Cerebellar liponeurocytoma with atypical histological features – a rare example of a glioneuronal tumor

Abstract OBJECTIVE AND IMPORTANCE Liponeurocytomas are rare cerebellar neoplasms in adults, with benign histological features and a favorable clinical prognosis. Current clinical opinion is based on a total of less than 20 published cases and suggests that gross total resection and long-term follow-up monitoring, with possible additional surgery and radiotherapy for treatment of recurrent tumors, represent the best treatment approach for this relatively benign tumor type. CLINICAL PRESENTATION A 51-year-old Caucasian woman presented with worsening unsteady gait and headaches, suggesting increased intracranial pressure. INTERVENTION The patient underwent subtotal resection of a cerebellar liponeurocytoma, followed by fractionated radiotherapy (total dose of 54 Gy). She experienced a local recurrence of the tumor 12 months later and underwent additional surgery for removal of the cerebellar mass. A second recurrent tumor was diagnosed on magnetic resonance imaging scans 3 months later and was surgically resected. The tumor histological findings were consistently devoid of atypical features, apart from leptomeningeal invasion noted in the first surgical specimen. CONCLUSION This unusual case demonstrated an atypical clinical course of a highly aggressive and radiation-resistant tumor, despite the consistent absence of aggressive histological features. Cerebellar liponeurocytomas may not be as benign as the current literature and typical low-grade cytological and histological features suggest.

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Glioneuronal Tumor With Features of Ganglioglioma and Neurocytoma Arising in the Fourth Ventricle: A Report of 2 Unusual Cases and a Review of Infratentorial Gangliogliomas

Journal of Neuropathology & Experimental Neurology ◽

10.1093/jnen/nlz060 ◽

2019 ◽

Vol 78 (9) ◽

pp. 780-787 ◽

Cited By ~ 1

Author(s):

William Harrison ◽

Aladine A Elsamadicy ◽

J Tanner McMahon ◽

Gustavo Chagoya ◽

Raymond A Sobel ◽

...

Keyword(s):

Surgical Resection ◽

Clinical Significance ◽

Fourth Ventricle ◽

Surgical Excision ◽

Glioneuronal Tumor ◽

Low Grade ◽

Dysplastic Gangliocytoma ◽

Comprehensive Review ◽

Papillary Glioneuronal Tumor ◽

Cerebellar Liponeurocytoma

Abstract Infratentorial glioneuronal neoplasms are overall quite rare and are more commonly low-grade with surgical excision usually being curative. Multiple distinct histologic entities have been described including rosette-forming glioneuronal tumor, papillary glioneuronal tumor, neurocytoma, dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease), cerebellar liponeurocytoma, and ganglioglioma. While each of these entities has distinct findings, in some instances a tumor may demonstrate overlapping histologic features with mixed components. Herein, we report 2 unusual adult cases of a fourth ventricular glioneuronal tumor with features of ganglioglioma and neurocytoma, with one coming from a surgical resection and one found incidentally at autopsy. To the best of our knowledge, this specific histologic combination has not previously been described. As such, the clinical significance is unknown although in both cases the neoplasms were circumscribed and appeared to be low grade. The presence of the gangliogliomatous component was of particular interest since these are extremely rare occurrences in the fourth ventricle and we provide a comprehensive review of infratentorial gangliogliomas.

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MULTIFOCAL COMPLEX GLIONEURONAL TUMOR IN AN ELDERLY MAN

Neurosurgery ◽

10.1227/01.neu.0000345640.40566.48 ◽

2009 ◽

Vol 64 (6) ◽

pp. E1193-E1195 ◽

Cited By ~ 18

Author(s):

Jian-Qiang Lu ◽

Bernd W. Scheithauer ◽

Pranshu Sharma ◽

James N. Scott ◽

Ian F. Parney ◽

...

Keyword(s):

Obstructive Hydrocephalus ◽

External Ventricular Drain ◽

Third Ventricle ◽

Glioneuronal Tumor ◽

Third Ventriculostomy ◽

Magnetic Resonance Imaging Scan ◽

Dysembryoplastic Neuroepithelial Tumor ◽

Histological Features ◽

The Third ◽

Glioneuronal Tumors

Abstract OBJECTIVE The clinicopathological spectra of a dysembryoplastic neuroepithelial tumor (DNT) and a rosette-forming glioneuronal tumor (RGNT) are expanding. We report here the autopsy findings of a case of complex glioneuronal tumor with combined histological features of both a DNT and an RGNT. CLINICAL PRESENTATION A 79-year-old man presented with a 1-month history of confusion and gait difficulties. A magnetic resonance imaging scan revealed obstructive hydrocephalus attributed to a mass in the posterior third ventricle. INTERVENTION A third ventriculostomy was performed. Postoperatively, the mass remained unchanged in size for more than 14 months. Thirty-eight months after his initial manifestations, he experienced minor head trauma and was then hospitalized. Despite placement of an external ventricular drain and other supportive treatment, he deteriorated and died. A full autopsy was performed, with emphasis on the brain. The mass lesion and a few independent microfoci situated primarily around the third ventricle showed histological features of pilocytic astrocytoma with recurrent hemorrhage. Far more numerous were microfoci with histological features of a DNT, including floating neurons, as well as typical RGNT-associated, synaptophysin-positive rosettes and perivascular pseudorosettes. CONCLUSION The advanced age of the patient, the coexisting histological features of the DNT and RGNT, and the distinctive anatomic distribution of the lesions, being centered on the third ventricle, may lend insight into the histogenetic relationship of a DNT, an RGNT, and mixed glioneuronal tumors.

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