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Case Reports
. 2019 Jan 5:2:100006.
doi: 10.1016/j.wnsx.2018.100006. eCollection 2019 Apr.

Malignant Transformation of a Rosette-Forming Glioneuronal Tumor with IDH1 Mutation: A Case Report and Literature Review

Ronie Romelean Jayapalan  1 , Kein Seong Mun  2 , Kum Thong Wong  2 , Sheau Fung Sia  1
Affiliations
Case Reports

Malignant Transformation of a Rosette-Forming Glioneuronal Tumor with IDH1 Mutation: A Case Report and Literature Review

Ronie Romelean Jayapalan et al. World Neurosurg X. .

Abstract

Background: Rosette-forming glioneuronal tumor (World Health Organization grade I) is considered as a benign tumor with very low potential for progression. The potential for malignant transformation of this tumor is not known and has never been reported before in literature.

Case description: We report a 42-year-old man, diagnosed with rosette-forming glioneuronal tumor of the fourth ventricle with a positive isocitrate dehydrogenase 1 mutation, progressed to glioblastoma after 6 years from diagnosis. We discuss the clinical history, radiological findings, and histopathological characteristic with immunohistochemistry findings observed in this unique case.

Conclusions: Despite being acceptable as benign, based on our observations in this case, there is a potential for malignant transformation of rosette-forming glioneuronal tumor. The role of isocitrate dehydrogenase 1 mutation leading to malignant transformation could not be established as our finding is novel and further prospective studies are required to prove this association.

Keywords: Glioblastoma IDH mutant; IDH 1 mutation; IDH 1, Isocitrate dehydrogenase 1; MRI, Magnetic resonance imaging; Malignant transformation; RGNT; RGNT, Rosette-forming glioneuronal tumor; WHO, World Health Organization.

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Figures

Figure 1
Figure 1
Initial magnetic resonance imaging in 2010-axial post-contrast T1-weighted images showing a small enhancing lesion with much larger areas of hypointensity along the cerebellar vermis and fourth ventricle.
Figure 2
Figure 2
(A) Neurocytes forming rosettes against a loose myxoid matrix (hematoxylin & eosin, original magnification ×100). (B) Glial component with a distinct oligodendroglioma-like appearance. There was no necrosis, mitosis, or any microvascular proliferation (hematoxylin & eosin, original magnification ×100). (C) IDH 1 immunostaining of rosette-forming glioneuronal tumor in 2010.
Figure 3
Figure 3
Magnetic resonance imaging in 2011—showing residual tumor postoperative period at 6 months.
Figure 4
Figure 4
Magnetic resonance imaging in 2012—unchanged appearance of residual tumor at 2 years after surgery.
Figure 5
Figure 5
Magnetic resonance imaging in 2016—on recurrence of symptoms, T1-weighted post-contrast images showing an enhancing mass with central necrosis.
Figure 6
Figure 6
Postoperative magnetic resonance imaging after gross total resection in 2016.
Figure 7
Figure 7
(A) Glial tumor showing increased cellularity, cytological dysplasia, and mitosis (thick black arrow) (hematoxylin & eosin, original magnification ×200). (B) High-grade tumor showing geographic necrosis (hematoxylin & eosin, original magnification ×100). (C) Microvascular proliferation (hematoxylin & eosin, original magnification ×100). (D) IDH 1 immunostaining of glioblastoma in 2016.
See this image and copyright information in PMC

References

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