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脂肪母细胞瘤和脂肪母细胞瘤病:14例临床病理研究

Lipoblastoma and lipoblastomatosis: a clinicopathological study of 14 cases.

作者信息

Mentzel T, Calonje E, Fletcher C D

机构信息

Department of Histopathology, St Thomas's Hospital (U.M.D.S.), London, UK.

出版信息

Histopathology. 1993 Dec;23(6):527-33. doi: 10.1111/j.1365-2559.1993.tb01238.x.

Abstract

The clinicopathological features of 14 cases of lipoblastoma and lipoblastomatosis are presented. The age of the patients at presentation ranged from 5 days to 6 years (mean 2.7 years); nine patients were male. Histologically, six cases were circumscribed (lipoblastoma) while eight were diffuse and ill-defined (lipoblastomatosis). In both groups and in individual cases there was distinct lobulation, as well as a spectrum of adipocytic maturation. Cytologically, the 10 most mature lesions were composed of uniform adipocytes intermixed with only scattered lipoblasts and primitive mesenchymal cells. A notable feature in the other four cases was a prominent myxoid stroma producing a very close resemblance to myxoid liposarcoma. Mitotic figures were rare and always normal in appearance. Atypical nuclei were not evident. Follow-up in eight patients revealed local recurrence in two. Liposarcoma in patients under 10 years is exceedingly rare, and, in myxoid form, may be almost impossible to distinguish histologically from lipoblastoma. Helpful clues are the lack of lobulation, variable growth pattern and increased nuclear atypia in liposarcoma.

摘要

本文报告了14例脂肪母细胞瘤和脂肪母细胞瘤病的临床病理特征。患者就诊时的年龄为5天至6岁(平均2.7岁);9例为男性。组织学上,6例为局限性(脂肪母细胞瘤),8例为弥漫性且边界不清(脂肪母细胞瘤病)。在两组及个别病例中均有明显的分叶,以及一系列脂肪细胞成熟过程。细胞学上,10个最成熟的病变由均匀的脂肪细胞组成,仅散在分布有脂肪母细胞和原始间充质细胞。另外4例的一个显著特征是有突出的黏液样间质,与黏液样脂肪肉瘤极为相似。有丝分裂象罕见,且形态总是正常的。未见非典型核。8例患者随访发现2例局部复发。10岁以下患者的脂肪肉瘤极为罕见,黏液样型在组织学上几乎无法与脂肪母细胞瘤区分。有用的线索是脂肪肉瘤缺乏分叶、生长方式多样且核异型性增加。

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