PMID- 36923225
OWN - NLM
STAT- MEDLINE
DCOM- 20230322
LR  - 20230324
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 14
DP  - 2023
TI  - Case report: Metastatic pancreatic neuroendocrine tumour associated with portal 
      vein thrombosis; successful management with subsequent pregnancies.
PG  - 1095815
LID - 10.3389/fendo.2023.1095815 [doi]
LID - 1095815
AB  - BACKGROUND: Splanchnic vein thrombosis due to co-existing metastatic pancreatic 
      neuroendocrine tumour (pNET) and JAK2V617F mutation is a rare condition. CASE 
      REPORT: 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.
CI  - Copyright (c) 2023 Sira, Zsiros, Bidiga, Barna, Kanyari, Nagy, Guillaume, Wild, 
      Razso, Ando, Balogh, Nagy and Balogh.
FAU - Sira, Livia
AU  - Sira L
AD  - Division of Endocrinology, Department of Internal Medicine, Faculty of Medicine, 
      University of Debrecen, Debrecen, Hungary.
FAU - Zsiros, Noemi
AU  - Zsiros N
AD  - Division of Endocrinology, Department of Internal Medicine, Faculty of Medicine, 
      University of Debrecen, Debrecen, Hungary.
FAU - Bidiga, Laszlo
AU  - Bidiga L
AD  - Department of Pathology, Faculty of Medicine, University of Debrecen, Debrecen, 
      Hungary.
FAU - Barna, Sandor
AU  - Barna S
AD  - Department of Nuclear Medicine, Faculty of Medicine, University of Debrecen, 
      Debrecen, Hungary.
FAU - Kanyari, Zsolt
AU  - Kanyari Z
AD  - Department of Surgery, Faculty of Medicine, University of Debrecen, Debrecen, 
      Hungary.
FAU - Nagy, Edit B
AU  - Nagy EB
AD  - Department of Radiology, Faculty of Medicine, University of Debrecen, Debrecen, 
      Hungary.
FAU - Guillaume, Nicolas
AU  - Guillaume N
AD  - Clinic of Radiology and Nuclear Medicine, University of Basel Hospital, Basel, 
      Switzerland.
FAU - Wild, Damian
AU  - Wild D
AD  - Clinic of Radiology and Nuclear Medicine, University of Basel Hospital, Basel, 
      Switzerland.
FAU - Razso, Katalin
AU  - Razso K
AD  - Division of Haematology, Department of Medicine, Faculty of Medicine, University 
      of Debrecen, Debrecen, Hungary.
FAU - Ando, Szilvia
AU  - Ando S
AD  - Division of Clinical Genetics, Department of Laboratory Medicine, Faculty of 
      Medicine, University of Debrecen, Debrecen, Hungary.
FAU - Balogh, Istvan
AU  - Balogh I
AD  - Division of Clinical Genetics, Department of Laboratory Medicine, Faculty of 
      Medicine, University of Debrecen, Debrecen, Hungary.
FAU - Nagy, Endre V
AU  - Nagy EV
AD  - Division of Endocrinology, Department of Internal Medicine, Faculty of Medicine, 
      University of Debrecen, Debrecen, Hungary.
FAU - Balogh, Zoltan
AU  - Balogh Z
AD  - Division of Endocrinology, Department of Internal Medicine, Faculty of Medicine, 
      University of Debrecen, Debrecen, Hungary.
LA  - eng
PT  - Case Reports
DEP - 20230227
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
RN  - 51110-01-1 (Somatostatin)
RN  - Non functioning pancreatic endocrine tumor
SB  - IM
MH  - Female
MH  - Humans
MH  - Pregnancy
MH  - *Adenoma, Islet Cell
MH  - *Liver Neoplasms/therapy/secondary
MH  - *Neoplasms, Second Primary
MH  - *Neuroectodermal Tumors, Primitive/complications
MH  - *Neuroendocrine Tumors/complications/therapy/pathology
MH  - *Pancreatic Neoplasms/complications/therapy/diagnosis
MH  - Portal Vein
MH  - Somatostatin
MH  - *Thrombosis
PMC - PMC10008953
OTO - NOTNLM
OT  - complete remission
OT  - metastatic pancreatic neuroendocrine tumour
OT  - pregnancy
OT  - splanchnic vein thrombosis
OT  - thrombocytosis
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/03/17 06:00
MHDA- 2023/03/21 06:00
PMCR- 2023/01/01
CRDT- 2023/03/16 02:18
PHST- 2022/11/11 00:00 [received]
PHST- 2023/02/10 00:00 [accepted]
PHST- 2023/03/16 02:18 [entrez]
PHST- 2023/03/17 06:00 [pubmed]
PHST- 2023/03/21 06:00 [medline]
PHST- 2023/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2023.1095815 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2023 Feb 27;14:1095815. doi: 
      10.3389/fendo.2023.1095815. eCollection 2023.