Rationale: Rosette-forming glioneuronal tumor (RGNT) is a rare tumor which has been first reported as the fourth ventricle tumor by Komori et al and is classified as a distinct clinicopathological entity by the WHO Classification of Tumors of the Central Nervous System as in 2007. Although RGNTs were reported to occur in both supratentorial and inflatentorial sites, only 4 case reports of spinal RGNT have been demonstrated.
Patient Concerns: A 37-year-old female presenting with slowly progressing right-sided clumsiness. Cervical magnetic resonance imaging revealed a spinal intramedullary tumor between the C2 and C5 levels.
Diagnoses: Pathological analysis showed unique biphasic cellular architecture consisting of perivascular pseudorosettes dominantly with few neurocytic rosettes and diffuse astrocytoma component. The tumor cells composed of perivascular pseudorosettes showed positivity for both synaptophysin and glial markers such as GFAP and Olig2. Therefore, the diagnosis of RGNT was made.
Interventions: Gross total resection of the tumor was achieved. No adjuvant chemotherapy nor radiotherapy was conducted after operation.
Outcomes: At 2 years after the operation, no recurrence was observed.
Lessons: Although RGNT arising from the spinal cord is extremely rare, we need to consider the tumor as a differential diagnosis for intramedullary spinal cord tumors.
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http://dx.doi.org/10.1097/MD.0000000000018271 | DOI Listing |
Neuropathology
November 2024
Department of Neurosurgery and Neurooncology, 1st Medical Faculty, Charles University, Military University Hospital Prague, Prague, Czech Republic.
Rosette-forming glioneuronal tumors (RGNTs) with FGFR1 tyrosine kinase domain internal tandem duplication (FGFR1 ITD) is exceedingly rare, with only a few cases reported in the literature. Hereby we present a case of a tumor with RGNT morphology occurring in area of septum pellucidum of 43-year-old male. The tumor showed FGFR1 ITD, no PIK3CA, PIK3R1 or NF1 alterations and inconclusive methylation profile with match for class of "low-grade glial/glioneuronal/neuroepithelial tumors".
View Article and Find Full Text PDFBiomedicines
October 2024
Department of Neurosurgery, Zhongshan Hospital, Fudan University, Shanghai 200030, China.
Background: Rosette-Forming Glioneuronal Tumors (RGNTs) are rare, typically benign central nervous system tumors primarily located in the fourth ventricle and pineal region. Despite being classified as WHO grade I with generally favorable prognoses, RGNTs present complexities in their molecular mechanisms, occasional malignant transformation, and epidemiological characteristics that require further investigation.
Method: This study systematically reviews the existing literature to analyze the epidemiological patterns, MRI characteristics, pathological features, diagnostic challenges, and molecular mechanisms associated with RGNTs, aiming to provide a comprehensive theoretical foundation for clinical practice and future research.
Acta Neuropathol
August 2024
Pathology Department, Toulouse University Hospital, Toulouse, France.
World Neurosurg
October 2024
Unit of Neurosurgery, Department of Neurosciences, Santobono-Pausilipon Children's Hospital, Naples, Italy.
Tectal plate tumors are a rare subset of midbrain tumors in pediatric populations. They are slow growing and low grade, with indolent and subtle manifestation unless they cause hydrocephalus. We present a tectal tumor in an 8-year-old girl (Video 1).
View Article and Find Full Text PDFAnn Med Surg (Lond)
July 2024
Department of Neurosurgery, HClinic, Ramallah, Palestine, Palestine.
Introduction: The association between primary brain tumors, such as glioneuronal tumors, with autosomal-dominant polycystic kidney disease (ADPKD) remains poorly understood, with only two cases reported excluding this one. This case of an ADPKD patient diagnosed with a rosette-forming glioneuronal tumor highlights an exceptionally rare potential association warranting further investigation.
Case Presentation: A 28-year-old male with ADPKD presented with progressive ataxia, dizziness, and headache.
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