{"id":6186,"date":"2025-07-08T16:32:11","date_gmt":"2025-07-08T19:32:11","guid":{"rendered":"https:\/\/revistaabcd.org.br\/?p=6186"},"modified":"2025-07-08T16:33:09","modified_gmt":"2025-07-08T19:33:09","slug":"pancreatoduodenectomy-in-patient-with-von-hippel-lindau-disease-a-literature-review","status":"publish","type":"post","link":"https:\/\/revistaabcd.org.br\/pt-br\/pancreatoduodenectomy-in-patient-with-von-hippel-lindau-disease-a-literature-review\/","title":{"rendered":"PANCREATODUODENECTOMY IN PATIENT WITH VON HIPPEL-LINDAU DISEASE: A LITERATURE REVIEW"},"content":{"rendered":"<h2><strong>BACKGROUND:<\/strong><\/h2>\n<p>The von Hippel-Lindau disease is a highly penetrant autosomal dominant syndrome characterized by tumor predisposition in different organs.<\/p>\n<h2><strong>AIM:<\/strong><\/h2>\n<p>This study aimed to describe a case of a pancreatoduodenectomy for a 30-year-old male patient with von Hippel-Lindau disease.<\/p>\n<h2><strong>METHODS:<\/strong><\/h2>\n<p>We present a case study and the literature review aiming at the state-of-the-art management of a patient with pheochromocytoma, capillary hemangioblastoma in the peripheral retina, and two neuroendocrine tumors in the pancreas.<\/p>\n<h2><strong>RESULTS:<\/strong><\/h2>\n<p>A larger pancreatic lesion was located in the uncinate process, measuring 31 mm. The smaller lesion was located in the proximal pancreas and was detected only on the positron emission tomography-computed tomography scan with DOTATOC-68Ga. Genetic investigation revealed a mutation in the locus NM_000551.3 c.482G&gt;A (p.Arg161Gln) of the Von Hippel-Lindau Human Suppressor gene. The uncinate process tumor was larger than 30 mm and the patient had a mutation on exon 3; therefore, we indicated a pancreatoduodenectomy involving the proximal pancreas to resect both tumors\u00a0<i>en bloc<\/i>. During the postoperative period, the patient presented a peripancreatic fluid collection, which was treated as a grade B pancreatic fistula with clinical resolution of the complication. On postoperative day 21, he was discharged home.<\/p>\n<h2><strong>CONCLUSION:<\/strong><\/h2>\n<p>The management of patients with von Hippel-Lindau disease and pancreatic neuroendocrine tumors is complex and must be centered on tertiary institutions with a large volume of pancreatic surgery. Although the current literature assists in decision-making in most situations, each step of the treatment requires analysis and discussion between different medical specialties, including surgeons, clinicians, radiologists, and anesthesiologists.<\/p>","protected":false},"excerpt":{"rendered":"<p>BACKGROUND: The von Hippel-Lindau disease is a highly penetrant autosomal dominant syndrome characterized by tumor predisposition in different organs. AIM: This study aimed to describe a case of a pancreatoduodenectomy for a 30-year-old male patient with von Hippel-Lindau disease. METHODS: We present a case study and the literature review aiming at the state-of-the-art management of [&hellip;]<\/p>","protected":false},"author":14,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"_lmt_disableupdate":"no","_lmt_disable":"","footnotes":""},"categories":[9],"tags":[1282,28,1451],"class_list":["post-6186","post","type-post","status-publish","format-standard","hentry","category-original-article","tag-neuroendocrine-tumors","tag-pancreaticoduodenectomy","tag-von-hippel-lindau-disease"],"acf":[],"modified_by":"Rodrigo","_links":{"self":[{"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/posts\/6186"}],"collection":[{"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/users\/14"}],"replies":[{"embeddable":true,"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/comments?post=6186"}],"version-history":[{"count":3,"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/posts\/6186\/revisions"}],"predecessor-version":[{"id":6190,"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/posts\/6186\/revisions\/6190"}],"wp:attachment":[{"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/media?parent=6186"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/categories?post=6186"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/revistaabcd.org.br\/pt-br\/wp-json\/wp\/v2\/tags?post=6186"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}