Del Baldo Giada, Vennarini Sabina, Toniutti Maristella, Abbas Rachid, Lorentini Stefano, Piccirilli Eleonora, Cacchione Antonella, Megaro Giacomina, Di Ruscio Valentina, De Ioris Maria Antonietta, De Salvo Andrea, Albino Giulia, Rossi Sabrina, Colafati Giovanna Stefania, Carai Andrea, Mastronuzzi Angela
Department of Pediatric Haematology and Oncology, and Cell and Gene Therapy Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.
Front Oncol. 2023 Oct 4;13:1259403. doi: 10.3389/fonc.2023.1259403. eCollection 2023.
Germ cell tumors (GCT) account for a minority of central nervous system (CNS) malignancies, highly prevalent in adolescents and young adults. Despite their aggressive biological behavior, prognosis is excellent in most cases with risk stratified treatment, consisting in a combination of chemotherapy and radiotherapy. Whole ventricular irradiation (WVI) and craniospinal irradiation, the treatment of choice for localized and metastatic disease, pose significant risk of collateral effects, therefore proton beam radiation (PBT) has been recently proposed for its steep dose fallout.
We report our experience in a consecutive series of 17 patients treated for CNS GCT at our Institution from 2015 to 2021.
Most frequent lesion location were sellar/suprasellar (35%) and bifocal germinoma (35%), followed by pineal (18%) and thalamic (12%). Two patients (12%), had evidence of disseminated disease at the time of diagnosis. At the latest follow-up all but one patient showed complete response to treatment. The only relapse was successfully rescued by additional chemotherapy and PBT. PBT was well tolerated in all cases. No visual, neurological or endocrinological worsening was documented during and after treatment. Neuropsychological evaluation demonstrated preservation of cognitive performance after PBT treatment.
Our data, albeit preliminary, strongly support the favourable therapeutic profile of PBT for the treatment of CNS germ cell tumors.
生殖细胞肿瘤(GCT)占中枢神经系统(CNS)恶性肿瘤的少数,在青少年和年轻成年人中高度流行。尽管其生物学行为具有侵袭性,但在大多数情况下,通过风险分层治疗(包括化疗和放疗的联合),预后良好。全脑室照射(WVI)和全脑全脊髓照射是局限性和转移性疾病的首选治疗方法,会带来显著的副作用风险,因此最近有人提出使用质子束放疗(PBT),因为其剂量下降陡峭。
我们报告了2015年至2021年在我们机构连续治疗的17例中枢神经系统GCT患者的经验。
最常见的病变部位是鞍区/鞍上区(35%)和双灶性生殖细胞瘤(35%),其次是松果体区(18%)和丘脑区(12%)。两名患者(12%)在诊断时已有播散性疾病的证据。在最近的随访中,除一名患者外,所有患者对治疗均显示完全缓解。唯一的复发通过额外的化疗和PBT成功挽救。所有病例对PBT耐受性良好。治疗期间和治疗后均未记录到视力、神经或内分泌功能恶化。神经心理学评估表明,PBT治疗后认知功能得以保留。
我们的数据尽管是初步的,但有力地支持了PBT治疗中枢神经系统生殖细胞肿瘤的良好治疗效果。