Endocrine Tumour Biology

We focus on clinical and biological aspects of rare endocrine diseases, such as adrenocortical cancer and neuroendocrine tumours with special emphasis on the multiple endocrine neoplasia type 1 syndrome (MEN1). We try to understand the basic mechanisms causing these diseases, and perform clinical trials in order to evaluate treatment regiments. As being part of the Uppsala Centre of Excellence for Neuroendocrine Tumors, a national referral centre, we are in a good position to translate our results and experiences into new and better clinical practise.
Description of our research
Carriers with mutations in the gene MEN1 develop multiple tumours of the parathyroids, endocrine pancreas and pituitary. MEN1 is thought to be a tumour suppressor gene, but it is unclear why MEN1 inactivation leads to tumour formation only in some endocrine organs. We try to address this question through analyses of the consequences of MEN1 inactivation, and through studies of other factors that may interact with MEN1. In our experimental studies we use contemporary cell- and molecular techniques, as well as animal models and tissue samples from patients. Through our collection of clinical data and materials from some 60 MEN1 families, we can convert our basics studies to clinical practise, for example through prospective clinical trials.
Our second research area concerns adrenocortical carcinoma, an aggressive cancer with poor prognosis. In order to evaluate existing treatment options, we have initiated and, together with several collaborators, conducted a randomized clinical trial (FIRM-ACT). The study, involving more than 300 patients, has recently been completed, and the results will shortly be at hand.
Group members
Publications
Part of ESMO Open, 2024
- DOI for Carcinoid heart disease in patients with advanced small-intestinal neuroendocrine tumors and carcinoid syndrome: a retrospective experience from two European referral centers
- Download full text (pdf) of Carcinoid heart disease in patients with advanced small-intestinal neuroendocrine tumors and carcinoid syndrome: a retrospective experience from two European referral centers
Part of Journal of Pathology, p. 357-370, 2024
- DOI for The evolutionary history of metastatic pancreatic neuroendocrine tumours reveals a therapy driven route to high-grade transformation
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Reinventing ESMO after the COVID-19 pandemic: moving towards a sustainable academic society
Part of ESMO Open, 2024
- DOI for Reinventing ESMO after the COVID-19 pandemic: moving towards a sustainable academic society
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Genetics-guided therapy in neuroendocrine carcinoma: response to BRAF- and MEK-inhibitors
Part of Upsala Journal of Medical Sciences, 2024
- DOI for Genetics-guided therapy in neuroendocrine carcinoma: response to BRAF- and MEK-inhibitors
- Download full text (pdf) of Genetics-guided therapy in neuroendocrine carcinoma: response to BRAF- and MEK-inhibitors
Part of Clinical Endocrinology, p. 234-242, 2024
Part of Nature Reviews Endocrinology, p. 168-184, 2024
Part of Endocrine-Related Cancer, 2024
Part of Endocrine, p. 171-179, 2023
Overall Survival in Patients with Stage IV Pan-NET Eligible for Liver Transplantation
Part of World Journal of Surgery, p. 340-347, 2023
- DOI for Overall Survival in Patients with Stage IV Pan-NET Eligible for Liver Transplantation
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ENETS standardized (synoptic) reporting for radiological imaging in neuroendocrine tumours
Part of Journal of neuroendocrinology, 2022
- DOI for ENETS standardized (synoptic) reporting for radiological imaging in neuroendocrine tumours
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Part of Neuroendocrinology, p. 298-309, 2022
- DOI for Long-Term Treatment with Telotristat Ethyl in Patients with Carcinoid Syndrome Symptoms: Results from the TELEPATH Study
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Part of Annals of Surgery Open, 2022
- DOI for Benefit of Primary Tumor Resection in Stage IV, Grade 1 and 2, Pancreatic Neuroendocrine Tumors: A Propensity-Score Matched Cohort Study
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Poor outcome after systemic therapy in secondary high-grade pancreatic neuroendocrine tumors
Part of Endocrine Connections, 2022
- DOI for Poor outcome after systemic therapy in secondary high-grade pancreatic neuroendocrine tumors
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Part of Journal of Clinical Medicine, 2022
- DOI for A Multinational Pilot Study on Patients' Perceptions of Advanced Neuroendocrine Neoplasms on the EORTC QLQ-C30 and EORTC QLQ-GINET21 Questionnaires
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Targeted Therapies in Pheochromocytoma and Paraganglioma
Part of Journal of Clinical Endocrinology and Metabolism, p. 2963-2972, 2022
Consensus on molecular imaging and theranostics in neuroendocrine neoplasms
Part of European Journal of Cancer, p. 56-73, 2021
Cardiac Imaging in Carcinoid Heart Disease
Part of JACC Cardiovascular Imaging, p. 2240-2253, 2021
ENSAT registry-based randomized clinical trials for adrenocortical carcinoma
Part of European Journal of Endocrinology, 2021
Antiproliferative Systemic Therapies for Metastatic Small Bowel Neuroendocrine Tumours
Part of Current Treatment Options in Oncology, 2021
Part of Neuroendocrinology, p. 831-839, 2021
Part of Neuroendocrinology, p. 330-343, 2021
- DOI for 177Lu-DOTATATE Therapy of Advanced Pancreatic Neuroendocrine Tumors Heavily Pretreated With Chemotherapy: Analysis of Outcome, Safety and Their Determinants
- Download full text (pdf) of 177Lu-DOTATATE Therapy of Advanced Pancreatic Neuroendocrine Tumors Heavily Pretreated With Chemotherapy: Analysis of Outcome, Safety and Their Determinants
Part of Clinical Cancer Research, p. 2989-2995, 2021
Part of World Journal of Gastroenterology, p. 976-989, 2021
Part of Scientific Reports, 2021
- DOI for MiR-486-3p was downregulated at microRNA profiling of adrenals of multiple endocrine neoplasia type 1 mice, and inhibited human adrenocortical carcinoma cell lines
- Download full text (pdf) of MiR-486-3p was downregulated at microRNA profiling of adrenals of multiple endocrine neoplasia type 1 mice, and inhibited human adrenocortical carcinoma cell lines
Part of Annals of Oncology, p. 1425-1433, 2021
Part of Annals of Surgical Oncology, p. 7506-7517, 2021
Systemic Treatment of Gastroenteropancreatic Neuroendocrine Carcinoma
Part of Current Treatment Options in Oncology, 2021
- DOI for Systemic Treatment of Gastroenteropancreatic Neuroendocrine Carcinoma
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Part of Neuroendocrinology, p. 609-630, 2021
18F-Radiolabeling and Preliminary Evaluation of a HSP90 ligand
Part of European Journal of Pharmaceutical Sciences, 2021
Tumor growth rate in pancreatic neuroendocrine tumor patients undergoing PRRT with 177Lu-DOTATATE.
