Papillary Glioneuronal Tumour in a 4-Year-Old

Abstract

The recently described ‘papillary glioneuronal tumour’ is not currently included in the WHO classification of tumours of the CNS. We present the youngest recorded case to date, and only the 3rd documented in the paediatric population. The incorporation of this neoplasm into the WHO classification would facilitate its wider recognition, providing an opportunity to elucidate its natural history and determine an evidence-based approach to treatment.

Papillary glioneuronal tumor: a case report and review of the literature

“…The vessels are enclosed by a uniform, single, or pseudostratified layer of small cuboidal cells with round reticular nuclei without atypia, and scant cytoplasm. These cells are uniformly immunoreactive for GFAP and S-100 antibodies [3], and one case showed immunostaining with synaptophysin antibody [8]. Antisynaptophysin staining was also seen in this case.…”

“…To date, all the cases in the literature have shown that removal of the tumor results in the resolution of these neurologic deficits. Clinically, the mean age at presentation is 27 years, the oldest being 75 years [9] and the youngest 4 years [8]. Prevalence is equal in both males and females.…”

“…There is a preference for temporal lobe involvement (38%). Three cases were found in the parietal lobe, 3 in the parietooccipital lobe, 5 in the frontal lobe, 1 in the occipital lobe, and 1 in the third ventricle [3,6,7,8,[10][11][12]. Both the cortex and white matter may be involved, and the male/female ratio is 1:1.…”

“…Almost all cases were treated with total tumor resection, without recourse to chemotherapy or radiation therapy. The followup of these patients after surgery spans between 3 and 84 months, and in all of the cases, there has not been a recurrence of the tumor [3,[6][7][8][9][10][11][12]. Time will tell as to the long-term prognosis of this recently discovered entity.…”

Papillary Glioneuronal Tumor

“…ecent advances in brain tumor characterization by histopathology have resulted in the discovery of tumors with neuronal elements including subtypes of gangliogliomas such as the rosette glioneuronal tumor and papillary glioneuronal tumor [1][2][3][4][5][6][7][8][9][10][11]. Papillary glioneuronal tumors have been reported in 17 patients in pathology journals [1][2][3][4][5][6][7][8][9].…”