Original ArticleSurgical Resection and Cellular Proliferation Index Predict Prognosis for Patients with Papillary Glioneuronal Tumor: Systematic Review and Pooled Analysis
Introduction
Papillary glioneuronal tumor (PGNT) is a rare brain tumor recently classified as a unique clinicopathologic entity in the 2007 World Health Organization (WHO) classification of central nervous system tumors.1 First characterized by Komori et al.,2, 3 PGNT is most commonly reported in young adults, occurring in the white matter of the cerebral hemispheres with proximity to the ventricular system. Patients often present with headaches, seizures, and mass effect symptoms.4
These tumors can demonstrate solid, cystic, and occasionally nodular components on magnetic resonance imaging (MRI).5 Histologically, they exhibit a mixed glial and neuronal differentiation. The glial component is characterized by glial fibrillary acidic protein (GFAP)-positive cuboidal cells arranged in a papillary architecture, overlaying hyalinized vessels. The neuronal component occurs in interpapillary regions, consisting of homogenous neurocyte-like cells, oligodendrocyte-like cells, and ganglioid cells.1 Using fluorescence in situ hybridization, several studies have identified SLC44A1-PRKCA, a novel and specific fusion product in PGNT consisting of a choline transporter-like protein and a serine/threonine-specific protein kinase.6, 7, 8 Routine diagnosis remains challenging and relies on neuroimaging and histology.
Although the 2007 and 2016 WHO classification of central nervous system tumors designated PGNT as a grade I tumor,1, 9 increasing numbers of cases of malignant or aggressive PGNT have been reported.10, 11, 12, 13, 14, 15, 16 Bourekas et al.17 reported a case of anaplastic PGNT in a 19-year-old patient who, after undergoing surgical resection, radiation therapy, and chemotherapy, presented with pleural metastases and subcarinal and retroperitoneal lymphadenopathy.
Several case series have attempted to characterize PGNT; however, because of its rarity, it has not yet been comprehensively described. The purpose of this study was to identify the common clinical characteristics of this rare tumor and to delineate predictors of long-term prognosis, information that can aid the diagnosis and management of PGNT.
Section snippets
Methods
PubMed and Embase were queried for “papillary glioneuronal tumor/tumour” or “PGNT” in a systematic review of the literature. PRISMA guidelines were followed, entailing the use of distinct inclusion and exclusion criteria, careful extraction of data, and a summarization of results.18 Inclusion criteria included patient-level data and English-language text. Purely histological or genetic analyses without patient data were excluded. The citations in all publications were cross-referenced for
Results
A total of 132 cases were identified in the literature, and 6 additional cases were identified in institutional databases, for a total of 138 cases.3, 5, 7, 8, 10, 11, 12, 13, 14, 16, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68 The mean patient age at presentation for PGNT was 26.9 ± 16.3 years. More than 80% of cases were reported in
Discussion
It has been 20 years since PGNT was first reported in the literature.3, 19 Studies of the natural history of PGNT are limited by the tumor's rarity and geographic scarcity. Long-term data on such rare tumors are severely lacking. The present study represents the most comprehensive clinical examination of PGNT published to date.
PGNT is considered a tumor of young adults, as demonstrated here by the mean age at presentation of approximately 27 years. However, several cases were reported in the
Conclusion
PGNT is a benign tumor of young adults, but can present atypically as high grade and across the human lifespan. It has a higher incidence in males, and has a predilection for the ventricular system. Male sex, low Ki-67 cellular proliferation index, and maximal surgical resection are positive prognostic indicators for PGNT. This study represents the most comprehensive clinical information on this rare tumor published to date.
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2019, World NeurosurgeryCitation Excerpt :Several dozen cases have been described in the literature since they were first described by Komori et al8 in 1998. Patients are typically young adults and present with headaches or seizures.2,9,10 The course of these tumors is typically benign; Ahmed et al2 demonstrated a 5-year progression-free survival rate of 85.9%.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.