血管瘤瘤是一种带有外周血管平滑肌分化的肿瘤，极少在食管发生，并且可能会根据以前的病例报告在这个部位有侵袭性表现。本研究描述了1984年至2022年期间，在两个大型学术机构诊断出的三例食管血管瘤瘤的临床病理特征。发现三例食管血管瘤瘤患者分别为两名女性和一名男性，年龄范围为19-65岁。所有三个肿瘤均表现为恶性，转移到不同的部位（淋巴结、肺、心包、胸膜、膈肌、头皮）。一名患者发展成主动脉食管瘘，导致致命性出血。肿瘤大小从4.5到8.1厘米不等。组织学上，所有肿瘤均具有多结节，外周性生长模式。这些肿瘤呈现出不同程度的细胞学异型性和纺锤样变形，提高了有丝分裂活性（10个高倍视野内2-12个有丝分裂像），在两例病例中观察到坏死。所有肿瘤均通过免疫组化检测表达平滑肌肌动蛋白，并携带NOTCH基因突变（两例病例中的MIR143 ::NOTCH2融合；一例病例中的NOTCH3重排和NOTCH1点突变）。在一例病例中也发现了ATRX剪接突变exon 10。食管血管瘤瘤在诊断上具有挑战性，因为其在此处的罕见性，但通过其特征的外周性生长模式、圆形中心细胞核和支持性辅助研究可予以识别。鉴于此部位具有侵袭性行为的倾向，我们建议通过多学科肉瘤团队进行管理以获得最佳治疗效果。©2023年作者。经John Wiley＆Sons Ltd出版的组织病理学。

Glomus tumours are neoplasms with perivascular smooth muscle differentiation, which rarely occur in the oesophagus and may behave aggressively in this site based upon prior case reports. This study describes the clinicopathologic features of three oesophageal glomus tumours diagnosed at two large academic institutions between 1984 and 2022.Three cases of oesophageal glomus tumours were identified. Patients included two females and one male, with an age range of 19-65 years. All three tumours behaved in a malignant fashion, with metastases to various sites (lymph nodes, lung, pericardium, pleura, diaphragm, scalp). One patient developed an aorto-oesophageal fistula, resulting in a fatal haemorrhage. Tumours ranged in size from 4.5 to 8.1 cm. Histologically, all tumours had a multinodular, perivascular growth pattern. The neoplasms showed varying degrees of cytologic atypia and spindling, elevated mitotic activity (2-12 mitotic figures per 10 high-power fields), and necrosis was seen in in two cases. All tumours expressed smooth muscle actin by immunohistochemistry, and harboured NOTCH gene alterations (MIR143::NOTCH2 fusion in two cases; NOTCH3 rearrangement and NOTCH1 point mutation in one case). An ATRX splicing mutation in exon 10 was also identified in one case.Oesophageal glomus tumours pose diagnostic challenges, given their rarity at this site, but can be recognised by their characteristic perivascular growth pattern, round central nuclei, and supportive ancillary studies. Given the propensity for aggressive behaviour in this location, we recommend management by a multidisciplinary sarcoma team for optimal outcome.© 2023 The Authors. Histopathology published by John Wiley & Sons Ltd.