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黏液乳头型室管膜瘤的原发性播散:成人中的一种不同疾病?病例报告及文献综述

Primary Seeding of Myxopapillary Ependymoma: Different Disease in Adult Population? Case Report and Review of Literature.

作者信息

Khan Nickalus R, VanLandingham Matthew, O'Brien Thomas, Boop Frederick A, Arnautović Kenan

机构信息

Department of Neurosurgery, University of Tennessee Health Science Center, Memphis, Tennessee, USA.

Memphis Pathology Group, Memphis, Tennessee, USA.

出版信息

World Neurosurg. 2017 Mar;99:812.e21-812.e26. doi: 10.1016/j.wneu.2016.12.022. Epub 2016 Dec 29.

Abstract

Myxopapillary ependymoma (MPE) is a slow-growing tumor, occurring most often in adults. It originates from the filum terminale in the area of the conus medullaris and cauda equina and is considered a benign lesion. Despite this classification, however, recurrence after both partial and gross total resection is well known. In the pediatric population, primary MPE seeding and generally more aggressive clinical course is well documented and treated through gross total resection, if possible, followed by irradiation. In adults, however, primary MPE seeding is rarely seen. There are few prior reports describing primary metastases into multiple spinal locations in an adult before resection of an MPE. The reason for this difference among pediatric and adult MPE remains unclear. We present the case of a 32-year-old man with primary seeding of an MPE into multiple lumbosacral areas. The patient underwent gross total resection of the lesions and had an uneventful postoperative course. Primary seeding could be a sign of aggressive behavior in this tumor. Complete craniospinal magnetic resonance imaging studies should be done before and after surgery in patients who present with a multifocal primary MPE. Furthermore, patients with a history of primary tumor seeding of MPE should be thoroughly evaluated radiologically. Unlike in pediatric populations, the need for postoperative irradiation in adults is less clear and further studies-particularly genetic ones-are warranted.

摘要

黏液乳头型室管膜瘤(MPE)是一种生长缓慢的肿瘤,最常发生于成年人。它起源于脊髓圆锥和马尾区域的终丝,被认为是一种良性病变。然而,尽管有这种分类,但部分切除和全切术后复发却是众所周知的。在儿童群体中,原发性MPE播散以及通常更具侵袭性的临床病程已有充分记录,若可能,通过全切术治疗,随后进行放疗。然而,在成年人中,原发性MPE播散很少见。很少有先前的报告描述在切除MPE之前成年人原发性转移至多个脊柱部位的情况。儿童和成人MPE之间这种差异的原因尚不清楚。我们报告一例32岁男性患者,其MPE原发性播散至多个腰骶部区域。该患者接受了病变全切术,术后过程顺利。原发性播散可能是该肿瘤侵袭性行为的一个迹象。对于出现多灶性原发性MPE的患者,术前和术后都应进行完整的全脊柱磁共振成像检查。此外,有MPE原发性肿瘤播散病史的患者应进行全面的放射学评估。与儿童群体不同,成人术后放疗的必要性不太明确,需要进一步研究——尤其是遗传学研究。

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