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脊柱副神经节瘤:一个大容量肿瘤中心的二十年临床经验。

Paraganglioma of the spine: A twenty-years clinical experience of a high volume tumor center.

机构信息

Department of Oncologic and Degenerative Spine Surgery - IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy.

GSpine4 Spine Unit - IRCCS Galeazzi Orthopedic Institute, Milano, Italy.

出版信息

J Clin Neurosci. 2019 Aug;66:7-11. doi: 10.1016/j.jocn.2019.05.037. Epub 2019 May 29.

DOI:10.1016/j.jocn.2019.05.037
PMID:31153748
Abstract

Paragangliomas (PGs) are rare tumours with a reported estimated annual incidence of up to 3 per million. Spinal involvement may possible with spinal metastasis and primary extradural localizations. The aim of this paper is to evaluate clinical outcomes of surgical treatment of a rare disease that can involve the spine and that should be considered in the differential diagnosis of spinal injuries. This is a retrospective observational study of the spinal paragangliomas treated at our institute. Five patients have been enrolled: three with metastatic PG and two with extradural PG. Metastatic PGs were treated with intralesional excision plus adjuvant therapies instead, extradural PGs with intralesional excision without adjuvant therapies. Among patients affected by metastastic paraganglioma two patients were Alive with disease (AWD) at the latest follow and one patient died for the spreading of disease at 240 months after surgery. Two patients with extradural paraganglioma of thoracic spine were AWD at the latest follow-up without pain and neurological deficits. Surgical management of spinal localizations can represent a challenge. Surgery has a main role in both diseases where intralesional excision plus adjuvant therapies seems to be able to achieve the local control and a satisfying prognosis in case of undisseminated tumour.

摘要

副神经节瘤(PGs)是一种罕见的肿瘤,据报道其年发病率高达每百万分之三。脊柱转移和原发性硬脊膜外局部病变可能涉及脊柱。本文旨在评估手术治疗一种可能涉及脊柱的罕见疾病的临床结果,这种疾病应在脊柱损伤的鉴别诊断中考虑。这是对我院治疗的脊柱副神经节瘤进行的回顾性观察性研究。共纳入 5 名患者:3 例转移性 PG 和 2 例硬脊膜外 PG。转移性 PG 采用病灶内切除术加辅助治疗,硬脊膜外 PG 采用病灶内切除术而不采用辅助治疗。在转移性副神经节瘤患者中,2 例患者在最新随访时仍患有疾病(AWD),1 例患者在术后 240 个月因疾病扩散而死亡。2 例胸段硬脊膜外副神经节瘤患者在最新随访时无疼痛和神经功能缺损,仍存活。脊柱病变的手术治疗可能是一个挑战。手术在这两种疾病中都起着主要作用,病灶内切除术加辅助治疗似乎能够在肿瘤未播散的情况下实现局部控制和满意的预后。

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