Department of Radiology, Bahçeşehir University School of Medicine, Göztepe Medical Park Training and Education Hospital, Istanbul, Turkey.
Department of Pathology, Bahçeşehir University School of Medicine, Göztepe Medical Park Training and Education Hospital, E5 Üzeri Merdivenköy, 23 Nisan Sokagi No: 17, 34732 Kadıköy, Istanbul, Turkey.
Curr Med Imaging. 2023;19(12):1415-1426. doi: 10.2174/1573405619666230213152949.
Diffuse leptomeningeal glioneuronal tumors (DL-GNT) are rare glioneuronal neoplasms with oligodendroglioma-like cells. These tumors can present as a dominant intracranial mass or as a solitary spinal cord mass without leptomeningeal involvement. In this study, we aimed to determine the magnetic resonance imaging and histopathological features, treatment modalities, and clinical outcomes of the parenchymal forms of DL-GNTs.
This is a retrospective three-center case series study of 5 patients with a confirmed parenchymal form of DLGTs, out of which 4 patients were adults. Brain and spinal cord MR imaging were performed in all patients at either 1.5 or 3T. The patients' age ranged from 5 years to 50 years with a mean age of 27.6 years at presentation.
Four of the tumors were located in the frontal lobe, and one in the tectum. They were usually solid-cystic enhancing tumors as the other mixed neuronal-glial tumors. All of the tumors had an extension to the superficial surface of a cerebral hemisphere. One had systemic bone metastases. The clinical signs and symptoms of the parenchymal form varied based on the location of the mass, in contrast to the leptomeningeal form associated with hydrocephalus. In one case, the tumor's initial grade was defined as intermediate. The initial histopathology of the two cases was low-grade and no upgrade occurred in the follow-up period. In two cases, although the tumors were low grade initially, they progressed to an anaplastic form in the follow-up period.
The parenchymal form of DL-GNTs is common in adults. Extension to the superficial surface of a cerebral hemisphere is a distinctive imaging feature. Systemic osseous metastasis may occur. Due to the presence of common histopathological features, including the biphasic composition of glial and neuronal cell elements and oligodendroglioma-like cells, a proposed classification approach might be more beneficial for the histopathological and imaging description, and management of the glioneuronal tumors with oligodendroglioma-like features.
弥漫性软脑膜胶质神经元肿瘤（DL-GNT）是一种罕见的具有少突胶质细胞瘤样细胞的胶质神经元肿瘤。这些肿瘤可以表现为颅内占位性病变，也可以表现为单纯的脊髓占位性病变，而无软脑膜受累。本研究旨在探讨 DL-GNT 实质型的磁共振成像（MRI）和组织病理学特征、治疗方式和临床转归。
本研究为回顾性三中心病例系列研究，纳入 5 例经组织学证实的 DLGT 实质型患者，其中 4 例为成人。所有患者均行 1.5T 或 3T 脑脊髓 MRI 检查。患者年龄 5~50 岁，平均 27.6 岁。
4 例肿瘤位于额叶，1 例位于脑桥。肿瘤通常为实性-囊性强化肿块，类似于其他混合性神经元-胶质肿瘤。所有肿瘤均向大脑半球表面延伸。1 例患者合并全身骨转移。与软脑膜受累相关的脑积水不同，实质型的临床症状和体征取决于肿瘤的位置。1 例患者初始肿瘤分级为间变。2 例患者初始组织病理学分级为低级别，随访期内未升级。2 例患者虽然初始肿瘤分级为低级别，但在随访期内进展为间变。
DL-GNT 实质型在成人中常见。向大脑半球表面延伸是其独特的影像学特征。可能发生全身骨转移。由于存在常见的组织病理学特征，包括胶质和神经元细胞成分以及少突胶质细胞瘤样细胞的双相组成，因此提出的分类方法可能更有助于胶质神经元肿瘤伴少突胶质细胞瘤样特征的组织病理学和影像学描述及管理。
