Department of Gastrointestinal and Hepatobiliary surgery, Hanoi Medical University Hospital, Hanoi, Vietnam.
Department of Surgery, Hanoi Medical University, 1st Ton That Tung Street, Dong Da, Ha Noi, Hanoi, 11521, Vietnam.
J Cardiothorac Surg. 2023 Jul 4;18(1):215. doi: 10.1186/s13019-023-02317-y.
Inflammatory pseudotumor (IPT) of the esophagus is a very rare benign lesions which clinical presentation is not clear and difficult to make a definitive diagnosis preoperatively.
In this report, we presented a case of a 24-year-old female with signs of severe malnutrition state due to dysphagia increasing gradually and losing 10 kg in weight for 2 months. Comprehensive preoperative radiologic investigations were proceeded with a circumferential severe stricture caused smooth submucosal swelling in the esophagus under 23 cm from the upper dental arch and two times of negative biopsy. Due to the aggressive clinical symptoms and gross lesion characteristics, the patient underwent laparoscopic-thoracoscopic esophagectomy and reconstruction with a gastric tube. Histopathological examination showed that the squamous epithelium of the esophagus had a small, benign nucleus, the submucosal layer and the smooth muscle layer increased fibrous, with infiltrating many lymphocytes, plasma cells, and macrophages. Immunohistochemical staining was negative for CD68, CD34, Desmin and ALK markers, and there was an increase in the number of IgG4-positive plasma cells. The final diagnosis was an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor.
Inflammatory pseudotumor of the esophagus is an extremely rare benign lesion but could led to aggressive clinical presentation. The gold standard of diagnosis is histopathological examination of surgically removed specimens. Radical resection is still the most efficient treatment method.
食管炎性假瘤（IPT）是一种非常罕见的良性病变，其临床表现不明确，术前难以做出明确诊断。
本报告介绍了一例 24 岁女性患者，因进行性吞咽困难和 2 个月体重减轻 10kg 而出现严重营养不良状态。进行了全面的术前影像学检查，发现距上齿弓 23cm 以下食管环状严重狭窄，黏膜下呈光滑肿胀，两次活检均为阴性。由于患者的临床症状和大体病变特征具有侵袭性，因此进行了腹腔镜-胸腔镜食管切除术和胃管重建术。组织病理学检查显示食管鳞状上皮细胞核小，良性，黏膜下层和平滑肌层纤维增生，有大量淋巴细胞、浆细胞和巨噬细胞浸润。免疫组织化学染色 CD68、CD34、结蛋白和 ALK 标志物均为阴性，IgG4 阳性浆细胞数量增加。最终诊断为侵袭性 IgG4 相关硬化性食管炎性假瘤。
食管炎性假瘤是一种极其罕见的良性病变，但可能导致侵袭性临床表现。诊断的金标准是手术切除标本的组织病理学检查。根治性切除术仍然是最有效的治疗方法。
