Review
Oncology
Agata Mormul, Emilia Wloszek, Julia Nowoszewska, Marta Fudalej, Michal Budzik, Anna Badowska-Kozakiewicz, Andrzej Deptala
Summary: Pancreatic ductal adenocarcinoma is the most common pancreatic tumor, accounting for 85% of cases. The remaining 15% consists of various rare neoplasms. This study gathered the latest data on the epidemiology, diagnosis, biomarkers, and management of six rare pancreatic tumors to provide important insights for their proper classification and treatment.
Article
Ophthalmology
Reza Sadeghi, Hanieh Pirankuraim, Shamim Taghvaei Javanshir, Maryam Arabi, Ahmad Bereimipour, Hamid Taghvaei Javanshir, Habibollah Mahmoodzadeh, Karim Nayernia
Summary: This study aimed to evaluate the incidence of secondary tumors in children with retinoblastoma. Through continuous and integrated bioinformatics analysis, high-expression genes including bladder cancer and small cell lung cancer, as well as low-expression genes including endometriosis, prostate, non-small cell lung cancer, glioblastoma, and renal cell carcinoma, were identified. Furthermore, proteins with high expression such as TP53, CDK2, SRC, and MAPK1, and proteins with low expression such as JUN, HSP90AA1, and UBC, were found to play a significant role in candidate cancers. Ultimately, it was discovered that retinoblastoma is strongly associated with seven other cancers.
Article
Oncology
Dominique S. V. M. Clement, Monique E. van Leerdam, Soraya de Jong, Martin O. Weickert, John K. Ramage, Margot E. T. Tesselaar, Rajaventhan Srirajaskanthan
Summary: Neuroendocrine tumours (NETs) are rare tumours arising in the digestive system, mainly the small bowel or pancreas. This study aimed to describe the presence of sarcopenia at the time of diagnosis of stage IV NET in the digestive system and correlate it with survival. Sarcopenia was present in 69% of patients, and its presence in pancreatic NETs was associated with poorer survival.
Article
Surgery
C. Ricci, S. Partelli, L. Landoni, M. Rinzivillo, C. Ingaldi, V Andreasi, C. Nessi, F. Muffatti, M. Fontana, D. Tamburrino, G. Deiro, L. Alberici, D. Campana, F. Panzuto, C. Bassi, M. Falconi, R. Casadei
Summary: This study evaluated outcomes after surgery for sporadic pancreatic neuroendocrine neoplasms (Pan-NENs), identifying 2019 WHO grade and ENETS TNM stage as important factors. Surgical approach may be without benefit for Pan-NECs, unnecessary for small G1 sporadic Pan-NENs, and insufficient for stage III-IV and G3 Pan-NENs.
BRITISH JOURNAL OF SURGERY
(2021)
Article
Endocrinology & Metabolism
Daniel J. Cuthbertson, Jorge Barriuso, Angela Lamarca, Prakash Manoharan, Thomas Westwood, Matthew Jaffa, Stephen W. Fenwick, Christina Nuttall, Fiona Lalloo, Andreas Prachalias, Michail Pizanias, Hulya Wieshmann, Mairead G. McNamara, Richard Hubner, Raj Srirajaskanthan, Gillian Vivian, John Ramage, Martin O. Weickert, D. Mark Pritchard, Sobhan Vinjamuri, Juan Valle, Vincent S. Yip
Summary: This study evaluated the role of (68)Gallium (Ga-68)-DOTA PET/CT in managing patients with panNETs, showing that the imaging findings provided additional information in 54% of patients and influenced management in 39% of cases. The PET/CT scan more accurately stages and guides treatment in patients with sporadic/familial panNETs.
