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. 2013 Sep;23(5):574-83.
doi: 10.1111/bpa.12048. Epub 2013 Mar 20.

Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E) mutation and expression

Céline Chappé  1 , Laetitia Padovani, Didier Scavarda, Fabien Forest, Isabelle Nanni-Metellus, Anderson Loundou, Sandy Mercurio, Frédéric Fina, Gabriel Lena, Carole Colin, Dominique Figarella-Branger
Affiliations

Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E) mutation and expression

Céline Chappé et al. Brain Pathol. 2013 Sep.

Abstract

Pediatric cortical glioneuronal benign tumors mainly include gangliogliomas (GG) [differential diagnoses pilocytic astrocytomas (PA) and pleomorphic xanthoastrocytomas (PXA)] and dysembryoplastic neuroepithelial tumor (DNT). DNT include the specific form and the controversial non-specific form that lack the specific glioneuronal element. Our aims were to search for BRAF(V600E) mutation and CD34 expression in DNT, PXA, GG and PA to correlate BRAF(V600E) mutation with BRAF(V600E) expression and to evaluate their diagnostic and prognostic values. Ninety-six children were included. BRAF(V600E) mutation was studied by sequencing and immunohistochemistry; CD34 expression was analyzed by immunohistochemistry. BRAF(V600E) mutation was detected in PXA (60%), GG (38.7%), DNT (30%, including 3/11 specific and 3/9 non-specific forms) and PA (12.5%). BRAF(V600E) expression was recorded in PXA (60%), GG (45.2%) and DNT (30%). CD34 expression was recorded in PXA (60%), GG (58.1%), DNT (25%) and PA (12.5%). Neither CD34 expression nor BRAF(V600E) status was predictive of prognosis, except for PA tumors where CD34 expression was associated with a shorter overall survival. In conclusion, DNT shared with PXA and GG, BRAF(V600E) mutation and/or CD34 expression, which represent molecular markers for these tumors, and we recommend searching for CD34 expression and BRAF(V600E) mutation in all DNT, especially the non-specific forms.

Keywords: BRAFV600E expression; BRAFV600E mutation; CD34; dysembryoplastic neuroepithelial tumors (DNT); gangliogliomas (GG); pleomorphic xanthoastrocytomas (PXA).

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Conflict of interest statement

The authors declare that they have no conflict of interest.

Figures

Figure 1
Figure 1
Photomicrographs showing the histopathological features of a specific form of a dysembryoplastic neuroepithelial tumor (DNT). A–C. Glial nodule of a complex DNT (DNT 11): A. hematoxylin‐eosin (HE) stain; B. negative CD34 immunostaining; and C. positive BRAFV600E immunostaining. D–F. Glioneuronal elements (DNT 3): D. HE stain; E. negative CD34 immunostaining; and F. negative BRAFV600E immunostaining. Original magnification: ×400 (A–D); ×200 (E, F).
Figure 2
Figure 2
Photomicrographs showing the histopathological features of non‐specific forms of dysembryoplastic neuroepithelial tumor (DNT). A–C. Case DNT 2: A. hematoxylin‐eosin (HE) stain, oligodendroglioma‐like pattern; B. diffuse CD34 immunostaining; and C. negative BRAFV600E immunostaining. D–F. Case DNT 16: D. HE stain, oligodendroglioma‐like pattern; E. diffuse CD34 immunostaining; and F. positive BRAFV600E immunostaining. Original magnification: ×400 (A–E); ×200 (F).
Figure 3
Figure 3
Photomicrographs showing the histopathological features of gangliogliomas (GGs) and pleomorphic xanthoastrocytomas (PXAs). A–C. GG 23: A. hematoxylin‐eosin (HE) stain (arrow: ganglionic cell); B. solitary CD34 immunostaining (arrow: positive cell); and C. positive BRAFV600E immunostaining. D–F. PXA 3. D. HE stain, pleomorphic cells of classic PXA; E. diffuse CD34 immunostaining; and F. positive BRAFV600E immunostaining. Original magnification: ×400 (A–C); ×200 (D–F).
Figure 4
Figure 4
A. Overall survival (OS, in months) according to pathological variants: pilocytic astrocytomas (PAs) and pilomyxoid astrocytomas (PMAs), pleomorphic xanthoastrocytomas (PXAs), gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNTs) (specific and non‐specific form: S + NS). B. OS according to tumor location whatever the pathological variant. C. OS according to CD34 expression in the PA‐PMA subgroup. Because no event was recorded in GG and DNT, the two curves are superimposed (A). Th same is recorded in (B) for cerebellum + posterior fossa and spinal cord + brainstem locations.
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