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脊髓哑铃状脂肪母细胞瘤:基于病例的最新进展

Spinal dumbbell lipoblastoma: a case-based update.

作者信息

Peter Spazzapan, Matevž Srpčič, Borut Prestor

机构信息

Department of Neurosurgery, University Medical Centre Ljubljana, Zaloška 7, 1000, Ljubljana, Slovenia.

Department of Thoracic Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia.

出版信息

Childs Nerv Syst. 2016 Nov;32(11):2069-2073. doi: 10.1007/s00381-016-3184-1. Epub 2016 Jul 21.

Abstract

INTRODUCTION

Lipoblastomas are benign tumors that arise from the proliferation of fetal adipose tissue. They are frequently found in the extremities, in the head, and in the neck. Dumbbell conformation (which refers to epidural tumors with extraspinal extension) has been described in only seven lipoblastoma cases so far.

CASE REPORT

We describe an 11-month-old patient with a large mediastinal dumbbell lipoblastoma. The mass was excised, but it recurred 1 year later when the MRI showed also multiple pleural lipoblastomatous lesions, thus establishing the diagnosis of lipoblastomatosis. The tumor has been removed with a second surgical procedure.

CONCLUSION

Radical excision of lipoblastomas is the gold standard but is not always possible to achieve. Residual lesions can be managed conservatively, on the basis of the benign nature of the tumor and of its potential maturation to normal fat. Recurrence of lipoblastoma appears in 15 % of non-radical excisions but has not yet been described in the form of lipoblastomatosis. A second surgery is indicated if the tumor mass reaches large dimensions, if it increases in size, and if it threatens the functionality of vital organs such as the spinal cord.

摘要

引言

脂肪母细胞瘤是由胎儿脂肪组织增殖形成的良性肿瘤。它们常见于四肢、头部和颈部。迄今为止,哑铃状形态(指硬膜外肿瘤伴脊柱外延伸)仅在7例脂肪母细胞瘤病例中被描述过。

病例报告

我们报告了一名11个月大患有巨大纵隔哑铃状脂肪母细胞瘤的患者。肿瘤被切除,但1年后复发,此时MRI显示也有多个胸膜脂肪母细胞瘤样病变,从而确诊为脂肪母细胞瘤病。肿瘤已通过第二次手术切除。

结论

脂肪母细胞瘤的根治性切除是金标准,但并非总是能够实现。基于肿瘤的良性性质及其向正常脂肪成熟的可能性,残留病变可进行保守处理。脂肪母细胞瘤在15%的非根治性切除中会复发,但尚未有以脂肪母细胞瘤病形式出现的报道。如果肿瘤块体积增大、尺寸变大且威胁到如脊髓等重要器官的功能,则需进行二次手术。

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