Mosteiro Alejandra, Diao Diouldé, Bedia Carmen, Pedrosa Leire, Caballero Gabriela Ailén, Aldecoa Iban, Mallo Mar, Solé Francesc, Sevilla Ana, Ferrés Abel, Cabrera Gloria, Muñoz-Tudurí Marta, Centellas Marc, Pineda Estela, Jiménez Àngels Sierra, González Sánchez José Juan
Laboratory of Experimental Oncological Neurosurgery, Neurosurgery Service, Hospital Clinic de Barcelona-FCRB, 08036 Barcelona, Spain.
Department of Neurosurgery, Hospital Clínic de Barcelona, Universitat de Barcelona, 08036 Barcelona, Spain.
Int J Mol Sci. 2025 Sep 12;26(18):8889. doi: 10.3390/ijms26188889.
Glioblastoma (GB) is one of the most aggressive brain tumors, characterized by high infiltrative capacity that enables tumor cells to invade healthy brain tissue and evade complete surgical resection. This invasiveness contributes to resistance against conventional therapies and a high recurrence rate. Strategies capable of eliminating residual tumor cells are urgently needed. Photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA), an FDA- and EMA-approved compound, induces selective accumulation of the photosensitizer protoporphyrin IX (PpIX) in metabolically active tumor cells, enabling targeted cytotoxicity through light activation. A major limitation to its clinical application is the unclear variation in the cytotoxic effect of PDT according to individual tumoral differences. In this study, we propose and validate an in vivo model of patient-derived GB initiating cells (GICs) and brain organoids to test the effects of PDT. First, patient-derived GICs were molecularly characterized by flow cytometry and copy number variation profiling using OncoScan CNV Assays, then co-cultured with human brain organoids to generate a hybrid model recapitulating key aspects of the tumor microenvironment. 5-ALA photodynamic therapy (PDT) efficacy was assessed in vitro by GFP-based viability measurements, LDH release assays, and TUNEL staining. Then, a murine model was generated to study PDT in vivo, based on a heterotopic (renal subcapsular engraftment) xenograft of the GICs-human brain organoid co-culture. PDT was tested in the model; in each subject, one kidney tumoral engraftment was treated and the contralateral served as a control. Immunofluorescence analysis was used to study the cell composition of the brain organoid-tumoral engraftment after PDT, and the effects on non-GIC cells. The antitumoral effect was determined by the degree of cell death analysis with the TUNEL technique. The GICs-brain organoid co-culture resulted in tumoral growth and infiltration both in vitro and in vivo. The pattern of growth and infiltration varied according to the tumoral genetic profile. 5-ALA PDT resulted in a reduction in the number of GICs and an increase in apoptotic cells in all four lines tested in vitro. A correlation was found between the induced phototoxicity in vivo with the molecular typification of GICs cell lines in vitro. There were no changes in the number or distribution of neuronal cells after the application of PDT, while a reduction in active astrocytes was observed. 5-ALA PDT could be effective in eradicating GICs with a heterogeneous molecular profile. The hybrid human-murine model presented here could be useful in investigating adjuvant therapies in GB, under the concept of personalized medicine.
胶质母细胞瘤(GB)是最具侵袭性的脑肿瘤之一,其特征是具有高浸润能力,使肿瘤细胞能够侵入健康脑组织并逃避完全手术切除。这种侵袭性导致对传统疗法的耐药性和高复发率。迫切需要能够消除残留肿瘤细胞的策略。使用5-氨基酮戊酸(5-ALA)的光动力疗法(PDT)是一种经美国食品药品监督管理局(FDA)和欧洲药品管理局(EMA)批准的化合物,可诱导光敏剂原卟啉IX(PpIX)在代谢活跃的肿瘤细胞中选择性积聚,通过光激活实现靶向细胞毒性。其临床应用的一个主要限制是,根据个体肿瘤差异,PDT的细胞毒性作用变化尚不清楚。在本研究中,我们提出并验证了一种源自患者的胶质母细胞瘤起始细胞(GICs)和脑类器官的体内模型,以测试PDT的效果。首先,通过流式细胞术和使用OncoScan CNV分析的拷贝数变异分析对源自患者的GICs进行分子表征,然后与人脑类器官共培养,以生成一个概括肿瘤微环境关键方面的混合模型。通过基于绿色荧光蛋白(GFP)的活力测量、乳酸脱氢酶(LDH)释放测定和末端脱氧核苷酸转移酶介导的缺口末端标记(TUNEL)染色,在体外评估5-氨基酮戊酸光动力疗法(PDT)的疗效。然后,基于GICs与人脑类器官共培养的异位(肾被膜下植入)异种移植,建立了一个小鼠模型来研究体内PDT。在该模型中测试了PDT;在每个实验对象中,对一个肾脏肿瘤植入物进行治疗,对侧作为对照。免疫荧光分析用于研究PDT后脑类器官-肿瘤植入物的细胞组成以及对非GIC细胞的影响。通过TUNEL技术进行细胞死亡分析的程度来确定抗肿瘤效果。GICs-脑类器官共培养在体外和体内均导致肿瘤生长和浸润。生长和浸润模式因肿瘤基因谱而异。5-ALA PDT导致体外测试的所有四条细胞系中GICs数量减少,凋亡细胞增加。发现体内诱导的光毒性与体外GICs细胞系的分子分型之间存在相关性。应用PDT后,神经元细胞的数量和分布没有变化,而活性星形胶质细胞减少。5-ALA PDT可能有效地根除具有异质分子谱的GICs。本文提出的人鼠混合模型可能有助于在个性化医疗的概念下研究胶质母细胞瘤的辅助治疗。