FRONTIERS IN ENDOCRINOLOGY
(2021)
Article
Oncology
Lewis A. A. Hall, Sarah Powell-Brett, Oscar Thompson, Daniel Smith, Elizabeth Bradley, Stacey Smith, Suzanne Vickrage, Joanne Kemp-Blake, Keith J. J. Roberts, Tahir Shah
Summary: This study evaluated the impact of SSAs on pancreatic exocrine function in patients with NETs. The results showed a reduction in exocrine function, decreased vitamin D levels, increased HbA1C levels, and no significant change in weight after SSA therapy.
Article
Medicine, General & Internal
Violetta Rosiek, Ksenia Janas, Magdalena Witkowska, Beata Kos-Kudla
Summary: We investigated the diagnostic capacity of selected circulating biomarkers for the early detection of bone metastasis in patients with pancreatic neuroendocrine neoplasms. CA125 and CY18 showed significantly higher levels in patients with BMets compared to non-BMets patients. CA125, CY18, and B2M demonstrated potential as fair biomarkers for BMets detection with moderate accuracy. Further studies in larger patient groups are needed.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Gastroenterology & Hepatology
Eduardo A. Vega, Onur C. Kutlu, Sylvia V. Alarcon, Omid Salehi, Vera Kazakova, Olga Kozyreva, Jennifer A. Chan, Claudius Conrad
Summary: In patients with <= 2cm NF-PanNETs, tumor location in the pancreatic body or tail, tumor grade III-IV, and younger age were associated with nodal metastases and the presence of M1 disease. While watchful waiting may be appropriate for most patients, individualized risk stratification is suggested for younger patients with high grade (III-IV) body/tail tumors due to increased risk of metastatic disease.
JOURNAL OF GASTROINTESTINAL SURGERY
(2021)
Article
Radiology, Nuclear Medicine & Medical Imaging
Luohai Chen, Shaunak Navalkissoor, Ann-Marie Quigley, Gopinath Gnanasegaran, Dalvinder Mandair, Christos Toumpanakis, Martyn E. Caplin, Aimee R. Hayes
Summary: In this study, patients aged 70 and older with advanced NET treated with Lu-177-DOTATATE showed efficacy and toxicity profiles similar to the broader NET population, without HRQoL deterioration.
EUROPEAN JOURNAL OF NUCLEAR MEDICINE AND MOLECULAR IMAGING
(2021)
Article
Medicine, Research & Experimental
Jacques Greenberg, Jessica Limberg, Akanksha Verma, David Kim, Xiang Chen, Yeon J. Lee, Maureen D. Moore, Timothy M. Ullmann, Jessica W. Thiesmeyer, Zachary Loewenstein, Kevin J. Chen, Caitlin E. Egan, Dessislava Stefanova, Rohan Bareja, Rasa Zarnegar, Brendan M. Finnerty, Theresa Scognamiglio, Yi-Chieh Nancy Du, Olivier Elemento, Thomas J. Fahey III, Irene M. Min
Summary: This study investigates the therapeutic options for metastatic pancreatic neuroendocrine tumors (PNETs). The results suggest that remodeling the tumor microenvironment (TME) may enhance susceptibility to immunotherapy. The TMEs of localized and metastatic PNETs were analyzed, revealing activation of inflammatory and immune-related pathways in metastatic tumors. Additionally, a histone deacetylase inhibitor, vorinostat, was shown to increase chemokine expression and enhance recruitment of T cells in PNET tissues.
Article
Endocrinology & Metabolism
Dirk-Jan van Beek, Carolina R. C. Pieterman, Frank J. Wessels, Annenienke C. van de Ven, Wouter W. de Herder, Olaf M. Dekkers, Wouter T. Zandee, Madeleine L. Drent, Peter H. Bisschop, Bas Havekes, Inne H. M. Borel Rinkes, Menno R. Vriens, Gerlof D. Valk
Summary: This study aimed to assess the diagnostic accuracy of conventional pancreatic imaging techniques and the added value of fine needle aspirations (FNA) in diagnosing MEN1-related pancreatic neuroendocrine tumors (PanNETs). MRI was found to have higher accuracy than CT in diagnosing PanNETs, making it the preferred non-invasive imaging modality. The high diagnostic accuracy of pancreatic imaging and the sporadic occurrence of pancreatic adenocarcinoma raise questions about the need for routine FNA.
