摘要
目的 探讨硬化性上皮样纤维肉瘤（sclerosing epithelioid fibrosarcoma，SEF）的临床病理学特点、免疫表型和鉴别诊断。方法 对3例SEF进行细胞病理学、组织病理学及免疫表型观察。结果患者年龄60～64岁，其中女性2例，男性1例。临床表现为病程不等（2～15年）的软组织深部肿物，1例肿物明显增大，1例有疼痛。肿瘤分别位于大腿、前臂及臀部，累及骨骼肌，最大直径分别为3、9、10cm。肿瘤境界清楚，分叶状或多结节状，切面灰白色，质地坚硬。1例伴有钙化。细胞学印片显示肿瘤细胞散在，主要由小至中等大的上皮样细胞组成，圆形或卵圆形，有不等量的嗜酸性胞质，单个核，染色质稍深染，核仁不清，未见核分裂象及瘤巨细胞。冷冻切片检查：肿瘤细胞主要由小至中等大的上皮样细胞组成，多呈单个、单行或索状排列，夹杂分布于大量嗜伊红色、玻璃样变的胶原纤维中，类似于分化差的转移癌。石蜡切片显示在硬化的胶原性间质背景中上皮样肿瘤细胞呈单个、巢状、索状或腺泡状排列，形态相对均匀，细胞质稀少、透明或嗜酸，细胞核大小一致，椭圆形、圆形和多角形，染色质呈泡状或细点状，可见小嗜碱性核仁，异型性不明显，核分裂活性低（1～2／10HPF）。1例伴有局灶凝固性坏死。3例肿瘤的局部区域均有典型的纤维肉瘤形态。免疫表型：3例肿瘤均弥漫性表达vimentin，部分不同程度表达bcl-2（1／3）、p53（1／3）、EMA（1／3）、S-100蛋白（1／3）、CD34（2／3）。所有肿瘤均不表达细胞角蛋白、HMB-45、CIM5、CD68、desmin和CD99等。增值指数MIB．1为0～5％。3例术后随访6～30个月，无复发和转移。结论硬化性上皮样纤维肉瘤是一种罕见的特殊类型的纤维肉瘤，诊断需与一些具有上皮样细胞形态和硬化性间质的良恶性肿瘤鉴别。
Purpose To study the clinicopathological characteristics and differential diagnosis of sclerosing epithelioid fibrosarcoma （SEF）. Methods Three cases of SEF were analyzed by cytopathology, histopathology and immunohistochemistry. Results The age of the patients was 60-, 60- and 64-year-old, respectively. There were 2 females and 1 male. Patients presented as a deep-seate mass with variable duration（2～15 years）. In one case the mass had enlarged noticeably and another one had pain. The tumors aroused in the skeletal muscles of the thigh, forearm, and buttocks, respectively. The tumor size was 3 cm ,9 cm, and 10 cm in greatest diameter, respectively. Grossly, the tumors were well circumscribed, lobulated or muhinodular with a firm and whitish cut surface. One case presented calcification. Cytologically ,the touch preparations were characterized by dispersed epithelioid tumor cells of small to medium size with uniformly round to ovoid shapes and variable eosinophilic cytoplasm. There was one nucleus to each cell with slight hyperchromasia and inconspicuous nucleolus. Mitotic figures and tumor giant cells were absent. Histopathologically,the frozen sections showed the tumors composed predominantly of small to medium-sized cells, which arranged in single, single file or cords and embedded in an abundant hyalinized fibrous stroma. The paraffin sections showed SEFs consisted of relatively uniform epithelioid tumor cells arranged in single, nests ,cords or acini patterns within a sclerotic collagenous matrix. The cells had scanty cleared-out or eosinophilic cytoplasm, oval to round, angulated uniform nuclei with finely stippled or vesicular chromatin, and small basophilic nucleoli. The heteromorphism were insignificant and mitotic figure was generally inconspicuous（ 1～2/10 HPF）. One case had focal necrosis. Foci of conventional fibrosarcoma structure could be seen in all cases. Immunohistochemically, the tumors stained positively for vimentin （ 3/3 ） , bcl-2 （ 1/3 ） , p53 （ 1/ 3） ,EMA（1/3）,S-100 protein（1/3） and CD34（2/3）,while negatively for CK, HMB-45, CD45, CD68, desmin, and CD99. MIB-1 index was 0～5%. Follow-up in all cases ranged from 6 to 30 months and none of them had local recurrence or metastases. Conclusions SEF is a rare variant of fibrosarcoma. More attention should be paid in the daily practices in order to differentiate this tumor from a variety of benign and malignant tumors exhibiting both epithelioid feature and sclerotic stromal response.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2006年第4期407-411,416,共6页
Chinese Journal of Clinical and Experimental Pathology
关键词
软组织肿瘤
纤维肉瘤
免疫表型
诊断
鉴别
soft tissue neoplasms
fibrosarcoma
immunophenotypes
diagnosis, differential
作者简介
郑锦阳（1967-），男，进修医师。Tel：（0595）22277351，E-mail：zjy_cyl@163.com
