Novel <i>NOTCH3</i> alteration expanding the molecular spectrum of pericytic tumours: report of two cases

Our findings suggest that NOTCH3 ITDs represent a novel oncogenic mechanism in pericytic tumour pathogenesis, likely driving constitutive activation of the NOTCH signalling pathway. Given the potential therapeutic relevance, particularly in aggressive or life-threatening cases with CNS involvement, our findings highlight the importance of extensive molecular profiling. Targeted therapy with NOTCH inhibitors may represent a promising strategy in the management of aggressive cases of ITD-driven pericytic tumours.

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Publication Details

Journal
Histopathology
Published
2025-11-20
DOI
https://doi.org/10.1111/his.70051
Primary Topic
Cerebrovascular and genetic disorders
Type
article
Field-Weighted Citation Impact
0.00

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article

Novel NOTCH3 alteration expanding the molecular spectrum of pericytic tumours: report of two cases

Mégane Le Quang, Rihab Azmani, François Le Loarer, Guillaume Chotard et al.
Histopathology
Cerebrovascular and genetic disorders
article

Novel NOTCH3 alteration expanding the molecular spectrum of pericytic tumours: report of two cases

Mégane Le Quang, Rihab Azmani, François Le Loarer, Guillaume Chotard, Henri Margot, Irena Antonia Ungureanu, Nathalène Truffaux, Marie Ancelle
article en

Abstract

Our findings suggest that NOTCH3 ITDs represent a novel oncogenic mechanism in pericytic tumour pathogenesis, likely driving constitutive activation of the NOTCH signalling pathway. Given the potential therapeutic relevance, particularly in aggressive or life-threatening cases with CNS involvement, our findings highlight the importance of extensive molecular profiling. Targeted therapy with NOTCH inhibitors may represent a promising strategy in the management of aggressive cases of ITD-driven pericytic tumours.

HistopathologyVol. 88(4)
Université de Bordeaux (FR), Inserm (FR), Université de Versailles Saint-Quentin-en-Yvelines (FR), Bordeaux Population Health (FR), Hôpital Pellegrin (FR), Hôpital Ambroise-Paré (FR), Institut Bergonié (FR)
Ligue Contre le Cancer
Openalex Percentile: Top 51%
Cerebrovascular and genetic disorders
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Novel <i>NOTCH3</i> alteration expanding the molecular spectrum of pericytic tumours: report of two cases — Mégane Le Quang, Rihab Azmani, et al. · Histopathology (2025) | TGRS Research Map | TGRS