上颌血管肉瘤是一种源自血管内皮细胞的侵袭性肿瘤，非常罕见。近年来，抗血管内皮生长因子（VEGF）疗法引起了广泛关注。我们描述了一名上颌血管肉瘤患者的临床病程，并讨论了通过免疫组织学分析评估的 VEGF 信号分子的表达。一名 81 岁男性左上颌窦出现侵袭性肿瘤。活检显示非典型核细胞增殖，肿瘤被怀疑是肉瘤。上颌恶性肿瘤的治疗采用多学科方法，结合手术、放疗和区域化疗。对第一次手术中获得的标本进行检查发现上颌血管肉瘤，CD31 呈阳性，而 CD34、D2-40 和因子 Ⅷ 呈阴性。虽然计划的大范围手术后未观察到病理残留肿瘤，但出现颈部淋巴结转移和远处转移。患者在第一次手术后 24 个月死亡。染色显示 VEGF 受体 (VEGFR) 1、VEGFR2、磷酸化 Ak 菌株转化、丝裂原激活蛋白激酶、信号转导子和转录激活子 3 呈阳性。尽管我们的研究结果并不表明抗 VEGF 治疗对治疗上颌血管肉瘤有益，但我们发现 VEGFR 信号通路在上颌血管肉瘤中被激活，类似于起源于其他部位的血管肉瘤。在此，我们报告一例上颌血管肉瘤，重点关注 VEGFR 和信号通路激活。据我们所知，这是第一份描述上颌血管肉瘤 VEGFR 系统免疫染色结果的报告。

Maxillary angiosarcoma, an aggressive tumor derived from vascular endothelial cells, is very rare. Recently, antivascular endothelial growth factor (VEGF) therapies have attracted considerable attention. We describe the clinical course of a patient with maxillary angiosarcoma and discuss the expression of VEGF signaling molecules assessed via immunohistological analysis. An 81-year-old man presented with an aggressive tumor in the left maxillary sinus. Biopsy revealed atypical nuclear cell proliferation, and the tumor was suspected to be a sarcoma. The maxillary malignancy was treated using a multidisciplinary approach with a combination of surgery, radiotherapy, and regional chemotherapy. Examination of the specimen obtained in the first surgery revealed maxillary angiosarcoma, found to be positive for CD31, while negative for CD34, D2-40, and factor Ⅷ. Although no pathological residual tumor was observed after the planned wide surgery, cervical lymph node and distant metastases occurred. The patient died 24 months after the first surgery. Staining revealed VEGF receptor (VEGFR) 1, VEGFR2, phosphorylated Ak strain transforming, mitogen-activated protein kinase, and signal transducer and activator of transcription 3 positivity. Although our findings do not indicate that anti-VEGF therapy is beneficial for treating maxillary angiosarcomas, we found that VEGFR signaling pathways were activated in maxillary angiosarcomas similar to angiosarcomas originating at other sites. Herein, we report a case of maxillary angiosarcoma, focused on VEGFR and signaling pathway activation. To our knowledge, this is the first report to describe VEGFR system immunostaining findings in maxillary angiosarcoma.