Prognostic significance of NAB2–STAT6 fusion variants and TERT promotor mutations in solitary fibrous tumors/hemangiopericytomas of the CNS: not (yet) clear
Grading of meningeal solitary fibrous tumors/hemangiopericytomas (SFTs/HPCs) of the central nervous system (CNS) is nowadays based on histologic criteria as described in the revised fourth edition of the WHO Classification of CNS tumors  or the more recently published, updated version of the Marseille Grading System (MGS) . Histology-based grading of CNS SFTs/HPCs allows for discriminating subgroups with significant differences in prognosis. However, the often piece meal resection of these tumors may hamper adequate evaluation of mitotic activity and necrosis, and thereby assessment of malignancy grade. NAB2–STAT6 fusion is the molecular hallmark of both soft tissue SFTs and CNS SFTs/HPCs, and the resulting fusion protein accumulates in the nucleus and acts as a transcriptional activator of early growth response mediated pathways with STAT6 immunohistochemistry being a very sensitive and specific tool for their diagnosis [5, 8, 12, 14]. For soft tissue SFTs, particular NAB2–STAT6...
This study was performed with a supporting research grant from the Stichting STOP Hersentumoren, the Netherlands.
French CNS SFT/HPC Consortium: Corinne Bouvier1,2, Philippe Cornu4, Henry Dufour5, Dominique Figarella-Branger1,3, Jacques Guyotat6, Anne Jouvet7, Nicolas Macagno1,2, Philippe Métellus2,3,4, Karima Mokhtari8, Alexandre Vasiljevic6, Pascale Varlet9. 1Department of Pathology and Neuropathology, Timone Hospital, APHM, Marseille, France; 2Aix-Marseille university, INSERM, MMG, Marseille, France; 3Aix-Marseille University, CNRS, INP, Inst Neurophysiopathol, Marseille, France; 4Department of Neurosurgery, Pitié-Salpétrière Hospital, Paris, France; 5Department of Neurosurgery, Timone Hospital, Marseille, France; 6Department of Neurosurgery, CHU Lyon, Lyon, France; 7Department of Neuropathology, CHU Lyon, Lyon, France; 8Department of Neuropathology, Pitié-Salpétrière Hospital, Paris, France; 9Department of Neuropathology, Sainte-Anne Hospital, Paris, France.
Dutch CNS SFT/HPC Consortium: R. Vogels1,2, U. Flucke1,3, B. Küsters1,4, P. Groenen1, P. Wesseling3,5, E. Bekers6, M. Verdijk1, M. Djafarihamedani1, E. Kurt7,8, H. Küsters-Vandevelde9, R. Fleischeuer10, S. Leenstra11,12, P. Robe13, W. Spliet14, D. Troost15, W. van Furth16, 1Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands; 2Department of Pathology, Stichting PAMM, Eindhoven, The Netherlands; 3Princess Máxima Center for Pediatric Oncology, Utrecht, and University Medical Center Utrecht, Utrecht, The Netherlands; 4Department of Pathology, Maastricht University Medical Center+, Maastricht, The Netherlands; 5Department of Pathology, Amsterdam Universities Medical Center/VU University Medical Center, Amsterdam, The Netherlands; 6Pathologie-DNA, Location Jeroen Bosch Hospital, Den Bosch, The Netherlands; 7Department of Neurosurgery, Radboud University Medical Center, Nijmegen, The Netherlands; 8Department of Neurosurgery, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands; 9Department of Pathology, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands; 10Department of Pathology, St. Elisabeth Hospital, Tilburg, The Netherlands; 11Department of Neurosurgery, St. Elisabeth Hospital, Tilburg, The Netherlands; 12Department of Neurosurgery, Erasmus Medical Center, Rotterdam, The Netherlands; 13Department of Neurosurgery, University Medical Center Utrecht, The Netherlands; 14Department of Pathology, University Medical Center Utrecht, The Netherlands; 15Department of Pathology, Amsterdam Medical Center, Amsterdam, The Netherlands; 16Department of Neurosurgery, Amsterdam Medical Center, Amsterdam, The Netherlands.
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