Han J, Wei J G, Gao X Z, Xu Y, Zhang L, Xie Y L, Liu Y Q, Fan X Y, Li W C, Li S L
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Department of Pathology, Shaoxing People's Hospital of Zhejiang Province, Shaoxing 312000, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Jun 8;49(6):550-555. doi: 10.3760/cma.j.cn112151-20200225-00131.
To investigate the clinicopathological features of pulmonary epithelioid hemangioendothelioma (PEHE). Eighteen cases of PEHE were collected from August 2011 to December 2018 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin and eosin staining and immunohistochemistry (IHC). The clinicopathological features were reviewed; the status of CAMTA1 and TFE3 gene was analyzed and patients' outcome was followed up. Of the 18 cases, there were 11 males and 7 females with a male to female ratio of 1.6 to 1.0. The patients' age ranged from 36 to 68 years (mean 52 years). Twelve cases (12/18) showed a single nodule and six cases (6/18) showed multiple bilateral nodules. Seven cases (7/18) involved other organs besides lung. Seventeen (17/18) patients presented with respiratory symptoms and one patient (1/18) presented with abdominal pain. Grossly, the tumors were greyish-white nodules with indistinct borders. Microscopically the tumor cells were epithelioid and arranged in strands and nests, and cytoplasmic vacuoles were commonly noted. The stroma was myxochondroid or hyaline. By IHC, the tumor cells were positive for CD31(18/18), CD34 (16/18), ERG (18/18) and Fli-1 (18/18); CKpan was focally positive in 5 cases (5/18). TFE3 was positive in 3 cases (3/18), and Ki-67 index ranged from 5% to 30%. FISH analysis showed seventeen cases (17/18) had CAMAT1 rearrangement, one case had TFE3 rearrangement displaying a split signal. Eight patients (8/18) had surgical excision, three patients (3/18) had surgery and chemotherapy, and seven patients (7/18) had chemotherapy only. Four patients (4/18) died of the disease. Patients with PEHE have non-specific symptoms, and correct diagnosis depends on pathologic biopsy and the exclusion of other tumors with epithelioid morphology. Some patients with PEHE have poor prognosis, particularly in those who have multiple nodules, peripheral invasion or metastasis.
探讨肺上皮样血管内皮瘤（PEHE）的临床病理特征。收集2011年8月至2018年12月在郑州大学第一附属医院的18例PEHE病例。所有病例均采用苏木精-伊红染色和免疫组织化学（IHC）进行回顾性研究。回顾临床病理特征；分析CAMTA1和TFE3基因状态并对患者预后进行随访。18例中，男性11例，女性7例，男女比例为1.6∶1.0。患者年龄36～68岁（平均52岁）。12例（12/18）表现为单个结节，6例（6/18）表现为双侧多发结节。7例（7/18）除肺外还累及其他器官。17例（17/18）患者有呼吸道症状，1例（1/18）患者有腹痛。大体上，肿瘤为灰白色结节，边界不清。显微镜下，肿瘤细胞呈上皮样，呈条索状和巢状排列，常见胞质空泡。间质为黏液软骨样或透明样。免疫组化显示，肿瘤细胞CD31阳性（18/18）、CD34阳性（16/18）、ERG阳性（18/18）、Fli-1阳性（18/18）；CKpan在5例（5/18）中局灶阳性。TFE3在3例（3/18）中阳性，Ki-67指数为5%～30%。荧光原位杂交分析显示17例（17/18）有CAMAT1重排，1例有TFE3重排，表现为分裂信号。8例（8/18）患者行手术切除，3例（3/18）患者行手术及化疗，7例（7/18）患者仅行化疗。4例（4/18）患者死于该病。PEHE患者症状不具特异性，正确诊断依赖病理活检并排除其他具有上皮样形态的肿瘤。部分PEHE患者预后较差，尤其是那些有多发结节、外周侵犯或转移的患者。
