Gupta Rohan V, Welch Jonathan C, Kaul Anand, Prayson Richard A, Rong Yuan, Almefty Rami O
Departments of Neurosurgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania.
Department of Pathology, Cleveland Clinic Foundation, Cleveland, Ohio.
J Neurosurg Case Lessons. 2024 Aug 12;8(7). doi: 10.3171/CASE24241.
Juvenile pilocytic astrocytoma (JPA) is the most common primary brain tumor of childhood and is rarely seen in adults. Neurofibromatosis type 1 (NF1), a common tumor predisposition syndrome, demonstrates a strong association with low-grade gliomas, most notably pilocytic astrocytoma, which are relatively indolent. Unlike its juvenile counterpart, reports of adult pilocytic astrocytoma (APA) vary widely in terms of disease progression from benign to much more malignant courses. Moreover, current studies discussing APA report different treatment approaches and outcomes (e.g., malignant transformation of JPA and APA with or without radiation), as little is known regarding the management of recurrent tumors and how adjuvant therapies may alter disease progression.
The authors report the unique case of an adult male with NF1 and APA who underwent rapid malignant conversion after intensity-modulated radiation therapy.
The authors demonstrate that caution should be taken in utilizing radiotherapy instead of resection in cases of APA and NF1, with close monitoring for posttreatment recurrence. https://thejns.org/doi/10.3171/CASE24241.
青少年毛细胞型星形细胞瘤（JPA）是儿童期最常见的原发性脑肿瘤，在成人中罕见。1型神经纤维瘤病（NF1）是一种常见的肿瘤易感综合征，与低级别胶质瘤密切相关，最显著的是相对惰性的毛细胞型星形细胞瘤。与青少年毛细胞型星形细胞瘤不同，关于成人毛细胞型星形细胞瘤（APA）的报道在疾病从良性到更恶性病程的进展方面差异很大。此外，目前讨论APA的研究报告了不同的治疗方法和结果（例如，JPA和APA伴或不伴放疗的恶性转化），因为对于复发性肿瘤的管理以及辅助治疗如何改变疾病进展知之甚少。
作者报告了1例患有NF1和APA的成年男性在调强放射治疗后迅速发生恶性转化的独特病例。
作者证明，对于APA和NF1病例，在使用放疗而非手术切除时应谨慎，并密切监测治疗后复发情况。https://thejns.org/doi/10.3171/CASE24241 。