FRONTIERS IN ENDOCRINOLOGY
(2022)
Article
Gastroenterology & Hepatology
Oscar Thompson, Lewis Hall, Keith Roberts, Elizabeth Bradley, Sarah Powell-Brett, Rupaly Pande, Tahir Shah
Summary: Pancreatic enzyme replacement therapy (PERT) may improve survival and nutrition in patients with pancreatic neuroendocrine tumors (pNETs) treated with somatostatin analogues (SSAs) or pancreaticoduodenectomy. However, further multicenter studies are needed to confirm the benefits due to the limited number of patients in this study.
Article
Oncology
Peeter Karihtala, Katja Porvari, Nelli Roininen, Sari Voutilainen, Johanna Mattson, Paivi Heikkila, Kirsi-Maria Haapasaari, Katri Selander
Summary: The pathophysiology and optimal treatment of breast neuroendocrine tumors (NETs) are still unknown. This study compared the mutational profiles of breast NETs with those of invasive ductal carcinoma (IDC) and pancreatic NET (PNET) cases. The results showed that breast NETs have similar mutational profiles to IDCs and have distinct differences from PNETs. Breast NETs have clock-like mutational signatures and signatures associated with defective DNA mismatch repair. This study suggests that breast NETs are a unique subtype of breast cancer, but more research is needed to determine the optimal treatment.
Article
Medicine, General & Internal
He Tong, Miaomiao Wang, Jingjing Liu, Chuangen Guo, Zhongqiu Wang, Jianhua Wang, Xiao Chen
Summary: This study investigated the prevalence and associated factors of bone loss in patients with pancreatic neuroendocrine tumors (PNETs). The results showed a high prevalence of osteoporosis and low bone mass in PNET patients, with age and diabetes being associated factors for bone loss.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Medicine, General & Internal
Martin A. Walter, Cedric Nesti, Marko Spanjol, Attila Kollar, Lukas Buetikofer, Viktoria L. Gloy, Rebecca A. Dumont, Christian A. Seiler, Emanuel R. Christ, Piotr Radojewski, Matthias Briel, Reto M. Kaderli
Summary: The study evaluates the safety and efficacy of therapies for neuroendocrine tumors (NETs) through a network meta-analysis, providing estimates of relative efficiency of different treatment options. Combination therapies, including somatostatin analogues, were found to be generally superior according to the results.
COCHRANE DATABASE OF SYSTEMATIC REVIEWS
(2021)
Article
Oncology
Olof Pettersson, Katarzyna Fross-Baron, Joakim Crona, Anders Sundin
FRONTIERS IN ONCOLOGY
(2020)
Article
Endocrinology & Metabolism
Clarisse Dromain, Anders Sundin, Pavan Najran, Hector Vidal Trueba, Marco Dioguardi Burgio, Joakim Crona, Marta Opalinska, Luciana Carvalho, Regis Franca, Philip Borg, Naik Vietti Violi, Niklaus Schaefer, Carlos Lopez, Daniela Pezzutti, Louis de Mestier, Angela Lamarca, Frederico Costa, Marianne Pavel, Maxime Ronot
Summary: TGR(3m) as an early radiological biomarker, can predict progression-free survival in neuroendocrine tumor patients, showing good performance in both univariable and multivariable analysis. It can help identify patients who require closer radiological follow-up.
NEUROENDOCRINOLOGY
(2021)
Article
Oncology
Abhishek Jha, David Taieb, Jorge A. Carrasquillo, Daniel A. Pryma, Mayank Patel, Corina Millo, Wouter W. de Herder, Jaydira Del Rivero, Joakim Crona, Barry L. Shulkin, Irene Virgolini, Alice P. Chen, Bhagwant R. Mittal, Sandip Basu, Joseph S. Dillon, Thomas A. Hope, Carina Mari Aparici, Andrei H. Iagaru, Rodney J. Hicks, Anca M. Avram, Jonathan R. Strosberg, Ali Cahid Civelek, Frank I. Lin, Neeta Pandit-Taskar, Karel Pacak
Summary: This study aims to summarize the survival outcomes of available TRTs, discuss personalized treatment strategies based on functional imaging scans, address practical issues including regulatory approvals, and compare toxicities and risk factors across different treatments.
