促结缔组织增生性小圆细胞肿瘤病理学及超微结构

J4 ›› 2010, Vol. 36 ›› Issue (4): 735-740.

促结缔组织增生性小圆细胞肿瘤病理学及超微结构

徐斌¹, 王波^2,3, 顾俊莲², 李馨², 李扬²

1. 福建省福州市第一医院病理科|福建 |福州 |350009|2. 吉林大学基础医学院病理生理学教研室吉林大学前列腺疾病防治研究中心|吉林长春 130021； 3. 内蒙古林业总医院病理科| 内蒙古牙克石 022150

收稿日期:2010-02-09 出版日期:2010-07-28 发布日期:2010-07-28
通讯作者: 李扬 (Tel: 0431-85619485,E-mail: lyang@jlu.edu.cn) E-mail:lyang@jlu.edu.cn
作者简介:徐斌（1972-）|男|福建省福州市人|主治医师,医学硕士|主要从事肿瘤病理的研究。
基金资助:
教育部博士点基金资助课题（20070183012）

Ultrastructure and pathology of desmoplastic small round cell tumor

XU Bin¹, WANG BO^2,3, GU Jun-Lian², LI Xin², LI Yang²

1. |Department of Pathology,First Hospital of Fuzhou,Fuzhou 350009,China；2. Department of Pathophysiology,School of Basic Medical Sciences,Prostate Diseases Prevention and TreatmentResearch Center,Jilin University,Changchun 130021,China；3. Department of Pathology,Forestry General Hospital of Inner Mongolia,Yakeshi 022150,China

Received:2010-02-09 Online:2010-07-28 Published:2010-07-28

摘要/Abstract

摘要：

目的:观察促结缔组织增生性小圆细胞肿瘤的病理学和超微结构改变，提高对促结缔组织增生性小圆细胞肿瘤的认识和诊断水平。方法: 对1例原发于右小腿的促结缔组织增生性小圆细胞肿瘤进行光镜和电镜观察及免疫组织化学染色分析,并结合文献复习。结果：肿瘤大体表现为大小为3.2 cm×2.4 cm×1.3 cm，切面灰黄色，见出血、坏死；光镜下表现为瘤细胞呈大小不等团巢状结构，巢之间有十分丰富的硬化性纤维结缔组织间质，并见坏死，巢团内瘤细胞排列成条索状，瘤细胞大小与形态较一致，呈小圆形或短梭形，核小、深染、核仁不明显；免疫组织化学显示为瘤细胞表达EMA(+)、CK(+)、NSE(+)及Desmin核旁点状染色(+)，而不表达CgA、Myogenin、Syn、LCA、SMA、S-100、NF、GFAP、HMB45、HHF-35、CD3、CD10、Actin、CD99及CD20；电镜特征性结构为瘤细胞胞浆内核旁区有中间丝聚集物，呈小球形或螺纹状排列。结论: 促结缔组织增生性小圆细胞肿瘤发生部位可在腹腔也可在腹腔外，发病年龄跨度较大。促结缔组织增生性小圆细胞肿瘤具有特殊的组织学和超微结构特征，免疫组织化学显示瘤细胞具有上皮源性、间质性和神经源性等多向分化的特点，并且RT-PCR在促结缔组织增生性小圆细胞肿瘤分子遗传学的诊断中有重要价值。促结缔组织增生性小圆细胞肿瘤预后差。

关键词: 软组织肿瘤；促结缔组织增生性小圆细胞肿瘤；光学显微镜；免疫组织化学；电子显微镜；超微结构

Abstract:

Abstract:Objective To observe the change of ultrastructure and pathology of desmoplastic small round cell tumor (DSRCT) and recognize the characteristics of DSRCT and improve the standard of diagnosis.Methods One case of primary DSRCT in right leg was observed by light microscope,immunohistochemical method and electron microscope and analyzed with review of the literatures.Results The size of tumor was 3.2 cm×2.4 cm×1.3 cm with gray-yellow on cross-section.Foci of hemorrhage and necrosis were noted.Under light microscope,the tumor was composed of sharply demarcated nests of small rounded or oval cells.The cellular aggregates were surrounded and separated by abundant fibrous connective tissue.The tumor cells were uniform in size and shape,and showed small to moderate amounts of pale cytoplasm with indistinct cell borders.The nuclei were round to oval,with clumped chromatin and marked hyperchromasia.Some cells had one or two indistinct nucleoli.Numerous mitotic figures and areas of necrosis were dentified.The immunohistochemical results showed that the tumor cells were strongly positive for CK,EMA and NSE.There was focal positive staining for desmin with a perinuclear dot-like pattern.However,the tumor cells were negative for CgA,Myogenin,Syn,LCA,SMA,S-100,NF,GFAP,HMB45,HHF-35,CD3,CD10,Actin,CD99, and CD20.Under electron microscope,the tumor cells showed paranuclear cytoplasmic intermediate filaments arranging in globular or whorl array.Conclusion DSRCT occurs both in the abdomen and at other sites.The patients with DSRCT range widely in age.DSRCT has distinctive histopathologic and ultrastructural features.This tumor shows immunohistochemical feature of epithelial,mesenchymal as well as neural multidirectional differentiation.RT-PCR may be served as an important diagnostic adjunct for DSRAT.The prognosis of the patients with DSRCT is very poor.

Key words: soft tissue neoplasms, desmoplastic small round cell tumor, light microscope, immunohistochemistry；electron microscope, ultrastructure

中图分类号:

R730.26

徐斌, 王波, 顾俊莲, 李馨, 李扬. 促结缔组织增生性小圆细胞肿瘤病理学及超微结构[J]. J4, 2010, 36(4): 735-740.

XU Bin, WANG BO, GU Jun-Lian, LI Xin, LI Yang. Ultrastructure and pathology of desmoplastic small round cell tumor[J]. J4, 2010, 36(4): 735-740.