{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T22:15:00Z","timestamp":1785795300791,"version":"3.56.0"},"reference-count":26,"publisher":"FapUNIFESP (SciELO)","license":[{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"am","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2022,1,1]],"date-time":"2022-01-01T00:00:00Z","timestamp":1640995200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["ABCD, arq. bras. cir. dig."],"abstract":"<jats:p>ABSTRACT  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 a patient with pheochromocytoma, capillary hemangioblastoma in the peripheral retina, and two neuroendocrine tumors in the pancreas.  RESULTS: 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 en bloc. 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.  CONCLUSION: 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.<\/jats:p>","DOI":"10.1590\/0102-672020220002e1697","type":"journal-article","created":{"date-parts":[[2022,11,25]],"date-time":"2022-11-25T14:16:39Z","timestamp":1669385799000},"source":"Crossref","is-referenced-by-count":2,"title":["PANCREATODUODENECTOMY IN PATIENT WITH VON HIPPEL-LINDAU DISEASE: A LITERATURE REVIEW"],"prefix":"10.1590","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5486-0132","authenticated-orcid":false,"given":"Jos\u00e9 Marcus Raso","family":"Eul\u00e1lio","sequence":"first","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5453-5586","authenticated-orcid":false,"given":"Thales Penna","family":"Carvalho","sequence":"additional","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6504-6769","authenticated-orcid":false,"given":"Elo\u00e1 Pereira","family":"Brabo","sequence":"additional","affiliation":[{"name":"University Hospital Clementino Fraga Filho,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6272-4253","authenticated-orcid":false,"given":"Antonio Luis Eiras","family":"Ara\u00fajo","sequence":"additional","affiliation":[{"name":"University Hospital Clementino Fraga Filho,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8737-3181","authenticated-orcid":false,"given":"Adriana de Oliveira","family":"Eul\u00e1lio","sequence":"additional","affiliation":[{"name":"National Institute of Traumatology and Orthopedics,  Brazil; University Hospital Clementino Fraga Filho,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3031-7530","authenticated-orcid":false,"given":"Felipe Nogueira","family":"Beir\u00e3o","sequence":"additional","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9694-7415","authenticated-orcid":false,"given":"Jos\u00e9 Eduardo Ferreira","family":"Manso","sequence":"additional","affiliation":[{"name":"Universidade Federal do Rio de Janeiro,  Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"530","published-online":{"date-parts":[[2022]]},"reference":[{"issue":"4","key":"ref1","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1016\/j.pan.2015.05.468","article-title":"Safety and efficacy of early drain removal and triple-drug therapy to prevent pancreatic fistula after distal pancreatectomy","volume":"15","author":"Adachi T","year":"2015","journal-title":"Pancreatology"},{"issue":"4","key":"ref2","article-title":"Serous cystadenoma of pancreas: why there is low accuracy in imaging exams?","volume":"34","author":"Amico EC","year":"2022","journal-title":"ABCD Arq Bras Cir Dig"},{"key":"ref3","series-title":"AJCC Cancer Staging Manual","author":"Amin MB","year":"2017"},{"issue":"2","key":"ref4","doi-asserted-by":"crossref","first-page":"267","DOI":"10.3390\/cancers11020267","article-title":"Preoperative imaging evaluation after downstaging of pancreatic ductal adenocarcinoma: a multi-center study","volume":"11","author":"Bele\u00f9 A","year":"2019","journal-title":"Cancers (Basel)"},{"issue":"4","key":"ref5","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1016\/S0750-7658(03)00060-1","article-title":"Maladie de von Hippel-Lindau et anesth\u00e9sie obst\u00e9tricale: \u00e0 propos de 3 cas","volume":"22","author":"Berl 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size: implications for radiation therapy planning","volume":"104","author":"Qiu H","year":"2012","journal-title":"Radiother Oncol"},{"issue":"1","key":"ref21","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1186\/s13037-019-0219-z","article-title":"Standardization of early drain removal following pancreatic resection: proposal of the \u201cOttawa pancreatic drain algorithm\u201d","volume":"13","author":"Smith H","year":"2019","journal-title":"Patient Saf Surg"},{"issue":"4","key":"ref22","doi-asserted-by":"crossref","first-page":"1631","DOI":"10.1210\/jc.2017-02434","article-title":"In silico VHL gene mutation analysis and prognosis of pancreatic neuroendocrine tumors in von Hippel-Lindau disease","volume":"103","author":"Tirosh A","year":"2018","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"ref23","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1001\/jamaoncol.2017.3428","article-title":"Association of VHL genotype with pancreatic neuroendocrine tumor phenotype in patients with von Hippel-Lindau disease","volume":"4","author":"Tirosh A","year":"2018","journal-title":"JAMA Oncol"},{"issue":"5","key":"ref24","first-page":"1083","article-title":"Epidural anesthesia for cesarean section in a patient with von Hippel-Lindau disease and multiple sclerosis","volume":"88","author":"Wang A","year":"1999","journal-title":"Anesth Analg"},{"issue":"1","key":"ref25","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1186\/s12881-019-0930-8","article-title":"Novel gene mutation in von Hippel-Lindau disease \u2013 a report of two cases","volume":"20","author":"Wang J","year":"2019","journal-title":"BMC Med Genet"},{"issue":"5","key":"ref26","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1136\/jmedgenet-2017-104995","article-title":"Risk factors for survival in patients with von Hippel-Lindau disease","volume":"55","author":"Wang J-Y","year":"2018","journal-title":"J Med Genet"}],"container-title":["ABCD. Arquivos Brasileiros de Cirurgia Digestiva (S\u00e3o Paulo)"],"original-title":["PANCREATICODUODENECTOMIA EM PACIENTE COM DOEN\u00c7A DE VON HIPPEL-LINDAU - REVIS\u00c3O DA LITERATURA"],"link":[{"URL":"http:\/\/www.scielo.br\/scielo.php?script=sci_pdf&pid=S0102-67202022000100405&tlng=en","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,11,25]],"date-time":"2022-11-25T14:17:04Z","timestamp":1669385824000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.scielo.br\/scielo.php?script=sci_arttext&pid=S0102-67202022000100405&tlng=en"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022]]},"references-count":26,"alternative-id":["S0102-67202022000100405"],"URL":"https:\/\/doi.org\/10.1590\/0102-672020220002e1697","relation":{},"ISSN":["2317-6326","0102-6720"],"issn-type":[{"value":"2317-6326","type":"electronic"},{"value":"0102-6720","type":"print"}],"subject":[],"published":{"date-parts":[[2022]]},"article-number":"e1697"}}