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儿童脂肪母细胞瘤和脂肪肉瘤:9例分析及文献复习

Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature.

作者信息

Miller G G, Yanchar N L, Magee J F, Blair G K

机构信息

Department of Surgery, University of British Columbia, British Columbia's Children's Hospital, Vancouver. miller@duke,usask.ca

出版信息

Can J Surg. 1998 Dec;41(6):455-8.

Abstract

OBJECTIVES

To review the experience at a children's hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other.

DESIGN

A retrospective case series.

SETTING

British Columbia's Children's Hospital a tertiary-care pediatric centre.

PATIENTS

All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years.

MAIN OUTCOME MEASURES

The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma.

RESULTS

One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?),+mar.

CONCLUSIONS

Lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.

摘要

目的

回顾一家儿童医院治疗脂肪母细胞瘤和脂肪肉瘤的经验,并确定能够区分这两种肿瘤类型的任何因素。

设计

一项回顾性病例系列研究。

地点

不列颠哥伦比亚省儿童医院,一家三级护理儿科中心。

患者

12年间所有经病理诊断为脂肪母细胞瘤和脂肪肉瘤的患者。

主要观察指标

从儿科脂肪肿瘤活检标本中确定的脂肪母细胞瘤和脂肪肉瘤的发生率。脂肪母细胞瘤和脂肪肉瘤之间的临床、病理和细胞遗传学变量。

结果

记录了149例脂肪肿瘤。其中7例(4.7%)为脂肪母细胞瘤,2例(1.3%)为脂肪肉瘤。所有肿瘤均表现为无症状、生长缓慢的软组织肿块。脂肪母细胞瘤患儿往往年龄较小,但29%超过3岁。脂肪肉瘤患者年龄分别为9岁和14岁。1例脂肪肉瘤为黏液样型,另1例为圆形细胞型。报告了1例脂肪母细胞瘤和1例脂肪肉瘤的核型。黏液样脂肪肉瘤的核型为46,XY,t(12;16)(q13;p11),脂肪母细胞瘤报告为46,XY,der(8)?t(8q;?),+mar。

结论

脂肪母细胞瘤是一种不常见的儿童肿瘤,脂肪肉瘤在儿童中非常罕见。两种肿瘤可能以相似的方式出现,在组织学上区分它们可能很困难。不能依靠年龄来准确预测它们的行为。肿瘤核型对区分这些肿瘤非常有帮助。

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