Pineal tumors of the pineal region (PTPR) are rare neuroepithelial tumors of the central nervous system, and their clinical features often lack specificity, so it is always difficult to make a definite diagnosis before surgery, and the treatment is mainly based on complete surgical resection. We report the case of a 21-year-old male who presented with intermittent dizziness and headache with vomiting for more than 2 months. The imaging revealed a mass in the region of the pineal region with obstructive hydrocephalus. After the hospitalization, the patient underwent a microscopic excision of the pineal mass, and the tissue was sent for histopathological examination and available immunohistochemical tests, which resulted in a definitive diagnosis of a papillary tumor of the pineal region. He had undergone several radiation treatments during his hospitalization and remained recurrence-free until the latest follow-up visit. This article highlights the similarities between this case and the PTPR described in WHO 2021 in terms of clinical presentation, imaging, and histopathological features. Therefore, this case report completes the sample database of PTPRs. By reviewing the literature related to PTPRs, we hope that this report will be helpful to improve the understanding of clinicians and pathologists about this rare tumor. However, more relevant studies are needed to clarify the pathogenesis, prognosis, and optimal treatment options to achieve early diagnosis, early treatment, and improved prognosis.
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