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- Kanakis, G., et al.
(författare)
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Unusual Complication of a Pancreatic Neuroendocrine Tumor Presenting with Malignant Hypercalcemia
- 2012
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Ingår i: Journal of Clinical Endocrinology and Metabolism. - : The Endocrine Society. - 0021-972X .- 1945-7197. ; 97:4, s. E627-E631
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Tidskriftsartikel (refereegranskat)abstract
- Context: Hypersecretion of PTHrP is a relatively common cause of malignancy-related hypercalcemia but has only been described in a few cases of neuroendocrine tumors (NET). Objective: The aim of this case report is to describe the clinical syndrome, complex therapeutic interventions, and unusual complications caused by persistent PTHrP hypersecretion in a patient with a pancreatic NET. Case Illustration: A 58-yr-old male patient presented with nonspecific abdominal pain and was found to have severe hypercalcemia secondary to a well-differentiated NET of the pancreas associated with extensive liver metastases. Elevated ionized calcium levels accompanied by low serum PTH and remarkably elevated PTHrP concentrations were consistent with PTHrP-related hypercalcemia that proved to be resistant to various chemotherapeutic regimens and supportive therapy. Partial control of the humoral syndrome was obtained only after the application of cytoreductive interventions and the introduction of various molecular targeted therapies. Due to persistent PTHrP action, bone disease emerged in the form of brown tumors. Discussion: The manifestation of paraneoplastic syndrome due to PTHrP hypersecretion, despite its rareness in NET, should be considered in the differential diagnosis of hypercalcemia in such tumors. Moreover, the appearance of bone lesions in this setting may be in the context of metabolic bone disease and could be misdiagnosed as bone metastases.
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- Karapanagioti, A., et al.
(författare)
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Aberrant expression pattern of circadian clock genes in type 1 gastric neuroendocrine neoplasms compared to ECL-cell hyperplasia
- 2021
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Ingår i: Journal of neuroendocrinology (Print). - : European Neuroendocrine Association. - 0953-8194 .- 1365-2826. ; 33:S1, s. 37-37
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Introduction: There is a continuity of changes from ECL-cell hyperplasia to type 1 gastric neuroendocrine neoplasms (GNEN1) development with important clinical implications. Aim(s): Although the effect of the circadian clock system on neuroendocrine tumorigenesis has been addressed, the role of the peripheral clock system in the transition from ECL-cell hyperplasia to GNEN1 remains to be explored.Materials and methods: Six GNEN1 patients and 10 patients with ECL-cell hyperplasia were included. Blood samples were collected at 8 am, 3pm and 10pm for peripheral blood mononuclear ells (PBMCs) isolation. The mRNA expression of clock-related genes (CLOCK, BMAL1, CRY-1, PER2, ROR-α and REV-ERBβ) were evaluated by real-time quantitative PCR from PBMCs. Results: In GNEN1 patients, BMAL genes where lower expressed at night than early in the morning (p=0.02), whereas patients with ECL-cell hyperplasia expressed lower levels of PER2 and REV-ERBβ (p=0.03 and p=0.05,respectively). In addition, GNEN1 patients expressed lower levels of CLOCK, PER2 and REV-ERBβ in the early evening than in the morning (p=0.04; p=0.03; p=0.05, respectively). When comparing the two groups (GNEN1 vs. ECL-cell hyperplasia) at the three different time points, a marginal increase in CLOCK, PER2 and REV-ERBβ expression early in the morning (p=0.06, 0.02 and 0.07, respectively) along with a marginal increase in REV-ERBβ and BMAL expression in the early evening (p=0.09 and p=0.08, respectively) and a marginal increase in BMAL at night (p=0.09) in GNEN1 patients was observed.Conclusion: Our findings point towards an upregulated expression of clock-related genes in patients with GNEN1 as compared to ECL-cell hyperplasia, suggesting a possible involvement in GNEN1 tumorigenesis that needs to be confirmed in a larger patients group.
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- Wedin, M., 1977-, et al.
