Papillary glioneuronal tumor: case report and review of literature

Abstract

Papillary glioneuronal tumor (PGNT) is a recently described central nervous system neoplasm that mostly occurs in the supratentorial system, adjacent to the lateral ventricles. Well-established examples of glioneuronal tumors include DNET (Dysembryoplastic NeuroEpithelial Tumor), ganglioglioma and desmoplastic infantile ganglioglioma. More recently recognized entities include rosette-forming tumor of fourth ventricle, rosette glioneuronal tumor (RGNT)/ glioneuronal tumor with neuropil-like islands and PGNT (1).In 2007, WHO classified PGNT as grade I neuronal-glial tumor because of the characteristic papillary architecture and bipartite (astrocytic and neuronal/neurocytic) cell population. As a newly established entity of mixed glioneuronal tumor family, PGNT attracted extensive attention recently.It was described in approximately 67 cases to date (Table 1).Both CT and MRI studies show PGNTs to be circumscribed supratentorial lesions, extending to the cortex and the white matter, or may be more deeply situated, displacing the ventricular system. Cystic alteration are frequent. Solid components are iso/hypointense in T1 and iso/hyperintense in T2, contrast enhancing in a diffuse, patchy or rimming fashion and may present as mural nodule or plaques within otherwise cystic masses. Calcifications may be seen. Bleeding of lesions are rare but recorded (3), associated with superficial siderosis of neuraxis. Surrounding edema and mass effect are seen in larger lesions. Reported tumor size ranges 1-9 cm (mean 4,5 cm).Hystology showed pseudopapillary pattern consisting of a single layer of astrocytic cells surrounding hyalinized blood cells.Follow-up indicated that this type of tumor had a benign behavior even if recently we found out 5 cases of aggressive PGNT (1,2,4,5,7,8). Case reportThe patient was a 34 yr old woman with complaints of progressive headache started 2 weeks before and more recently confusion, vomiting and dizziness. She had a va-

Papillary glioneuronal tumors: histological and molecular characteristics and diagnostic value of SLC44A1-PRKCA fusion

“…On MRI, PGNT is frequently described as a well-demarcated mass with a cystic component, contrast enhancement, isointensity on T1-weighted and a predominant location in the periventricular area [22]. However, there is no pathognomonic radiographic appearance and in the review by Carangelo and colleagues, around 80 % of PGNT showed a ventricular association [3]. All our cases show a ventricular association, one is clearly intraventricular, similar to the case reported by Matyja and colleagues [23] and the two others, the tumor have a direct contact with the temporal ventricle.…”

“…This tumor is rare, with approximately 70 cases reported in the last decade [3, 4]. Classifying PGNT is a challenge with a diagnosis generally based on neuroimaging and histology.…”

Enlargement of papillary glioneuronal tumor in an adult after a follow-up period of 10 years: a case report

“…The present case, however, may question this clinical attitude as the tumor did enlarge during its 10 years of clinical course and the Ki-67 (MIB-1)-labeling index was 3%, which could be considered as borderline. Close to a hundred cases have been reported in the English literature [ 4 , 7 , 8 ]. Mean age at diagnosis is 25.7 years (range: 4–75 years); 75% have been <30 years old [ 4 , 7 , 8 ].…”

“…Close to a hundred cases have been reported in the English literature [ 4 , 7 , 8 ]. Mean age at diagnosis is 25.7 years (range: 4–75 years); 75% have been <30 years old [ 4 , 7 , 8 ]. The patient in the present case was relatively old compared to the mean age for this pathology and MIB-1 was borderline (3%) but the tumor was considered as a WHO grade I tumor as histological findings were compatible to WHO grade I PGNT.…”

Glioneuronal Tumors: Insights into a Rare Tumor Entity

“…Previous limitations in diagnosis were related to limited ability to elicit radiographic differences, scant material for pathological analysis and the overall rarity of glioneuronal tumors. Ganglioglioma, paraganglioma, central neurocytoma and DNET have been reported on for some time (Figure 1) (1)(2)(3)(35)(36)(37)(38)(39)(40)(41)(42)(43)(44). RGNT (39)(40)(41), PGNT (42,43) and GNTNI (44) were added to the WHO classification in 2007 (1).…”