{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T22:35:20Z","timestamp":1763591720744,"version":"3.41.2"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T00:00:00Z","timestamp":1677456000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Splanchnic vein thrombosis due to co-existing metastatic pancreatic neuroendocrine tumour (pNET) and JAK2V617F mutation is a rare condition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case report<\/jats:title><jats:p>Here we present a case of a young woman with complete remission of a non-functioning grade 2 pNET with unresectable liver metastases, coexisting with JAK2V617F mutation. Splenectomy and distal pancreatectomy were performed. Neither surgical removal, nor radiofrequency ablation of the liver metastases was possible. Therefore, somatostatin analogue (SSA) and enoxaparine were started. Peptide receptor radionuclide therapy (PRRT) was given in 3 cycles 6-8 weeks apart. Genetic testing revealed no multiple endocrine neoplasia type 1 (MEN-1) gene mutations. After shared decision making with the patient, she gave birth to two healthy children, currently 2 and 4 years old. On pregnancy confirmation, SSA treatment was interrupted and resumed after each delivery. Ten years after the diagnosis of pNET, no tumour is detectable by MRI or somatostatin receptor scintigraphy. PRRT followed by continuous SSA therapy, interrupted only during pregnancies, resulted in complete remission and enabled the patient to complete two successful pregnancies.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fendo.2023.1095815","type":"journal-article","created":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T06:08:21Z","timestamp":1677478101000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":6,"title":["Case report: Metastatic pancreatic neuroendocrine tumour associated with portal vein thrombosis; successful management with subsequent pregnancies"],"prefix":"10.3389","volume":"14","author":[{"given":"L\u00edvia","family":"Sira","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"No\u00e9mi","family":"Zs\u00edros","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"L\u00e1szl\u00f3","family":"Bidiga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S\u00e1ndor","family":"Barna","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zsolt","family":"Kany\u00e1ri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Edit B.","family":"Nagy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicolas","family":"Guillaume","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Damian","family":"Wild","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katalin","family":"R\u00e1zs\u00f3","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Szilvia","family":"And\u00f3","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Istv\u00e1n","family":"Balogh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Endre V.","family":"Nagy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zolt\u00e1n","family":"Balogh","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2023,2,27]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.2147\/VHRM.S19773","article-title":"Splanchnic vein thrombosis: Current perspective","volume":"15","author":"Valeriani","year":"2019","journal-title":"Vasc Health Risk Manage"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1097\/MPA.0000000000000770","article-title":"Complicated case presentation: Management of pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1","volume":"46","author":"Mulvey","year":"2017","journal-title":"Pancreas"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1590\/1806-9282.20200778","article-title":"The evaluation of patients with essential thrombocythemia in terms of risk of thrombosis","volume":"67","author":"Sunu","year":"2021","journal-title":"Rev Assoc Med Bras"},{"key":"B4","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1038\/s41408-021-00470-y","article-title":"Mutations and thrombosis in essential thrombocythemia","volume":"11","author":"Guglielmelli","year":"2021","journal-title":"Blood Cancer J"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29099","article-title":"Exploring the rising incidence of neuroendocrine tumors: a population-based analysis of epidemiology, metastatic presentation, and outcomes","volume":"121","author":"Hallet","year":"2015","journal-title":"Cancer"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2008.05.047","article-title":"Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors","volume":"135","author":"Metz","year":"2008","journal-title":"Gastroenterology"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1001\/jamaoncol.2017.0589","article-title":"Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the united states","volume":"3","author":"Dasari","year":"2017","journal-title":"JAMA Oncol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1080\/0284186X.2021.1921262","article-title":"Nordic Guidelines 2021 for diagnosis and treatment of gastroenteropancreatic neuroendocrine neoplasms","volume":"60","author":"Janson","year":"2021","journal-title":"Acta Oncol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.31137","article-title":"Survival in neuroendocrine neoplasms; A report from a large Norwegian population-based study","volume":"142","author":"Cetinkaya","year":"2018","journal-title":"Int J Cancer"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.21037\/jgo.2020.03.07","article-title":"Pancreatic neuroendocrine neoplasms: Current state and ongoing controversies on terminology, classification and prognostication","volume":"11","author":"Taskin","year":"2020","journal-title":"J Gastrointest Oncol"},{"volume-title":"WHO classification of tumours of the digestive system","year":"2010","author":"Bosman","key":"B11"},{"key":"B12","first-page":"295","article-title":"Tumors of the pancreas","volume-title":"WHO classification of tumors: Digestive system tumours","author":"Gill","year":"2019"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1097\/MPA.0000000000000825","article-title":"Treatment of pancreatic neuroendocrine tumors in multiple endocrine neoplasia-type 1 (MEN1): Some clarity but continued controversy","volume":"46","author":"Jensen","year":"2017","journal-title":"Pancreas"},{"key":"B14","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1007\/s11899-009-0005-6","article-title":"The 