or proven metastasis. We describe a case of a 29-year-old male with a recurrent
papillary tumor of the pineal region, 9 years after primary surgery where it was misdiagnosed
as a pineocytoma. The tumor was effectively controlled with surgical excision, cerebrospinal
fluid diversion, and adjuvant radiation for 8 years before showing two recurrences
within a span of 6 months with a rising proliferation index.
Key-words:
Cytokeratin - papillary - pineal - pineocytoma