(författare)
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Virchow's node metastasis from small intestinal neuroendocrine neoplasms : A bi-center cohort study
- 2022
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Ingår i: Journal of neuroendocrinology (Print). - : John Wiley & Sons. - 0953-8194 .- 1365-2826. ; 34:Suppl. 1, s. 88-88
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- Introduction: Small Intestinal Neuroendocrine Neoplasms (SI-NENs) may rarely metastasize to the left supraclavicular lymph nodes, also known as Virchow’s node metastasis (VM).Aim(s): Data on prevalence, prognostic significance and clinical course of disease for SI-NEN patients with VM is limited.Materials and methods: In this retrospective analysis of 231 SI-NEN patients treated at two tertiary referal centers we found nine patients with VM. We used a control group of 18 age-and sex-matched SI-NEN patients from the same cohort with stage IV disease, but no extrahepatic metastases.Results: VM prevalence was 3.9% (9/231; 5 females, median age at VM diagnosis 65 years). Two patients had G1, 5 G2 tumours and 2 of unspecified grade. Four patients presented with synchronous VM, whereas 3 developed metachronous VM after a median of 24 months (range: 4.8–117.6 months). Hepatic metastases were present in 7 patients, extrahepatic metastases (EM) in 8 (6 para-aortic distant lymph node metastases, 1 lung and 1 pancreatic metastasis) and peritoneal carcinomatosis in 2 patients. There was no difference in best-recorded responses to 1st line treatment according to RECIST 1.1 as well as progression-free (PFS) and overall survival rates (PFS) between patients with VM and those in the control group (Chi-square p=0.516; PFS: 71.7 vs. 106.9 months [95%CI 38.1-175.8]; log-rank p=0.855; OS: 138.6 [95%CI 17.2–260] vs. 109.9 [95%CI 91.7–128] months; log-rank p=0.533).Conclusion: VM is relatively rare in patients with SI-NENs. It is more often encountered in patients withG2 tumors and EM, mainly to distant para-aortic lymph nodes. Its presence does not seem to impact patients’ survival outcomes and treatment responses, when compared to age-and sex-matched patients with stage IV disease.
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- Ambrosini, Valentina, et al.
(författare)
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Consensus on molecular imaging and theranostics in neuroendocrine neoplasms
- 2021
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Ingår i: European Journal of Cancer. - : Elsevier. - 0959-8049 .- 1879-0852. ; 146, s. 56-73
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Forskningsöversikt (refereegranskat)abstract
- Nuclear medicine plays an increasingly important role in the management neuroendocrine neoplasms (NEN). Somatostatin analogue (SSA)-based positron emission tomography/computed tomography (PET/CT) and peptide receptor radionuclide therapy (PRRT) have been used in clinical trials and approved by the European Medicines Agency (EMA) and the US Food and Drug Administration (FDA). European Association of Nuclear Medicine (EANM) Focus 3 performed a multidisciplinary Delphi process to deliver a balanced perspective on molecular imaging and radionuclide therapy in well-differentiated neuroendocrine tumours (NETs). NETs form in cells that interact with the nervous system or in glands that produce hormones. These cells, called neuroendocrine cells, can be found throughout the body, but NETs are most often found in the abdomen, especially in the gastrointestinal tract. These tumours may also be found in the lungs, pancreas and adrenal glands. In addition to being rare, NETs are also complex and may be difficult to diagnose. Most NETs are non-functioning; however, a minority present with symptoms related to hypersecretion of bioactive compounds. NETs often do not cause symptoms early in the disease process. When diagnosed, substantial number of patients are already found to have metastatic disease. Several societies' guidelines address Neuroendocrine neoplasms (NENs) management; however, many issues are still debated, due to both the difficulty in acquiring strong clinical evidence in a rare and heterogeneous disease and the different availability of diagnostic and therapeutic options across countries. EANM Focus 3 reached consensus on employing 68gallium-labelled somatostatin analogue ([68Ga]Ga-DOTA-SSA)-based PET/CT with diagnostic CT or magnetic resonance imaging (MRI) for unknown primary NET detection, metastatic NET, NET staging/restaging, suspected extra-adrenal pheochromocytoma/paraganglioma and suspected paraganglioma. Consensus was reached on employing 18fluorine-fluoro-2-deoxyglucose ([18F]FDG) PET/CT in neuroendocrine carcinoma, G3 NET and in G1-2 NET with mismatched lesions (CT-positive/[68Ga]Ga-DOTA-SSA-negative). Peptide receptor radionuclide therapy (PRRT) was recommended for second line treatment for gastrointestinal NET with [68Ga]Ga-DOTA-SSA uptake in all lesions, in G1/G2 NET at disease progression, and in a subset of G3 NET provided all lesions are positive at [18F]FDG and [68Ga]Ga-DOTA-SSA. PRRT rechallenge may be used for in patients with stable disease for at least 1 year after therapy completion. An international consensus is not only a prelude to a more standardised management across countries but also serves as a guide for the direction to follow when designing new research studies.
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