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Prognosticating survival of pineal parenchymal tumors of intermediate differentiation (PPTID) by grade

  • Clinical Study
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Abstract

Background

Pineal parenchymal tumors of intermediate differentiation (PPTID) are a rare group of pineal parenchymal tumors classified by histology as either World Health Organization (WHO) Grades 2 or 3. The rarity of these tumors in adults has left a number of clinical management questions open. Correspondingly, the aim of this study was to aggregate a large PPTID cohort with sufficient statistical power from a large national cancer database to analyze prognostic parameters.

Methods

All PPTID patients aged over 18 years in the U.S. National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized and survival was modeled using Kaplan–Meier and Cox regression analyses.

Results

A total of 103 adult PPTID patients were identified in the NCDB with 63 (61%) WHO Grade 2 and 40 (39%) WHO Grade 3 tumors. Overall, mean age was 53 ± 18 years with even gender distribution. A total of 75 (73%) patients underwent surgical resection for diagnosis, with gross total resection (GTR) was the most common resection outcome in 50/75 (67%). Chemotherapy was utilized in 18 (17%) patients, and radiation therapy in 37 (36%) patients. Overall, 5-year survival rate was estimated to be 54% (95% CI 42–64%), with mean survival was 84 (95% CI 69–99) months. Patients with Grade 2 tumors survived statistically longer than Grade 3 tumor counterparts (P < 0.01). Overall, older age (HR 1.09, P < 0.01) was associated with shorter survival, whereas GTR (HR 0.43, P = 0.02) was associated with longer survival. Both these parameters were significant within Grade 2 and Grade 3 subgroup analyses as well.

Conclusions

PPTID are rare tumors with expected mean survival more than 5 years, although Grade 2 tumors are expected to survive longer than Grade 3 tumors. Age and gross total resection are significant independent predictors of survival in PPTID overall, as well as within Grade 2 and Grade 3 subgroups separately. The prognostic role and benefit of adjuvant therapy is yet to be elucidated, mandating more molecular and biologic research be done to further optimize clinical management in the future.

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This manuscript has data included as electronic supplementary materials.

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Contributions

Conception and design: VML, EML, MEI. Acquisition of data: VML. Analysis and interpretation of data: all authors. Drafting the article: all authors. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors Approved the final version of the manuscript on behalf of all authors: VML, MEI. Statistical analysis: VML. Study supervision: MEI.

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Correspondence to Victor M. Lu.

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Lu, V.M., Luther, E.M., Eichberg, D.G. et al. Prognosticating survival of pineal parenchymal tumors of intermediate differentiation (PPTID) by grade. J Neurooncol 155, 165–172 (2021). https://doi.org/10.1007/s11060-021-03863-y

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