Case reportHemorrhagic papillary glioneuronal tumor mimicking cavernoma: Two case reports
Introduction
Mixed glioneuronal tumors of the central nervous system are rare but well recognized neoplasms. They are characterized by a wide morphological, immunohistochemical and ultrastructural spectrum of glial and neuronal differentiation. They include the papillary glioneuronal tumor that was recently identified as low-grade tumor (WHO grade I); it is composed of pseudopapillary structures consisting of a uniform single layer of small astrocytes surrounding thickened hyalinized blood vessels, and a proliferation of neurocytic cells, which are possibly admixed with ganglioid cells.
Theses tumors usually affect young women and occur preferentially in the supratentorial region of brain. Their presentation by hemorrhagic onset is possible. To date, only few cases revealed by cerebral hemorrhage were previously described in the literature [1], [2], [3]. Herein, we report 2 cases of hemorrhagic cerebral papillary glioneuronal tumors with radiographic appearances mimicking cavernous haemangioma. We also review the clinical, radiological, histological, and therapeutic features of this rare entity.
Section snippets
Case 1
The patient was a 74-year-old woman with unremarkable past medical history who presented with two months history of episodic positional vertigo associated with headache and nausea. The neurologic, vestibular and otoscopic examinations were normal. In addition, she was found to have papilledema and a bilateral decreased visual acuity. The visual fields were normal.
Cerebral MRI demonstrated right occipital periventricular heterogeneous lesion composed of a polycystic mass and a nodular portion
Discussion
In 1997, Kim and Suh firstly described this tumor as pseudopapillary neurocytoma [4]. One year later, Komori et al. reported a series of 9 cases and were the first to employ the term of “papillary glioneuronal tumor” [5]. Since then, 38 cases were reported in the literature [1], [2], [3], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24].
In the newly updated World Health Organization (WHO) classification of central nervous system
Conclusion
PGNT is a rare and distinct glial tumor characterized by a generally younger age of presentation and a female predominance. It is a slow growing tumor having typical morphological and histological features, low rate of cell proliferation and commonly good prognosis.
Radiologically, it can be solid contrast-enhancing tumor or cystic lesion with mural nodule. Its revelation by cerebral hemorrhage can mimic cavernous haemangioma. Therefore, it is necessary to keep in mind the possibility of PGNT in
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