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脊髓髓内脂肪瘤的发病机制:两例报告。

Pathogenesis of spinal intramedullary lipomas: two case reports.

机构信息

Neurosurgery Department, University Hospital "12 de Octubre", Avda de Córdoba, s/n, 28041, Madrid, Spain.

出版信息

J Med Case Rep. 2023 Jul 25;17(1):317. doi: 10.1186/s13256-023-04048-z.

Abstract

BACKGROUND

Spinal lipomas not associated with dysraphism are rare and have an unknown natural history. In this report, we describe two cases; they showed recurrence during long-term follow-up, which makes us doubt a benign malformative etiology.

CASE REPORTS

Two patients, a 19-year-old South American woman and a 14-year-old boy with spinal lipomas, underwent surgical resection. The lipomas were not associated with dysraphism and were located in the cervicothoracic and craniocervical junctions. In both cases, we decided to operate due to clinical progression; the former had a progressive natural course, and the latter experienced clinical worsening after recurrence from previous surgeries. The surgery took place with the assistance of neurophysiological monitoring and intraoperative ultrasound; a partial resection and medullary decompression were done, following the more recent recommendations.

DISCUSSION

The natural history of these lesions is currently unknown due to their rarity and the heterogeneity in the long-term follow-up of previously reported cases. Although previous reports describe good outcomes after surgical resection, long follow-ups, especially in young subjects, may show differences in these outcomes with progression and recurrence. We contribute to this last piece of evidence by describing two more cases of progression and recurrence.

LESSONS

Long-term close follow-up should be done in young subjects with spinal lipomas, as they are more prone to an aggressive course. Metabolism and hormonal changes may be behind this progression. Reoperation must be considered if neurological decline is detected.

摘要

背景

不伴有发育异常的脊髓脂肪瘤罕见,其自然病史尚不清楚。本研究报告了两例病例;它们在长期随访中复发,这使我们怀疑其为良性畸形性病因。

病例报告

两名患者,一名 19 岁的南美女性和一名 14 岁的男孩,患有脊髓脂肪瘤,接受了手术切除。脂肪瘤不伴有发育异常,位于颈胸和颅颈交界处。由于临床进展,我们决定对这两例病例进行手术;前者表现为进行性自然病程,后者在经历了先前手术的复发后出现临床恶化。手术在神经生理监测和术中超声的协助下进行;根据最近的建议进行了部分切除和脊髓减压。

讨论

由于这些病变罕见,且既往报道的病例长期随访存在异质性,因此目前尚不清楚这些病变的自然病史。尽管之前的报道描述了手术切除后的良好结果,但长期随访,尤其是在年轻患者中,可能会显示出不同的结果,出现进展和复发。我们通过描述另外两例进展和复发的病例对此提供了更多证据。

经验教训

对于患有脊髓脂肪瘤的年轻患者,应进行长期密切随访,因为他们更容易出现侵袭性病程。代谢和激素变化可能是导致这种进展的原因。如果发现神经功能下降,必须考虑再次手术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/93d1/10367250/6752ee9a2fb3/13256_2023_4048_Fig1_HTML.jpg

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