Clinicoradiological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: report of four cases and literature review.
BACKGROUND
Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a recently characterized rare tumor entity. Despite benign histological features and a reported favorable postoperative course, there is still limited clinical experience with this tumor.


METHODS
Retrospective analysis of the clinical, radiological, and surgical data in four patients with RGNT was performed. Mean age at diagnosis was 35 years, and the median follow-up was 19 (range 2-30) months. The results were compared with the literature.


RESULTS
Patient 1 presented on an emergency basis due to intratumoral hemorrhage and tumor enlargement followed by life-threatening obstructive hydrocephalus. Patient 2 suffered from headaches and left-sided hemiparesthesia 6 months prior to surgery. Patient 3 developed headaches with nausea and vomiting, followed by left-sided tinnitus 1 year prior to surgery. In patient 4, RGNT was detected incidentally. No differentiating radiological characteristics were seen except for the presence of minute satellite lesions in two patients. Histopathological findings were distinct and showed their typical biphasic neurocytic and glial architecture. No progression/recurrence was seen in the postoperative course.


CONCLUSIONS
The spectrum of presenting symptoms of RGNT is wide, nonspecific, and typically depends on tumor size and extent. This tumor entity should be considered in the differential diagnosis of posterior fossa masses in order to avoid undue surgical aggressiveness.
Marhold
Franz
F
Department of Neurosurgery, Medical University of Vienna, Währinger Gürtel 18-20, 1090, Vienna, Austria.
Preusser
Matthias
M
Dietrich
Wolfgang
W
Prayer
Daniela
D
Czech
Thomas
T
D000328:Adult; D002551:Cerebral Ventricle Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D020546:Fourth Ventricle / Q000000981:diagnostic imaging* / Q000473:pathology; D018303:Ganglioglioma / Q000000981:diagnostic imaging* / Q000473:pathology* / Q000628:therapy; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D055815:Young Adult
D005904:Glial Fibrillary Acidic Protein
Acta Neuropathol. 2003 Nov;106(5):506-8
12915951
Pathol Res Pract. 2007;203(8):613-9
17651910
Surg Neurol. 2005 Apr;63(4):375-9
15808729
Brain Pathol. 2007 Jul;17(3):308-13
17598823
Neuropathol Appl Neurobiol. 2006 Apr;32(2):217-20
16599951
J Neurooncol. 2005 Jan;71(2):195-7
15690138
Am J Surg Pathol. 2002 May;26(5):582-91
11979088
Arch Pathol Lab Med. 2007 Feb;131(2):228-33
17284107
J Radiol. 2006 Jun;87(6 Pt 2):748-63
16778745
Acta Neuropathol. 2006 Aug;112(2):231-3
16816942
J Neurosurg. 2006 Jul;105(1):129-31
16871887
Acta Neuropathol. 1995;89(4):385-90
7610772