CAJ | 학술논문

目的探讨上皮样肉瘤临床及组织病理诊断及鉴别诊断的要点.方法回顾性分析中国医学科学院皮肤病医院2000-2014年诊治的9例上皮样肉瘤患者的临床表现、组织病理学、免疫组化、治疗方法及预后.结果 9例患者中位发病年龄34.5岁,就诊时平均病程8.3年.8例始发于肢端,表现为结节、溃疡及淋巴水肿.组织病理示肿瘤细胞主要由上皮样细胞和梭形细胞构成,可呈栅栏状排列,中央有坏死,肿瘤细胞共表达细胞角蛋白、上皮膜抗原、波形蛋白.6例患者皮损切除后原位复发,4例伴肺部转移.随访5例,其中2例死于肺转移.结论上皮样肉瘤切除后局部复发率高,容易发生淋巴结和肺部转移,发生肺部转移后预后不佳.
목적 탐토상피양육류림상급조직병리진단급감별진단적요점.방법 회고성분석중국의학과학원피부병의원2000-2014년진치적9례상피양육류환자적림상표현、조직병이학、면역조화、치료방법급예후.결과 9례환자중위발병년령34.5세,취진시평균병정8.3년.8례시발우지단,표현위결절、궤양급림파수종.조직병리시종류세포주요유상피양세포화사형세포구성,가정책란상배렬,중앙유배사,종류세포공표체세포각단백、상피막항원、파형단백.6례환자피손절제후원위복발,4례반폐부전이.수방5례,기중2례사우폐전이.결론 상피양육류절제후국부복발솔고,용역발생림파결화폐부전이,발생폐부전이후예후불가.
Objective To better characterize the clinical and histopathological manifestations, diagnosis and differential diagnosis of epithelioid sarcoma (ES).Methods Clinical data were collected from nine patients with ES diagnosed and treated at the Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College from 2000 to 2014.The clinical and histopathological manifestations, immunophenotype, treatment and prognosis of ES were reviewed retrospectively.Results The median age at onset of ES was 34.5 years in these patients, and the average disease duration was 8.3 years.Lesions began on the extremities in 8 patients, and manifested as nodules,ulceration and lymphedema.Histopathological examination showed that tumor cells mainly consisted of spindle cells and epithelioid cells, some of which were in a palisade arrangement with central necrosis.Cytokeratin, epithelial membrane antigen (EMA) and vimentin were coexpressed by tumor cells.Recurrence was observed at the original site in 6 patients after lesion resection, and pulmonary metastasis occurred in 4 patients.Five patients were followed up and two of them died of pulmonary metastasis.Conclusions Local recurrence is frequent in patients with ES after lesion removal.Lymph node and pulmonary metastases of ES are common, and pulmonary metastasis is usually associated with a poor prognosis.