树突状纤维黏液脂肪瘤10例临床病理分析

树突状纤维黏液脂肪瘤10例临床病理分析

乔海国1

，张

昶2，庄一林1

，王

坚

3

摘要：目的探讨树突状纤维黏液脂肪瘤（dendritic fibromyxolipoma ，DFML ）的临床病理学特征和鉴别诊断。方法回顾性分

析10例DFML 的临床资料、病理学形态和免疫表型，并复习相关文献。结果男性9例，女性1例，年龄39 67岁，平均54

岁。多发生于颈项部和肩背部，表现为皮下缓慢性生长的无痛性肿块，直径1 6cm ，平均3.6cm 。肿瘤周界清晰或有菲薄包膜，切面呈灰黄至灰白色，黏液样。镜下见肿瘤由增生的小梭形至星状纤维母细胞样细胞、多少不等的成熟脂肪细胞和黏液样基质组成，细胞之间可见绳索样或纤细的胶原纤维，肿瘤内偶可见丛状小血管，间质内含有少量散在的肥大细胞。免疫组化标记小梭形和星状纤维母细胞样细胞表达vimentin 、CD34和BCL-2，其中标记vimentin 和CD34可清晰显示树突状的胞质突起。结论

DFML 是一种好发于中老年男性的特殊亚型脂肪瘤，与梭形细胞脂肪瘤关系密切，可能属于梭形细胞脂肪瘤的黏

液样变型。其需与其他黏液样肿瘤相鉴别。

关键词：树突状纤维黏液脂肪瘤；免疫组织化学；鉴别诊断中图分类号：R 738．6

文献标志码：A

文章编号：1001－7399（2012）12－1332－04

Dendritic fibromyxolipoma ：a clinicopathologic analysis of 10cases

QIAO Hai-guo 1，ZHANG Chang 2，ZHUANG Yi-lin 1，WANG Jian 3（1Department of Pathology ，Lianshui People ’s Hospital ，Lianshui 223400，China ；

2

Department of Pathology ，Affiliated Huai ’an

First People ’s Hospital ，Nanjing Medical University ，Huai ’an 223001，China ；3

Department of Pathology ，Cancer Hospital ，Shang-

hai Medical College ，Fudan University ，Shanghai 200032，China ）Abstract ：Purpose

To investigate the clinicopathological characteristics of dendritic fibromyxolipoma （DFML ）and to discuss the dif-ferential diagnosis．Methods

The clinical ，pathological and immunophenotypic features in 10cases of DFML were analyzed．The lit-eratures were reviewed．Results

Nine patients were male and 1was female with age ranging from 39 67years （mean ，54years ）．

The tumor presented as a slowly growing subcutaneous nodule with a predilection for the posterior neck ，back and shoulder．They were well circumscribed or encapsulated ，measuring in size from 1to 6cm （mean ，3．6cm ）with yellowish to gray in color and myxoid ap-pearance on cut surface．Histologically ，the tumor was composed of small spindle to stellate fibroblasts intermixed with rope-like or slender collagen fibers and varying amount of mature lipocytes embedded in a myxoid stroma．A few scattered mast cells were seen．By immunohistochemistry ，tumor cells expressed vimentin ，CD34and BCL-2．In particular ，the vimentin and CD34expression accentua-ted the dendritic phenotype of the cyoplasmic processes．Conclusions

DFML is a special variant of lipoma with a predilection for mid-dle to old aged males．The overlapping features between DFML and spindle cell lipoma suggest that they lie within the same spectrum of a single entity．Awareness of this rare tumor type is helpful in the differential diagnosis from a variety of other myxoid tumors．Key words ：dendritic fibromyxolipoma ；immunohistochemistry ；differential diagnosis

收稿日期：2012－06－08

作者单位：1

江苏省涟水县人民医院病理科，涟水223400

2南京医科大学附属淮安市第一人民医院病理科，淮安223001

3

复旦大学附属肿瘤医院病理科，上海200032

作者简介：乔海国，男，副主任医师。Tel ：（0517）82288227，

E-mail ：qiaohaiguo@163．com 王

坚，男，教授，通讯作者。E-mail ：softtissuetumor@ya-hoo．com．cn

树突状纤维黏液脂肪瘤（dendritic fibromyxoli-poma ，DFML ）由Suster 等［1］于1998年首先报道，是脂肪瘤的一种特殊亚型，

由小梭形至星状的纤维母细胞样细胞、绳索样胶原纤维以及多少不等的成熟脂肪细胞混合组成，间质黏液样。DFML 非常少见，

迄今为止国外文献仅报道14例［1－3］

，国内较罕见

［4－6］

。因DFML 镜下形态较特殊，易误诊为黏液

瘤或黏液样脂肪肉瘤（myxoid liposarcoma ，MLS ）。本文现报道10例DFML ，并结合文献复习阐述其临

床病理学特点，以提高人们对该少见肿瘤的认识。1材料与方法

1．1

材料收集2000 2011年3家医院诊断的10

例DFML ，其中涟水县人民医院病理科诊断1例，南