Muliang Jiang1, Liling Long2, Jingjing Zeng3, Wei Meng4, ChiShing Zee5. 1. Department of Radiology, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China. 2. Department of Radiology, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China. cjr.longliling@vip.163.com. 3. Department of Pathology, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, Guangxi, China. 4. Department of Radiology, Shunde Hospital of Southern Medical University, Shunde, 528308, Guangdong, China. 5. Department of Radiology, Keck School of Medicine, University of Southern California, Los Angeles, CA, 90033, USA.
Abstract
PURPOSE: Extraventricular neurocytoma (EVN) is an exceedingly rare tumor. In this study, we sought to characterize the imaging and pathological features of this uncommon tumor. METHODS: Retrospective review of 18 patients (9 male; 9 female) with pathologically confirmed EVN treated at a single center between 2005 and 2017. RESULTS: All patients had a solitary lesion. Sixteen lesions were found in hemispheres. The greatest tumor diameter ranged from 2.6 to 8 cm. The lesions were generally solid with cystic components; the solid portion appeared isodense or hyperdense on CT, isointense to hypointense on T1WI, and slightly hyperintense on T2WI. Heterogeneous hyperintensity interspersed with isointense or hypointense areas suggestive of hemorrhage, calcification or vascular flow voids were seen on T2WI. Heterogeneous enhancement was noted in 17 lesions; no enhancement was observed in one lesion. Cystic components were observed in 13 lesions; 9 of these showed characteristic perilesional cysts (9/13). Mild to moderate peritumoral edema (15/18), calcification (4/16), intratumoral hemorrhage (11/18) and vascular flow voids (10/16) were observed in some lesions. Pathologically, tumor cells showed round nucleus and fine neuropil matrix. Foci of calcification in the solid portion of the tumor were seen in five cases. Microcystic changes were observed in almost all cases. Some lesions exhibited positive staining for synaptophysin (Syn) (15/16) and neuronal nuclei (NeuN) (7/8). MIB-1 was determined for 10 patients; seven of these had an MIB-1 ≥ 3. These six patients experienced recurrence; four of them relapsed twice. CONCLUSIONS: EVNs occur as single intracranial solid mass with cystic components (especially peripherally located cysts); solid portion exhibits slight hyperintensity or heterogeneous signal intensity. Mild to moderate peritumoral edema, calcification, intratumoral hemorrhage and vascular flow voids were characteristic features of extraventricular neurocytoma. Positive staining for synaptophysin and neuronal nuclei confirmed the diagnosis. A combination of atypical pathologic features and atypical radiologic features should be considered for prognostic assessment.
PURPOSE: Extraventricular neurocytoma (EVN) is an exceedingly rare tumor. In this study, we sought to characterize the imaging and pathological features of this uncommon tumor. METHODS: Retrospective review of 18 patients (9 male; 9 female) with pathologically confirmed EVN treated at a single center between 2005 and 2017. RESULTS: All patients had a solitary lesion. Sixteen lesions were found in hemispheres. The greatest tumor diameter ranged from 2.6 to 8 cm. The lesions were generally solid with cystic components; the solid portion appeared isodense or hyperdense on CT, isointense to hypointense on T1WI, and slightly hyperintense on T2WI. Heterogeneous hyperintensity interspersed with isointense or hypointense areas suggestive of hemorrhage, calcification or vascular flow voids were seen on T2WI. Heterogeneous enhancement was noted in 17 lesions; no enhancement was observed in one lesion. Cystic components were observed in 13 lesions; 9 of these showed characteristic perilesional cysts (9/13). Mild to moderate peritumoral edema (15/18), calcification (4/16), intratumoral hemorrhage (11/18) and vascular flow voids (10/16) were observed in some lesions. Pathologically, tumor cells showed round nucleus and fine neuropil matrix. Foci of calcification in the solid portion of the tumor were seen in five cases. Microcystic changes were observed in almost all cases. Some lesions exhibited positive staining for synaptophysin (Syn) (15/16) and neuronal nuclei (NeuN) (7/8). MIB-1 was determined for 10 patients; seven of these had an MIB-1 ≥ 3. These six patients experienced recurrence; four of them relapsed twice. CONCLUSIONS:EVNs occur as single intracranial solid mass with cystic components (especially peripherally located cysts); solid portion exhibits slight hyperintensity or heterogeneous signal intensity. Mild to moderate peritumoral edema, calcification, intratumoral hemorrhage and vascular flow voids were characteristic features of extraventricular neurocytoma. Positive staining for synaptophysin and neuronal nuclei confirmed the diagnosis. A combination of atypical pathologic features and atypical radiologic features should be considered for prognostic assessment.
Entities:
Keywords:
Central neurocytomas; Cerebral; Extraventricular neurocytomas; Imaging characteristics; Pathological
Authors: Claudia Gaggiotti; Giuseppe Roberto Giammalva; Marco Raimondi; Ada Maria Florena; Rosa Maria Gerardi; Francesca Graziano; Silvana Tumbiolo; Domenico Gerardo Iacopino; Rosario Maugeri Journal: Surg Neurol Int Date: 2021-03-08