原发性肾上腺上皮样血管肉瘤伴肝肺转移1例

Primary Epithelioid Angiosarcoma of the Adrenal Gland with Liver and Lung Metastases: A Case Report

摘要: 原发性肾上腺上皮样血管肉瘤是一类极为罕见、具有高度侵袭性的血管内皮源性恶性肿瘤，国内外文献报道病例数极少，且易发生远处转移。该肿瘤多表现为肾上腺无功能性占位，临床症状缺乏特异性，影像学亦无典型特征，极易与其他肾上腺恶性肿瘤或转移性病灶相混淆，给术前诊断带来极大挑战。本文报道1例44岁男性原发性肾上腺上皮样血管肉瘤伴肝肺转移病例，该患者因发现左侧肾上腺肿物入院，仅伴咳嗽、咳痰症状，体格及实验室检查无特殊，腹部MR及胸部CT提示肾上腺巨大占位并出血、坏死，肝肺多发异常结节，术后经病理组织学及免疫组化检测确诊，肿瘤组织CD31、ERG、Fli-1呈阳性表达，AE1/AE3呈部分阳性表达。此类罕见肿瘤的临床报道，对提升临床医师对其的认知、提高术前诊断准确率及制定个体化诊疗策略具有重要的参考价值。

Abstract: Primary epithelioid angiosarcoma of the adrenal gland is an extremely rare, highly aggressive malignant tumor of vascular endothelial origin, with only a handful of cases reported in the domestic and international literature and a high tendency for distant metastasis. This tumor mostly presents as a non-functional adrenal space-occupying lesion with non-specific clinical manifestations and no typical imaging features, which is easily confused with other malignant adrenal tumors or metastatic lesions, posing a great challenge to preoperative diagnosis. Herein, we report a case of primary epithelioid angiosarcoma of the left adrenal gland with liver and lung metastases in a 44-year-old male patient. The patient was admitted to hospital due to the detection of a left adrenal mass, presenting only with cough and expectoration, and no abnormalities were found in physical and laboratory examinations. Abdominal magnetic resonance imaging (MRI) and chest computed tomography (CT) revealed a large adrenal mass complicated with hemorrhage and necrosis, as well as multiple abnormal nodules in the liver and lungs. The diagnosis was confirmed by postoperative pathological histology and immunohistochemical examination, which showed positive expression of CD31, ERG and Fli-1, and focal positive expression of AE1/AE3 in the tumor tissue. Clinical reports of such rare tumors are of great reference value for improving clinicians’ understanding of the disease, raising the accuracy of preoperative diagnosis and formulating individualized diagnosis and treatment strategies.