导致四肢瘫痪的颈部脂肪母细胞瘤病：化疗辅助手术治疗及10年随访病例报告

Cervical lipoblastomatosis producing quadriparesis: case report of surgery with chemotherapy and 10-year follow-up.

作者信息

O'Brien Donncha, Aquilina Kristian, Farrell Michael, Breathnach Fin, Allcutt Daivd

机构信息

Department of Neurosurgery, Royal Liverpool Children's Hospital NHS Trust, Alder Hey, Eaton Road, Liverpool, L12 2AP, UK.

出版信息

Childs Nerv Syst. 2005 Feb;21(2):165-70. doi: 10.1007/s00381-004-0949-8. Epub 2004 Apr 7.

DOI:10.1007/s00381-004-0949-8

PMID:15071750

Abstract

INTRODUCTION

Cervical lipoblastomatosis is a rare spinal tumour. Management of recurrence and long-term outcome data are not well described.

CASE REPORT

A 10-month-old infant presented with an upper extremity weakness. Magnetic resonance imaging (MRI) revealed an extradural cervical spinal tumour. It was debulked and histopathology revealed it to be lipoblastomatosis. The infant improved postoperatively. However, 5 months later the patient deteriorated and developed quadriparesis. The patient was managed with a more extensive resection and had chemotherapy. Ten years post-presentation the patient is well having made a full recovery and is living a normal life. Recent MRI shows minimal residual quiescent tumour.

CONCLUSION

The treatment of cervical lipoblastomatosis should involve the resection of as much tumour as possible at the first sitting as recurrence can be a problem. In cases of spinal recurrence we recommend aggressive decompression and adjuvant chemotherapy.

摘要

引言

颈椎脂肪母细胞瘤是一种罕见的脊柱肿瘤。关于其复发的处理及长期预后数据的描述并不充分。

病例报告

一名10个月大的婴儿出现上肢无力症状。磁共振成像（MRI）显示硬膜外颈椎肿瘤。进行了肿瘤减瘤手术，组织病理学显示为脂肪母细胞瘤。婴儿术后情况改善。然而，5个月后患者病情恶化并出现四肢瘫。患者接受了更广泛的切除手术并进行了化疗。发病10年后，患者已完全康复，生活正常。近期MRI显示残留的静止肿瘤极少。