Zhang Yifan, He Jialin, Xue Wei, Fang Jingqin, Du Xuesong
Department of Radiology, The Second Affiliated Hospital of Anhui Medical University, Hefei, China.
Department of Stomatology, The 940th Hospital of Joint Logistic Support Force of Chinese Peoples Liberation Army, Lanzhou, China.
Front Oncol. 2025 Aug 13;15:1598058. doi: 10.3389/fonc.2025.1598058. eCollection 2025.
Papillary glioneuronal tumors (PGNTs) are classified by the World Health Organization (WHO) as Grade I neoplasms, with only sporadic reports of anaplastic variants demonstrating aggressive clinical behavior and distinct histopathological characteristics. This study presents two cases of anaplastic PGNT, including one that ultimately progressed to glioblastoma (WHO Grade IV). The first case involved a 47-year-old female patient without a history of seizures. Magnetic resonance imaging (MRI) revealed an irregular mass containing multiple cysts and a mural nodule in the left parietal lobe. Histopathological examination confirmed the diagnosis of anaplastic PGNT. Nineteen months later, follow-up brain MRI demonstrated a recurrent mass at the prior surgical site. During the second resection, histological analysis identified glioblastoma arising from the glial component of the original tumor. The second case concerned a 7-year-old boy presenting with progressive headache. MRI showed a solid-cystic mass in the right frontal lobe accompanied by marked peritumoral edema. Postoperative pathological examination revealed anaplastic PGNT with extensive necrosis. MRI characteristics including prominent peritumoral edema, ring-enhancing cyst walls, restricted diffusion, and elevated lipid/lactate peaks may predict the aggressive nature of anaplastic PGNT. Furthermore, this case series suggests that anaplastic PGNTs harbor malignant potential to transform into more aggressive neoplasms.
乳头状胶质神经元肿瘤(PGNTs)被世界卫生组织(WHO)归类为I级肿瘤,仅有零星报道称间变性变体表现出侵袭性临床行为和独特的组织病理学特征。本研究报告了2例间变性PGNT病例,其中1例最终进展为胶质母细胞瘤(WHO IV级)。首例病例为一名47岁女性患者,无癫痫病史。磁共振成像(MRI)显示左顶叶有一个不规则肿块,包含多个囊肿和一个壁结节。组织病理学检查确诊为间变性PGNT。19个月后,脑部MRI随访显示原手术部位有复发性肿块。在第二次切除术中,组织学分析确定胶质母细胞瘤起源于原肿瘤的胶质成分。第二例病例为一名7岁男孩,表现为进行性头痛。MRI显示右额叶有一个实性囊性肿块,伴有明显的瘤周水肿。术后病理检查显示间变性PGNT伴广泛坏死。MRI特征,包括明显的瘤周水肿、环形强化的囊肿壁、扩散受限以及脂质/乳酸峰升高,可能预示着间变性PGNT的侵袭性。此外,该病例系列表明,间变性PGNTs具有转变为更具侵袭性肿瘤的恶性潜能。