Clinicopathologic and neuroradiologic studies of papillary glioneuronal tumors

Abstract

PGNTs display distinct clinicopathologic and imaging characteristics and indicate a favorable prognosis. However, recurrences sometimes occur. Immunohistochemistry facilitates the appropriate diagnosis of these tumors. Complete resection of the tumor is important for a favorable outcome.

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.…”

“…Some reports have described genetic alternations in PGNT, including MGMT methylation [ 3 ] and negative results for IDH 1 and EGFR mutation [ 4 ]. BRAF V600E mutation in PGNT has not been described in the literature.…”

Uncommon low-grade brain tumors

“…These tumors can occur in both sexes, with the median age at presentation being 23 years. 17,18 They are usually hemispheric, mainly in the temporal lobe, but also can involve the paraventricular white matter. Clinical presentation is nonspecific, with headache and seizures common.…”

“…18 Radiologically, they are comparable to gangliogliomas, being cystic lesions with a mural nodule that may enhance with contrast and can be located close to the ventricles. 17,19 Histologically, they are defined by the presence of pseudopapillary structures composed of a central fibrovascular core with a layer of spindled to cuboidal cells strongly immunoreactive for glial fibrillary acidic protein (GFAP). There is also typically an interpapillary population of synaptophysin-positive neurocytic cells.…”