Elsevier

World Neurosurgery

Volume 108, December 2017, Pages 996.e17-996.e25
World Neurosurgery

Case Report
Ganglioglioma Progression to Combined Anaplastic Ganglioglioma and Anaplastic Pleomorphic Xanthoastrocytoma. Case Report and Literature Review

https://doi.org/10.1016/j.wneu.2017.09.072Get rights and content

Background

Composite ganglioma and pleomorphic xanthoastrocytoma with anaplastic features in both components is an extremely rare glioneuronal tumor. Five cases of anaplastic progression in the glioma component have been reported. These tumors generally affect young patients who have brain tumor-related epilepsy, which are usually located in the temporal lobe or in the cerebellum and may have associated leptomeningeal spreading. Its current optimal treatment consists of maximal safe surgical resection and adjuvant chemoradiotherapy. Overall survival at 5 years is 33% in anaplastic pleomorphic xanthoastrocytoma and 53% in anaplastic ganglioglioma.

Case Description

We describe a progression from ganglioglioma to this composite anaplastic entity after 32 months of follow-up, with apparently nontumoral parenchyma separating the 2 components. Polymerase chain reaction showed a wild-type BRAF gene. Seven months after concomitant chemoradiotherapy, radiologic progression led to a second line of chemotherapy, and a third line of chemotherapy was initiated after a subsequent progression at 11 months.

Conclusions

This case may add some evidence in favor of the glioneuronal maldevelopment hypothesis to explain the oncogenesis of these neuroepithelial tumors.

Introduction

The pleomorphic xanthoastrocytoma (PXA)–ganglioglioma (GG) composite is a rare entity described by Kros et al.,1 with 23 cases reported since its first description. Its anaplastic progression, described by Lindboe et al.,2 has been reported in only 5 patients.3 We present a case of GG that progressed to composite PXA and GG, both with anaplastic features, and separated by normal brain tissue.

The term mixed glioneuronal tumor has been proposed as an entity to group ganglioneuroma, gangliocytoma, GG, desmoplastic gangliocytoma, neurocytoma, hamartomatous glioneuronal tumors, and PXA.4 Assuming a hypothetical common origin, it has been proposed that there may be a continuum among these entities.5, 6, 7

We recently treated a patient with a temporoinsular GG that progressed to composite frontal PXA–temporoinsular GG, with anaplastic features in both components. We describe the case and discuss the etiopathogenesis of this rare glioneuronal neoplasm.

Section snippets

Case Description

A 43-year-old woman presented with lateral temporal epilepsy. Magnetic resonance imaging (MRI) showed a temporoinsular tumor with the appearance of low-grade glioma (Figure 1). Seizures were successfully treated with levetiracetam. A subtotal resection was achieved with extent of resection of 88% and a residual volume of 6.1 mL (Figure 2).

The pathologic specimen showed mild hypercellularity because of dystrophic neurons and neoplastic glial cells. We did not observe mitosis, necrosis, or

Discussion

We report a rare neoplasm that we classify as a mixed glioneuronal tumor, with apparently normal parenchyma distinguishing the PXA from the GG as a distinctive feature.

Conclusions

We report a case of anaplastic progression from insular GG to composite PXA-GG that we classified as a mixed glioneuronal tumor. This case may add some evidence in favor of the glioneuronal maldevelopment hypothesis to explain the oncogenesis of these neuroepithelial tumors, and it provides further information on the natural history of this rare entity.

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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.

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