Part of Endocrine Connections, p. 422-431, 2021
- DOI for Tumor growth rate in pancreatic neuroendocrine tumor patients undergoing PRRT with 177Lu-DOTATATE.
- Download full text (pdf) of Tumor growth rate in pancreatic neuroendocrine tumor patients undergoing PRRT with 177Lu-DOTATATE.
Para-chloro-2-[18F]fluoroethyl-etomidate: A promising new PET radiotracer for adrenocortical imaging
Part of International Journal of Medical Sciences, p. 2187-2196, 2021
- DOI for Para-chloro-2-[18F]fluoroethyl-etomidate: A promising new PET radiotracer for adrenocortical imaging
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Symptom Diaries of Patients with Midgut Neuroendocrine Tumors Treated with 177Lu-DOTATATE
Part of Journal of Nuclear Medicine, p. 1712-1718, 2021
- DOI for Symptom Diaries of Patients with Midgut Neuroendocrine Tumors Treated with 177Lu-DOTATATE
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Whole genome sequencing of apparently mutation-negative MEN1 patients
Part of European Journal of Endocrinology, p. 35-45, 2020
Part of The Lancet Oncology, 2020
High-grade progression confers poor survival in pancreatic neuroendocrine tumors
Part of Neuroendocrinology, p. 891-898, 2020
Neuroendocrine Neoplasms of the Small Bowel and Pancreas
Part of Neuroendocrinology, p. 444-476, 2020
Part of International Journal of Cancer, p. 2326-2335, 2020
Part of Journal of Hypertension, p. 1443-1456, 2020
Part of Neuroendocrinology, p. 198-216, 2020
The clinical applications of a multigene liquid biopsy (NETest) in neuroendocrine tumors
Part of Advances in Medical Sciences, p. 18-29, 2020
- DOI for The clinical applications of a multigene liquid biopsy (NETest) in neuroendocrine tumors
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Part of Neuroendocrinology, p. 185-197, 2020
Part of Scientific Reports, 2020
- DOI for Generation and characterization of CRISPR/Cas9-mediated MEN1 knockout BON1 cells: a human pancreatic neuroendocrine cell line
- Download full text (pdf) of Generation and characterization of CRISPR/Cas9-mediated MEN1 knockout BON1 cells: a human pancreatic neuroendocrine cell line
Part of Journal of Pathology, p. 183-194, 2020
Part of Annals of Oncology, p. 844-860, 2020
Part of Frontiers in Oncology, 2020
- DOI for Tumor Contrast-Enhancement for Monitoring of PRRT Lu-177-DOTATATE in Pancreatic Neuroendocrine Tumor Patients
- Download full text (pdf) of Tumor Contrast-Enhancement for Monitoring of PRRT Lu-177-DOTATATE in Pancreatic Neuroendocrine Tumor Patients
Part of EJNMMI Physics, 2020
- DOI for Kidney dosimetry in 777 patients during 177Lu-DOTATATE therapy: aspects on extrapolations and measurement time points
- Download full text (pdf) of Kidney dosimetry in 777 patients during 177Lu-DOTATATE therapy: aspects on extrapolations and measurement time points
Part of European Journal of Nuclear Medicine and Molecular Imaging, p. 2372-2382, 2020
- DOI for Impact of liver tumour burden, alkaline phosphatase elevation, and target lesion size on treatment outcomes with Lu-177-Dotatate: an analysis of the NETTER-1 study
- Download full text (pdf) of Impact of liver tumour burden, alkaline phosphatase elevation, and target lesion size on treatment outcomes with Lu-177-Dotatate: an analysis of the NETTER-1 study
Part of Upsala Journal of Medical Sciences, p. 325-329, 2020
A meta-analysis of the accuracy of a neuroendocrine tumor mRNA genomic biomarker (NETest) in blood
Part of Annals of Oncology, p. 202-212, 2020
Part of The Oncologist, 2019