Surgical Resection and Cellular Proliferation Index Predict Prognosis for Patients with Papillary Glioneuronal Tumor: Systematic Review and Pooled Analysis.

BACKGROUND

Although the World Health Organization classifies papillary glioneuronal tumor (PGNT) as a grade I tumor, several malignant cases have been reported. In this study, we examined the clinical and prognostic characteristics of PGNT.

METHODS

PubMed, Embase, and institutional databases were queried for patient-level reports of PGNT, resulting in identification of 138 cases. Descriptive and Kaplan-Meier survival analyses were conducted. The threshold of significance was 0.05.

RESULTS

The mean age at presentation was 26.9 ± 16.3 years, and the incidence was higher in males (1.42:1). Tumors with a high Ki-67 index (≥5) were more likely to exhibit perilesional edema and ring enhancement on magnetic resonance imaging, trending toward significance (P = 0.114 and 0.113, respectively). Compared with tumors with a low Ki-67 index (<5), those with a high Ki-67 index were more likely to be treated with subtotal resection (STR) than with gross total resection (GTR) (Kruskal-Wallis test, P = 0.006) and with radiation therapy (χ test, P = 0.010). At 5 years post-treatment, PGNT had a mean progression-free survival (PFS) of 85.9 ± 3.9%. Males had a better 5-year PFS than females (94.0 ± 3.4% vs. 74.8 ± 7.8%; Mantel-Cox test, P = 0.002). Two-year PFS was higher after GTR than after STR (91.9 ± 3.6% vs. 46.7 ± 21.4%; Mantel-Cox test, P < 0.001). A low Ki-67 index was associated with a higher 5-year PFS compared with a high Ki-67 index (94.8 ± 3.6% vs. 55.6 ± 12.9%; Mantel-Cox test, P < 0.001).

CONCLUSIONS

PGNT is a benign tumor of young adults, but can present atypically as high grade. Male sex, low cellular proliferation, and maximal surgical resection are positive prognostic indicators for PGNT.