摘要
目的:探讨研究小肠黏液样脂肪肉瘤的临床病理特征.方法:结合文献对1例小肠黏液样脂肪肉瘤进行形态学观察并分析其鉴别诊断及预后.结果:女,60岁,以急性肠梗阻收住.CT扫描见回肠末端黏膜下实性低密度肿块.大体检查:肌壁间灰黄色圆形肿物大小约5.0cm,突向黏膜导致急性肠套叠,肿物边界清楚但部分肠黏膜显著糜烂.形态学显示肿瘤由大量的黏液样间质和成熟性脂肪细胞样细胞组成,其间可见丰富的分支状薄壁血管及纤细的纤维间隔支撑.黏液样间质呈粉色或云雾状,宛如"肺水肿"样,少许多泡性脂肪母细胞散布其中.免疫组织化学检查:肿瘤细胞S-100呈阳性表达而CD34及CD117均为阴性.局部手术切除后随访10mo,未见肿瘤复发或转移.结论:小肠黏液样脂肪肉瘤为罕见的恶性肿瘤,分化程度高,如处置得当,预后良好.充分认识其组织病理学特点有助于确诊.
AIM： To investigate the clinicopathologic characteristics of primary myxoid liposarcoma （MLS） of the small intestine. METHODS： One case of MLS of the small intestine was reported, and its morphological features, differential diagnosis and prognosis were analyzed by reviewing the relevant literature. RESULTS： A 60-year-old female was admitted for acute bowel obstruction. A CT scan revealed a solitary, hypodense submucosal mass in the terminal ileum. Grossly, the tumor was round in shape, pale-yellow in color, and 5 cm large, with a well-defined margin but partly with severe mucosal erosion. The mass was located in the intramural portion, protruded into the mucosa and caused acute ileal intussusception. Histologically, the tumor was composed predominantly of myxoid stroma and of small amounts of mature lipocyte-like cells that were supported by rich thin arborizing vasculature and delicate fibrous septa. Some multi vacuolated lipoblasts were scattered in pink or cloudy myxoid matrix that showed a ＂pulmonary edema-like＂ pattern. Immunohistochemically, the neoplastic cells were positive for S-100, but negative for CD117 and CD34. The mass was resected, and no recurrence or metastasis was found 14 mo after local excision. CONCLUSION： Primary MLS of the small intestine is a rare, well-differentiated neoplasm that has a good prognosis if managed properly. Full recognition of its histopathologic features is helpful to the diagnosis of this rare condition.
出处
《世界华人消化杂志》
CAS
北大核心
2013年第10期931-935,共5页
World Chinese Journal of Digestology
关键词
小肠肿瘤
黏液样脂肪肉瘤
临床病理
免疫组织化学
鉴别诊断
Small intestine neoplasms
Myxoid liposarcoma
Clinicopathology
Immunohistochemistry
Differential diagnosis