CLINICAL CANCER RESEARCH
(2021)
Review
Endocrinology & Metabolism
Joakim Crona, Eric Baudin, Massimo Terzolo, Alexandra Chrisoulidou, Anna Angelousi, Cristina L. Ronchi, Cristina Lamas Oliveira, Els J. M. Nieveen van Dijkum, Filippo Ceccato, Francoise Borson-Chazot, Giuseppe Reimondo, Guido A. M. Tiberi, Hester Ettaieb, Andreas Kiriakopoulos, Canu Letizia, Darko Kastelan, Esthr Osher, Eugenia Yiannakopoulou, Giorgio Arnaldi, Guillaume Assie, Isabel Paiva, Isabelle Bourdeau, John Newell-Price, Karolina M. Nowak, M. Tous Romero, Maria Cristina De Martino, Maria Joao Bugalho, Mark Sherlock, Marie-Christine Vantyghem, Michael Conall Dennedy, Paula Loli, Patrice Rodien, Richard Feelders, Ronald de Krijger, Sam Van Slycke, Simon Aylwin, Valentina Morelli, Laurent Vroonen, Zulfiya Shafigullina, Irina Bancos, Malgorzata Trofimiuk-Mueldner, Marcus Quinkler, Michaela Luconi, Matthias Kroiss, Mitsuhide Naruse, Peter Igaz, Radu Mihai, Silvia Della Casa, Alfredo Berruti, Martin Fassnacht, Felix Beuschlein
Summary: Adrenocortical carcinoma is a rare disease with limited treatment options. Registry-based clinical trials are seen as a cost-effective approach with high scientific potential. ENSAT is considering transforming their current registry into a clinical trial infrastructure to conduct randomized registry-based trials for ACC patients.
EUROPEAN JOURNAL OF ENDOCRINOLOGY
(2021)
Article
Endocrinology & Metabolism
Olof Joakim Pettersson, Katarzyna Fross-Baron, Joakim Crona, Anders Sundin
Summary: In PanNET patients undergoing PRRT with Lu-177-DOTATATE, the tumor growth rate (TGR) decreases significantly during the treatment. TGR may serve as a useful biomarker for identifying patients with the shortest progression-free survival (PFS).
ENDOCRINE CONNECTIONS
(2021)
Review
Oncology
Kazhan Mollazadegan, Staffan Welin, Joakim Crona
Summary: Treatment recommendations for advanced gastroenteropancreatic neuroendocrine carcinomas (GEP-NEC) are based on mainly uncontrolled retrospective data. First-line treatment usually involves platinum-based chemotherapy, but progression is inevitable for most patients and effective second-line treatment options are limited. Improved understanding of GEP-NEC biology may lead to more effective interventions.
CURRENT TREATMENT OPTIONS IN ONCOLOGY
(2021)
Review
Oncology
Mohammed Dawod, Teresa Alonso Gordoa, Mauro Cives, Louis De Mestier, Joakim Crona, Francesca Spada, Kjel Oberg, Marianne Pavel, Angela Lamarca
Summary: Neuroendocrine neoplasms are a group of malignancies with rising incidence and prevalence, where the outcome and therapy for small bowel neuroendocrine tumors depend on various factors, necessitating a multidisciplinary approach. Recent advances in treatment options for SBNETs have been significant, but there are still unresolved questions in this field.
