Case ReportGanglioglioma Progression to Combined Anaplastic Ganglioglioma and Anaplastic Pleomorphic Xanthoastrocytoma. Case Report and Literature Review
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.
References (47)
- et al.
The pleomorphic xanthoastrocytoma and its differential diagnosis: a study of five cases
Hum Pathol
(1991) - et al.
Pleomorphic xanthoastrocytoma with intraventricular extension and anaplastic transformation in an adult patient: case report
Neurocirugia
(2012) - et al.
Pleomorphic xanthoastrocytoma with gangliogliomatous component
Pathol Res Pract
(1997) - et al.
Malignant potential of pleomorphic xanthoastrocytoma
J Clin Neurosci
(2012) - et al.
Extent of resection influences outcomes for patients with gliomas
Rev Neurol (Paris)
(2011) - et al.
Pleomorphic xanthoastrocytoma as a component of a cerebellar ganglioglioma: case report
Neurosurgery
(1992) - et al.
Composite pleomorphic xanthoastrocytoma and ganglioglioma: report of four cases and review of the literature
Am J Surg Pathol
(1997) - et al.
Neuronal and mixed neuronal glial tumors associated to epilepsy. A heterogeneous and related group of tumours
Histol Histopathol
(2001) - et al.
The CD34 epitope is expressed in neoplastic and malformative lesions associated with chronic, focal epilepsies
Acta Neuropathol
(1999) - et al.
Evidence for developmental precursor lesions in epilepsy-associated glioneuronal tumors
Microsc Res Tech
(1999)
Gangliogliomas: an intriguing tumor entity associated with focal epilepsies
J Neuropathol Exp Neurol
Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma
N Engl J Med
Pleomorphic xanthoastrocytoma: a distinctive meningocerebral glioma of young subjects with relatively favorable prognosis. A study of 12 cases
Cancer
Non-anaplastic pleomorphic xanthoastrocytoma with neuroradiological evidences of leptomeningeal dissemination
Child’s Nerv Syst
Pleomorphic xanthoastrocytoma
Pleomorphic xanthoastrocytoma: a comparative pathological study between conventional and anaplastic types
Histopathology
Histopathological features of recurrent pleomorphic xanthoastrocytomas: further corroboration of the glial nature of this neoplasm
Acta Neuropathol
Pleomorphic xanthoastrocytoma: what do we really know about it?
Cancer
Cerebellar pleomorphic xanthoastrocytoma: case report
Neurosurgery
A case of pleomorphic xanthoastrocytoma with anaplastic features in the pineal gland
Brain Tumor Pathol
Molecular neuropathology of epilepsy-associated glioneuronal malformations
J Neuropathol Exp Neurol
Gangliogliomas: experience with 34 patients and review of the literature
Am J Clin Oncol
Outcome and prognostic features in anaplastic ganglioglioma: analysis of cases from the SEER database
J Neurooncol
Cited by (0)
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.