Long term follow-up in a patient with papillary glioneuronal tumor

Abstract

We report a case of a young female patient with a rare and recently described form of brain tumor. This patient had a history of headache, hemiparesis and motor simple partial seizures. Her investigation revealed a brain tumor involving the left frontal and parietal lobes. The radiological images showed a cystic mass with multiple nodular masses and a rim of contrast enhancement extending from the right parietal cortex to the ipsilateral ventricle and corpus callosum. The patient underwent gross resection of the lesion and the histological analysis of the surgical specimen revealed a pseudopapillary structure formed by delicate vessels intermixed with a fibrillary pattern and bordered by intense astrocytic reaction with Rosenthal fibers. These features correspond to the recently described mixed neuronal-glial neoplasm, the papillary glioneuronal tumor. The patient has been followed for five years since the surgical treatment, without evidence of tumor recurrence, confirming the indolent behavior of this type of tumor.

Papillary glioneuronal tumor radiologically mimicking a cavernous hemangioma with hemorrhagic onset

“…Follow‐up data indicate a benign outcome with no evidence of recurrence during intervals ranging from 3 months to 7 years (Table 1). 2–12,15 Histopathological features of papillary glioneuronal tumor are similar in all reported cases, invariably reproducing the first description of this tumor by Komori 2…”

“…Typical morphological features are pseudopapillary structures composed of frequently thickened hyalinized blood vessels lined by uniform small astrocytes and a proliferation of neurocytic cells eventually admixed with ganglioid and ganglion cells. Rabdoid, minigemistocytic, and Olig2 (a recently established oligodendroglial marker) positive cells have been recognized too 1–16 …”

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“…A detailed review of literature suggests that besides these features, degenerative changes are also common in this neoplasm (Table 1). They may be in the form of Rosenthal fibers [2,5,7], calcification, foam cell collection, hemosiderin laden macrophages [2,5,6,8,9] or mild lymphocytic infiltrate [2]. All these degenerative changes were absent in the present case.…”

“…Mitosis, necrosis and endothelial proliferation were not seen in any of the cases. MIB-1 labeling index has also been found to be low (0.5%–2.5%, mean 1.3%) [2,4,7]. The presence of pseudopapillae, degenerative changes and low MIB-1 labeling index all indicate that PGNTs are generally slow growing and low grade neoplasms and thus, appear to carry a good prognosis.…”

Papillary Glioneuronal Tumor: Radiological Evidence of a Newly Established Tumor Entity

“…PGNT was first described by Komori et al in 1998 in a series of 9 cases 3 . After that, about 33 cases were reported in succession in the English language literature 5–30 . In 2007, WHO classified PGNT as a Grade I neuronal‐glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell populations.…”