2008 WHO diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis","volume":"4","author":"Thiele","year":"2009","journal-title":"Curr Hematol Malig Rep"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1200\/JCOOP.20.00010","article-title":"Understanding the management and treatment of well-differentiated pancreatic neuroendocrine tumors: A clinician\u2019s guide to a complex illness","volume":"16","author":"Li","year":"2020","journal-title":"JCO Oncol Pract"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.3389\/fendo.2021.679000","article-title":"Role of somatostatin receptor in pancreatic neuroendocrine tumor development, diagnosis, and therapy","volume":"12","author":"Hu","year":"2021","journal-title":"Front Endocrinol (Lausanne)"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13071701","article-title":"Targeted cancer therapy: What\u2019s new in the field of neuroendocrine neoplasms","volume":"13","author":"La Salvia","year":"2021","journal-title":"Cancers (Basel)"},{"key":"B18","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1159\/000443612","article-title":"Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors (Promid): Results of long-term survival","volume":"104","author":"Rinke","year":"2017","journal-title":"Neuroendocrinology"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1409757","article-title":"Lanreotide in metastatic enteropancreatic neuroendocrine tumors","volume":"371","author":"Caplin","year":"2014","journal-title":"N Engl J Med"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1607427","article-title":"Phase 3 trial of Lu-dotatate for midgut neuroendocrine tumors","volume":"376","author":"Strosberg","year":"2017","journal-title":"New Engl J Med"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(21)00572-6","article-title":"177Lu-dotatate plus long-acting octreotide versus high\u2212dose long-acting octreotide in patients with midgut neuroendocrine tumours (NETTER-1): Final overall survival and long-term safety results from an open-label, randomised, controlled, phase 3 trial","volume":"22","author":"Strosberg","year":"2021","journal-title":"Lancet Oncol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1016\/annonc\/annonc1060","article-title":"First multicentric randomized phase II trial investigating the antitumor efficacy of peptide receptor radionucleide therapy with 177lutetium\u2013octreotate (OCLU) in unresectable progressive neuroendocrine pancreatic tumor: results of the OCLURANDOM trial","volume":"33","author":"Baudin","year":"2022","journal-title":"Ann Oncol"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.2967\/jnumed.113.125336","article-title":"Predictors of long-term outcome in patients with well-differentiated gastroenteropancreatic neuroendocrine tumors after peptide receptor radionuclide therapy with 177Lu-octreotate","volume":"55","author":"Ezziddin","year":"2014","journal-title":"J Nucl Med"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.15357","article-title":"Essential thrombocythemia due to janus kinase 2 mutation unmasked after splenectomy","volume":"13","author":"Khanduri","year":"2021","journal-title":"Cureus"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.3748\/wjg.v26.i19.2305","article-title":"Pancreatic neuroendocrine tumors: A review of serum biomarkers, staging, and management","volume":"26","author":"Ma","year":"2020","journal-title":"World J Gastroenterol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1007\/s00423-008-0346-y","article-title":"Non-functioning, malignant pancreatic neuroendocrine tumour (PNET): A rare entity during pregnancy","volume":"394","author":"Kamphues","year":"2009","journal-title":"Langenbecks Arch Surg"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2021.646992","article-title":"Case report: Grade 2 metastatic pancreatic neuroendocrine tumor with progression of one metastasis after pregnancy to grade 3 Large-cell neuroendocrine carcinoma: One case cured by resection with genomic characterization of the two components","volume":"11","author":"Raoul","year":"2021","journal-title":"Front Oncol"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2019-233263","article-title":"Complete remission of inoperable hepatic and bone metastases due to neuroendocrine pancreatic tumour 3 years after peptide receptor radionucleotide therapy","volume":"13","author":"Soeiro","year":"2020","journal-title":"BMJ Case Rep"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1097\/RLU.0000000000004537","article-title":"Complete remission upon peptide receptor radionuclide therapy in a G2 pancreatic neuroendocrine tumor","author":"Weich","year":"2023","journal-title":"Clin Nucl Med"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1016\/j.annonc.2020.03.304","article-title":"Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up","volume":"31","author":"Pavel","year":"2020","journal-title":"Ann Oncol"},{"key":"B31","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1159\/000475526","article-title":"ENETS consensus guidelines for the standards of care in neuroendocrine neoplasms: Peptide receptor radionuclide therapy with radiolabelled somatostatin analogues","volume":"105","author":"Hicks","year":"2017","journal-title":"Neuroendocrinology"},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.3390\/cancers14235792","article-title":"A clinical guide to peptide receptor radionuclide therapy with 177Lu-DOTATATE in neuroendocrine tumor patients","volume":"14","author":"Becx","year":"2022","journal-title":"Cancers (Basel)"}],"container-title":["Frontiers in Endocrinology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2023.1095815\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,2,27]],"date-time":"2023-02-27T06:08:25Z","timestamp":1677478105000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2023.1095815\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,2,27]]},"references-count":32,"alternative-id":["10.3389\/fendo.2023.1095815"],"URL":"https:\/\/doi.org\/10.3389\/fendo.2023.1095815","relation":{},"ISSN":["1664-2392"],"issn-type":[{"type":"electronic","value":"1664-2392"}],"subject":[],"published":{"date-parts":[[2023,2,27]]},"article-number":"1095815"}}