Complete regression of papillary tumor of the pineal region after radiation therapy: case report and review of the literature

Abstract

Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial tumor that arises in the pineal region. The optimal treatment for PTPR remains controversial, as no definitive treatment strategy exists for this lesion. It is not clear whether aggressive surgical removal is superior to biopsy followed by radiotherapy. The majority of cases in the literature have undergone attempted gross total resection with a supracerebellar-infratentorial or a transcallosal-transventricular approach. In this report, we describe a case of PTPR in a 23 year-old male that presented as a third ventricular mass causing obstructive hydrocephalus. An endoscopic third ventriculostomy was performed followed by an endoscopic biopsy. Postoperative radiotherapy resulted in complete regression of the tumor with no evidence of tumor recurrence at 25 months. This case highlights a minimally invasive strategy for a rare neoplasm that resulted in a favorable response to radiation therapy, thereby avoiding the risks of aggressive surgical removal. We also review the radiographic and histopathologic features of PTPR and discuss various options of treatment reported in the literature.

Outcomes After Surgery and Radiotherapy for Papillary Tumor of the Pineal Region

“…Similarly, a pineal region tumor initially treated empirically with upfront Gamma Knife SRS was controlled for 7 years before the patient developed symptomatic recurrence and underwent GTR, at which time pathologic diagnosis of PTPR was made (1). Other authors have reported complete resolution and no tumor recurrence at 2 years of follow-up with fractionated radiation alone, in the absence of definitive surgery, for biopsy-confirmed PTPR (14).…”

Papillary tumor of the pineal region. Report of two cases and literature review

“…On the other hand, tumor regression after radiotherapy [10,32] or even tumor disappearance [33] was observed. Whole brain irradiation, local radiotherapy, gamma knife surgery, stereotactic linac radiosurgery were all used with different protocols [32,34,35].…”

Stereotactic Radiosurgery of a Papillary Tumor of the Pineal Region: Case Report and Review of the Literature

“…However, due to recurrence after seven years, gross total resection was performed in this particular case [16]. In another reported case, radiation therapy after endoscopic biopsy and third ventriculostomy has resulted in complete tumor regression at 25 months [17]. …”