Brain Tumor Res Treat.  2023 Oct;11(4):254-265. 10.14791/btrt.2023.0033.

Survival and Malignant Transformation of Pineal Parenchymal Tumors: A 30-Year Retrospective Analysis in a Single-Institution

• Park TH
• Kim SK
• Phi JH
• Park CK
• Kim YH
• Paek SH
• Lee CH
• Park SH
• Koh EJ

• KMID: 2547414
• DOI: http://doi.org/10.14791/btrt.2023.0033

Abstract

Background
This study aims to elucidate clinical features, therapeutic strategies, and prognosis of pineal parenchymal tumors (PPT) by analyzing a 30-year dataset of a single institution.
Methods
We reviewed data from 43 patients diagnosed with PPT at Seoul National University Hospital between 1990 and 2020. We performed survival analyses and assessed prognostic factors.
Results
The cohort included 10 patients with pineocytoma (PC), 13 with pineal parenchymal tumor of intermediate differentiation (PPTID), and 20 with pineoblastoma (PB). Most patients presented with hydrocephalus at diagnosis. Most patients underwent an endoscopic third ventriculostomy and biopsy, with some undergoing additional resection after diagnosis confirmation. Radiotherapy was administered with a high prevalence of gamma knife radiosurgery for PC and PPTID, and craniospinal irradiation for PB. Chemotherapy was essential in the treatment of grade 3 PPTID and PB. The 5-year progression-free survival rates for PC, grade 2 PPTID, grade 3 PPTID, and PB were 100%, 83.3%, 0%, and 40%, respectively, and the 5-year overall survival rates were 100%, 100%, 40%, and 55%, respectively. High-grade tumor histology was associated with lower survival rates. Significant prognostic factors varied among tumor types, with World Health Organization (WHO) grade and leptomeningeal seeding (LMS) for PPTID, and the extent of resection and LMS for PB. Three patients experienced malignant transformations.
Conclusion
This study underscores the prognostic significance of WHO grades in PPT. It is nec-essary to provide specific treatment according to tumor grade. Grade 3 PPTID showed a poor prognosis. Potential LMS and malignant transformations necessitate aggressive multimodal treatment and close-interval screening.

Keyword

Pineal parenchymal tumor; Pineocytoma; Pineoblastoma; Survival; Malignant transformation

Figure

• 

  Fig. 1 Kaplan-Meier curves of PFS (A) and OS (B) in PPTs according to histological group; and PFS (C) and OS (D) on stratification of PPTID by WHO grade. The p value was calculated by log-rank test. PC, pineocytoma; PPTID, pineal parenchymal tumor of intermediate differentiation; PB, pineoblastoma; PFS, progression-free survivial; OS, overall survival; WHO, World Health Organization.

• 

  Fig. 2 Kaplan-Meier curves of PFS in PC according to tumor diameter (A) and the extent of resection (B). PC, pineocytoma; PFS, progression-free survivial; NTR, near total resection; GTR, gross total resection; PR, partial resection; STR, subtotal resection.

• 

  Fig. 3 Kaplan-Meier curves of PFS according to WHO grade (A), OS according to WHO grade (B), and OS according to LMS (C) in PPTID. PFS, progression-free survivial; OS, overall survival; WHO, World Health Organization; LMS, leptomeningeal seeding; PPTID, pineal parenchymal tumor of intermediate differentiation.

• 

  Fig. 4 Kaplan-Meier curves of OS in PB according to the extent of resection (A), LMS at dianosis (B), LMS during whole period of disease (C), and age in the pediatric subgroup (D). OS, overall survival; PB, pineoblastoma; STR, subtotal resection; NTR, near total resection; GTR, gross total resection; PR, partial resection; LMS, leptomeningeal seeding.

• 

  Fig. 5 The diagnostic and treatment timelines of patients who experienced malignant transformation. PC, pineocytoma; PPTID, pineal parenchymal tumor of intermediate differentiation; PB, pineoblastoma; PR, partial resection; IFRT, involved field radiotherapy; CR, complete remission; LMS, leptomeningeal seeding; RT, radiotherapy; PCV, chemotherapy with procarbazine, lomustine (CCNU), and vincristine; GKRS, gamma knife radiosurgery; GTR, gross total resection; CCRT, concurrent chemoradiotherapy; CTx, chemotherapy; HDCT, high dose chemotherapy; aPBSCT, autologous peripheral blood stem cell transplantation.

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