Nakamura Naoto, Kasai Yosuke, Nagai Kazuyuki, Sato Asahi, Kadono Kentaro, Uza Norimitsu, Ohno Tsuyoshi, Koyasu Sho, Nakamoto Yuji, Sano Noritaka, Takahashi Ayako, Otsuki Shinya, Ito Hiroaki, Yamane Kei, Anazawa Takayuki, Ogiso Satoshi, Uchida Yoichiro, Ito Takashi, Ishii Takamichi, Hatano Etsuro
Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Kyoto, Japan.
Department of Gastroenterological Surgery, Kyoto-Katsura Hospital, Kyoto, Kyoto, Japan.
Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.25-0247. Epub 2025 Sep 5.
von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary disorder characterized by the development of tumor-like lesions in multiple organs. While central nervous system hemangioblastomas, pancreatic neuroendocrine tumors, and pancreatic cysts are commonly associated with VHL disease, there have been few reported cases of pancreatic hemangioblastoma in patients with VHL disease.
A male patient in his 30s had been diagnosed with VHL disease and had been followed for cerebellar and spinal hemangioblastomas, and renal cell carcinoma, for which he had undergone several tumor resections, radiation therapy, and a ventriculoperitoneal shunt. A pancreatic head tumor deemed to be a neuroendocrine tumor on imaging findings exhibited a gradual increase in size from 12 to 33 mm for the past 2 years, but it had been monitored due to his comorbidities and declining daily living activities. Severe anemia was detected during his regular outpatient visit, and an emergency esophagogastroduodenoscopy revealed a submucosal tumor near the duodenal papilla with ulceration and active bleeding, making endoscopic hemostasis challenging. Dynamic contrast-enhanced CT showed active bleeding from the pancreatic tumor. Subsequently, emergency angiography was performed via the superior mesenteric artery, successfully embolizing vessels supplied by the inferior pancreaticoduodenal artery to achieve hemostasis. Due to concerns about rebleeding, we performed pancreaticoduodenectomy 1 month after the emergency angiography, during which we awaited the improvement of the patient's overall condition. Microscopic findings of the tumor showed multinodular proliferation with hematoxylin-eosin staining, revealing cells with clear cytoplasm and abundant capillaries and dilated branching vessels within the nests. Immunohistochemical analysis demonstrated positivity for alpha-inhibin and S100, with partial positivity for carbonic anhydrase IX, leading to a diagnosis of pancreatic hemangioblastoma.
This paper reports a rare case of pancreatic hemangioblastoma arising in a patient with VHL disease. It is crucial to consider the possibility of pancreatic hemangioblastoma when treating pancreatic tumors in VHL disease patients.
冯·希佩尔-林道（VHL）病是一种常染色体显性遗传性疾病，其特征是多个器官出现肿瘤样病变。虽然中枢神经系统血管母细胞瘤、胰腺神经内分泌肿瘤和胰腺囊肿通常与VHL病相关，但VHL病患者中胰腺血管母细胞瘤的报道病例很少。
一名30多岁的男性患者被诊断为VHL病，一直在接受小脑和脊髓血管母细胞瘤以及肾细胞癌的随访，为此他接受了多次肿瘤切除术、放射治疗和脑室腹腔分流术。影像学检查发现胰头肿瘤被认为是神经内分泌肿瘤，在过去2年中其大小从12毫米逐渐增加到33毫米，但由于他的合并症和日常生活活动能力下降，一直在对其进行监测。在他定期门诊就诊期间检测到严重贫血，急诊食管胃十二指肠镜检查发现十二指肠乳头附近有一个黏膜下肿瘤，伴有溃疡和活动性出血，使得内镜止血具有挑战性。动态对比增强CT显示胰腺肿瘤有活动性出血。随后，通过肠系膜上动脉进行了急诊血管造影，成功栓塞了胰十二指肠下动脉供应的血管以实现止血。由于担心再次出血，我们在急诊血管造影1个月后进行了胰十二指肠切除术，在此期间我们等待患者整体状况的改善。肿瘤的显微镜检查结果显示苏木精-伊红染色呈多结节增殖，可见细胞质清晰的细胞以及巢内丰富的毛细血管和扩张的分支血管。免疫组织化学分析显示α-抑制素和S100呈阳性，碳酸酐酶IX部分呈阳性，从而诊断为胰腺血管母细胞瘤。
本文报道了一例VHL病患者发生胰腺血管母细胞瘤的罕见病例。在治疗VHL病患者的胰腺肿瘤时，考虑胰腺血管母细胞瘤的可能性至关重要。