CURRENT TREATMENT OPTIONS IN ONCOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Karin Saljo, Anders Thornell, Chunsheng Jin, Peter Stalberg, Olov Norlen, Susann Teneberg
Summary: The glycosphingolipid patterns of the human parathyroid and thyroid glands were very similar, with the major acid glycosphingolipids being sulfatide and gangliosides. Complex glycosphingolipids with terminal blood group determinants were found in both tissues. The glycosylation of human thyroid and parathyroid glands is more complex than previously appreciated, providing a platform for further studies of alterations in cancers.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Surgery
J. Kjaer, P. Stalberg, J. Crona, S. Welin, P. Hellman, A. Thornell, O. Norlen
Summary: This retrospective study evaluated the effects of liver surgery and thermal hepatic ablation (THA) on pancreatic neuroendocrine tumors (Pan-NETs) with liver metastases, showing that patients treated with these interventions had significantly higher five-year survival rates and longer median survival times compared to the control group. Surgery or THA was associated with a significant decrease in death rate.
Article
Endocrinology & Metabolism
Kazhan Mollazadegan, Britt Skogseid, Johan Botling, Tobias Akerstrom, Barbro Eriksson, Staffan Welin, Anders Sundin, Joakim Crona
Summary: This retrospective study investigated the treatment patterns and outcomes for patients with secondary panNET-G3. The results found that both platinum-etoposide chemotherapy and conventional panNET therapies had relatively short median overall survival and progression-free survival. The study highlights the challenges in treating this type of tumor.
ENDOCRINE CONNECTIONS
(2022)
Article
Surgery
Matilda Anneback, Edward McHale Sjodin, Per Hellman, Peter Stalberg, Olov Norlen
Summary: This retrospective cohort study investigated the potential benefit of preoperative active vitamin D supplementation on hypocalcaemia and its symptoms after thyroidectomy. The study found that preoperative alfacalcidol treatment was associated with reduced biochemical hypocalcaemia and duration of hospital stay.
Review
Endocrinology & Metabolism
Katharina Wang, Joakim Crona, Felix Beuschlein, Ashley B. Grossman, Karel Pacak, Svenja Noelting
Summary: Molecular targeted therapy plays an important role in the treatment of metastatic pheochromocytomas and paragangliomas. Some therapies are already in use with promising results, while others are still under evaluation in clinical trials. The development of molecular targeted therapies is of great significance for future tumor treatment.
JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM
(2022)
Article
Surgery
Josefine Kjaer, Sara Smith, Per Hellman, Peter Stalberg, Joakim Crona, Staffan Welin, Olov Norlen
Summary: This study aimed to determine the survival rates of patients with stage IV neuroendocrine pancreatic tumors who met the criteria for liver transplantation. The results showed a 5-year survival rate of 64.7% for stage IV pan-NET patients, and the survival rates for patients meeting the Milan, ENETS, and UNOS criteria were 64.9%, 85.7%, and 55.4% respectively.
WORLD JOURNAL OF SURGERY
(2023)
Article
Endocrinology & Metabolism
Suman Ghosal, Katerina Hadrava Vanova, Ondrej Uher, Shaoli Das, Mayank Patel, Leah Meuter, Thanh-Truc Huynh, Abhishek Jha, Sara Talvacchio, Marianne Knue, Tamara Prodanov, Martha A. Zeiger, Naris Nilubol, David Taieb, Joakim Crona, Uma T. Shankavaram, Karel Pacak
Summary: In this study, we analyzed transcriptomic data from NENs to identify immune cell infiltration signatures, particularly in PCPG. We validated the immune signature of PCPG using an independent cohort. We identified five dominant immune clusters in PCPG, associated with different immune cell types. Furthermore, we developed a prognostic immune score model that could predict non-aggressive and non-metastatic diseases.
Meeting Abstract
Oncology
Tahsin M. Khan, Elizabeth A. Hedges, Alexander Rossi, Tamara Prodanov, Henri Timmers, Joakim Crona, Tobias Akerstrom, Karel Pacak, Naris Nilubol
ANNALS OF SURGICAL ONCOLOGY
(2022)