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脊髓毛细胞型星形细胞瘤的临床特征及长期手术疗效:20例报告

Clinical characteristics and long-term surgical outcomes of spinal pilocytic astrocytoma: a report of twenty cases.

作者信息

Zhang Liang, Li Teng, Qiao Guangyu, Yang Wuyang, Shang Aijia, Yu Xinguang

机构信息

Medical School of Nankai University, No. 94, Weijin Road, Naikai District, Tianjin, 300071, People's Republic of China.

Department of Neurosurgery, The First Medical Center, Chinese PLA General Hospital, No. 28, Fuxing Road, Haidian District, Beijing, 100853, People's Republic of China.

出版信息

Acta Neurochir (Wien). 2021 Nov;163(11):3005-3013. doi: 10.1007/s00701-020-04606-0. Epub 2020 Oct 10.

Abstract

BACKGROUND

Primary spinal pilocytic astrocytoma (PA) is an extremely rare low-grade astrocytoma with unclear natural history. The demographic characteristics, imaging features, and long-term surgical outcomes have not been clarified due to low prevalence and limited reports.

METHODS

A retrospective review within a single institution between 2004 and 2018 of all patients with pathologically proven PA was conducted. Patient data including demographics, radiographic features, treatment modalities, and long-term outcomes were evaluated.

RESULTS

Twenty consecutive patients were identified, and 16 (80%) were male patients, with a mean age at presentation of 29 ± 13 years. The lesion was primarily located in cervical (n = 10, 50%), thoracic (n = 7, 35%), cervico-thoracic junction (n = 2), and lumbar level (n = 1, 5%). The tumor had a mean extension of 4 ± 2 (1-7) vertebral segments. Most PAs were located eccentrically (n = 16, 80%), with most being heterogeneous in appearance (cystic and solid) or purely cystic (n = 14, 70%), and had unclear margins (n = 16, 80%). Eleven patients (55%) had associated syringomyelia. Gross total resection (GTR) was achieved in 11 (55%) patients, and subtotal resection (STR) in 9 (45%). During a mean follow-up of 104 ± 56 months, 2 patients died and recurrence was found in 4 patients (20%), translating to a mean progression-free survival of 21 ± 11 months.

CONCLUSION

Primary spinal PA is a rare entity with acceptable progression-free survival if treated appropriately. Surgical resection may provide reasonable prolongation of survival, and GTR should be achieved if possible. A close follow-up is recommended especially for residual lesions, and a further in-depth investigation of molecular biomarkers is needed to stratify risk and prognostic factors.

摘要

背景

原发性脊髓毛细胞型星形细胞瘤(PA)是一种极为罕见的低级别星形细胞瘤,其自然病史尚不明确。由于发病率低且报告有限,其人口统计学特征、影像学特征和长期手术结果尚未阐明。

方法

对2004年至2018年间在单一机构内所有经病理证实为PA的患者进行回顾性研究。评估患者数据,包括人口统计学、影像学特征、治疗方式和长期结果。

结果

共确定了20例连续患者,其中16例(80%)为男性患者,就诊时的平均年龄为29±13岁。病变主要位于颈椎(n = 10,50%)、胸椎(n = 7,35%)、颈胸交界处(n = 2)和腰椎水平(n = 1,5%)。肿瘤平均累及4±2(1 - 7)个椎体节段。大多数PA位于偏心位置(n = 16,80%),大多数外观为异质性(囊性和实性)或纯囊性(n = 14,70%),边界不清(n = 16,80%)。11例患者(55%)伴有脊髓空洞症。11例(55%)患者实现了全切除(GTR),9例(45%)患者实现了次全切除(STR)。在平均104±56个月的随访期间,2例患者死亡,4例患者(20%)复发,无进展生存期平均为21±11个月。

结论

原发性脊髓PA是一种罕见疾病,若治疗得当,无进展生存期可接受。手术切除可能会合理延长生存期,若可能应实现GTR。建议对残留病变进行密切随访,并且需要对分子生物标志物进行进一步深入研究以分层风险和预后因素。

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