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脊髓毛细胞型星形细胞瘤的临床特征及长期预后:日本神经脊髓学会的一项多中心回顾性研究

Clinical Characteristics and Long-term Outcomes of Spinal Pilocytic Astrocytomas: A Multicenter Retrospective Study by the Neurospinal Society of Japan.

作者信息

Muto Jun, Murata Hidetoshi, Shigekawa Seiji, Mitsuhara Takafumi, Umebayashi Daisuke, Kanematsu Ryo, Joko Masahiro, Inoue Tatsushi, Inoue Tomoo, Endo Toshiki, Hirose Yuichi

机构信息

Department of Neurosurgery, Fujita Health University, Aichi, Japan.

Department of Neurosurgery, St. Marianna University School of Medicine, Yokohama, Japan.

出版信息

Neurospine. 2023 Sep;20(3):774-782. doi: 10.14245/ns.2346450.225. Epub 2023 Sep 30.

DOI:10.14245/ns.2346450.225
PMID:37798969
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10562249/
Abstract

OBJECTIVE

The characteristics, imaging features, long-term surgical outcomes, and recurrence rates of primary spinal pilocytic astrocytomas (PAs) have not been clarified owing to their rarity and limited reports. Thus, this study aimed to analyze the clinical presentation, radiological features, pathological findings, and long-term outcomes of spinal PAs.

METHODS

Eighteen patients with spinal PAs who were surgically treated between 2009 and 2020 at 58 institutions were included in this retrospective multicenter study. Patient data, including demographics, radiographic features, treatment modalities, and long-term outcomes, were evaluated.

RESULTS

Among the 18 consecutive patients identified, 11 were women and 7 were men; the mean age at presentation was 31 years (3-73 years). Most PAs were located eccentrically, were solid or heterogeneous in appearance (cystic and solid), and had unclear margins. Gross total resection (GTR), subtotal resection (STR), partial resection (PR), and biopsy were performed in 28%, 33%, 33%, and 5% of cases, respectively. During a follow-up period of 65 ± 49 months, 4 patients developed a recurrence; however, the recurrence-free survival did not differ significantly between the GTR and non-GTR (STR, PR, and biopsy) groups.

CONCLUSION

Primary spinal PAs are rare and present as eccentric and intermixed cystic and solid intramedullary cervical tumors. The imaging features of spinal PAs are nonspecific, and a definitive diagnosis requires pathological support. Surgical resection with prevention of neurological deterioration can serve as the first-line treatment; however, the resection rate does not affect recurrence-free survival. Investigation of relevant molecular biomarkers is required to elucidate the regrowth risk and prognostic factors.

摘要

目的

原发性脊髓毛细胞型星形细胞瘤(PAs)因其罕见性及有限的报道,其特征、影像学表现、长期手术结果和复发率尚未明确。因此,本研究旨在分析脊髓PAs的临床表现、放射学特征、病理结果及长期预后。

方法

本回顾性多中心研究纳入了2009年至2020年期间在58家机构接受手术治疗的18例脊髓PAs患者。评估患者数据,包括人口统计学、影像学特征、治疗方式和长期预后。

结果

在连续纳入的18例患者中,女性11例,男性7例;就诊时的平均年龄为31岁(3 - 73岁)。大多数PAs位于偏心位置,外观为实性或异质性(囊性和实性),边界不清。分别有28%、33%、33%和5%的病例进行了全切除(GTR)、次全切除(STR)、部分切除(PR)和活检。在65±49个月的随访期内,4例患者复发;然而,GTR组和非GTR组(STR、PR和活检)之间的无复发生存率无显著差异。

结论

原发性脊髓PAs罕见,表现为偏心性且混合囊性和实性的颈髓内肿瘤。脊髓PAs的影像学特征不具有特异性,明确诊断需要病理支持。预防神经功能恶化的手术切除可作为一线治疗;然而,切除率不影响无复发生存率。需要研究相关分子生物标志物以阐明再生长风险和预后因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cadf/10562249/514e5df61134/ns-2346450-225f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cadf/10562249/0622ba54692a/ns-2346450-225f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cadf/10562249/514e5df61134/ns-2346450-225f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cadf/10562249/0622ba54692a/ns-2346450-225f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cadf/10562249/514e5df61134/ns-2346450-225f2.jpg

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