Bielle Franck, Villa Chiara, Giry Marine, Bergemer-Fouquet Anne-Marie, Polivka Marc, Vasiljevic Alexandre, Aubriot-Lorton Marie-Hélène, Bernier Michèle, Lechapt-Zalcman Emmanuèle, Viennet Gabriel, Sazdovitch Véronique, Duyckaerts Charles, Sanson Marc, Figarella-Branger Dominique, Mokhtari Karima
*Neuropathology Department Raymond-Escourolle ***AP-HP, Neurology 2 Mazarin Department, Pitié-Salpêtrière Hospital §§§Onconeurotek, Pitié-Salpêtrière Hospital ††Pathology Department, Hôpital Lariboisière, Assistance publique - Hôpitaux de Paris †Sorbonne Universités, UPMC Univ Paris 06, UM 75 ‡Institut National de la Santé et de la Recherche Médicale, U 1127 §Centre National de la Recherche Scientifique, UMR 7225, ICM ∥Brain and Spine Institute #Institut National de la Santé et de la Recherche Médicale, U1016, Institut Cochin, Université Paris Descartes, Paris ¶Department of Anatomical and Cytological Pathology, Hôpital Foch, Suresnes **Pathology Department, Hôpital Bretonneau, CHRU Tours, Tours ‡‡Pathology and Neuropathology Department, Centre de Biologie et Pathologie Est, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron §§Pathology Department, Plateau technique de Biologie, CHU, Dijon ∥∥Department of Pathology, Caen University Hospital ¶¶Centre National de la Recherche Scientifique, UMR 6301 ISTCT CERVOxy Group, Caen ##Pathology Department, CHU Besançon, Besançon †††Pathology and Neuropathology Department, Assistance Publique-Hôpitaux de Marseille, AP-HM CHU, Timone ‡‡‡Aix-Marseille university, INSERM U911, Marseille, France.
Am J Surg Pathol. 2015 Jul;39(7):948-56. doi: 10.1097/PAS.0000000000000421.
Chordoid glioma of the third ventricle (CG3V) is a rare tumor developing in a stereotyped localization. It has been related to the circumventricular organ of the lamina terminalis, in the anterior part of the third ventricle, but its oncogenesis is poorly understood. TTF-1 transcription factor is involved in the development and adult physiology of the ventral forebrain. We studied the histopathologic and immunohistochemical features of a multicentric series of 17 cases of CG3V. We described additional histologic patterns (solid, fibrosing, and fusiform) to the typical chordoid pattern. TTF-1 was constantly expressed in CG3V, as in developing and adult lamina terminalis. The anti-TTF-1 SPT24 clone was more sensitive than the 8G7G3/1 clone. No mutation of IDH1 R132, IDH2 R172, or BRAF V600 codons was found. We showed TTF-1 as a useful marker for the diagnosis of CG3V and the understanding of its oncogenesis.
第三脑室脊索样胶质瘤（CG3V）是一种发生于特定部位的罕见肿瘤。它与第三脑室前部终板室周器官有关，但其肿瘤发生机制尚不清楚。TTF-1转录因子参与腹侧前脑的发育和成年期生理功能。我们研究了17例多中心CG3V病例的组织病理学和免疫组化特征。我们描述了除典型脊索样模式外的其他组织学模式（实性、纤维化和梭形）。TTF-1在CG3V中持续表达，如同在发育中的和成年的终板中一样。抗TTF-1 SPT24克隆比8G7G3/1克隆更敏感。未发现IDH1 R132、IDH2 R172或BRAF V600密码子的突变。我们表明TTF-1是诊断CG3V及其肿瘤发生机制研究的有用标志物。
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