Background: Hypercalcemia is a common paraneoplastic syndrome which can occur in up to 10% of patients with advanced neoplasms. Paraneoplastic parathyroid hormone-related protein (PTHrP) represents the most frequent cause of this syndrome. In neuroendocrine neoplasms (NENs) paraneoplastic hypercalcemia is rare.
Case Series: The present series includes all patients with NENs and paraneoplastic hypercalcemia from four Italian centres: A 40-year-old man was hospitalized for repeated episodes of falls, hyposthenia and drowsiness. Severe hypercalcemia was found. Metastatic pancreatic G2 NEN and PTHrP-related hypercalcemia were diagnosed. The patient started therapy with somatostatin analogs (SSA) and Denosumab. After disease progression peptide receptor radionuclide therapy (PRRT) was started with an objective response associated with PTHrP reduction and normocalcemia. A 45-year-old man was referred for pancreatic G2 NEN. SSA and subsequently everolimus were administered for metastases occurrence. Hypercalcemia occurred and PRRT and Denosumab were started for disease progression with the onset of bone metastases. Despite disease stability after four cycles of PRRT the patient's performance status worsened until death. A 49-year-old woman was hospitalized for psychic slowdown, confusional state, sensory dullness. A severe hypercalcemia, associated with a pancreatic G1 NEN was diagnosed and treated with haemodialysis, bisphosphonates injections and continuous infusion of calcitonin. 1,25-dihydroxyvitamin D was high, PTHrP was undetectable. After surgery serum calcium levels and 1,25-dihydroxyvitamin D were normalized. A 69-year-old man was hospitalized after the onset of shortness of breath and dyspnea, asthenia and weight loss. Computed Tomography (CT) and Ga DOTATOC Positron Emission Tomography (PET)-CT revealed a left pulmonary nodule. Hypercalcemia and markedly elevated PTHrP levels were detected. The histological examination revealed an atypical carcinoid. After surgery, calcium levels were normalized, PTHrP was significantly reduced with an improvement of general conditions.
Conclusion: In our series, paraneoplastic PTHrP-related hypercalcemia occurred in pancreatic NEN and in one bronchial carcinoid representing the third case in the literature. Our case associated with 1,25-dihydroxyvitamin D secretion represents the fourth case in the literature. PTHrP secretion should be considered in NENs' patients with hypercalcemia. Acute treatment should be focused on lowering calcium levels, and long-term control can be achieved by tumor cytoreduction inhibiting PTHrP release.
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http://dx.doi.org/10.3389/fendo.2021.665698 | DOI Listing |
Fam Cancer
January 2025
Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Multiple endocrine neoplasia type 1 (MEN1) syndrome is an autosomal dominant disorder caused by a germline pathogenic variant in the MEN1 tumor suppressor gene. Patients with MEN1 have a high risk for primary hyperparathyroidism (PHPT) with a penetrance of nearly 100%, pituitary adenomas (PitAd) in 40% of patients, and neuroendocrine neoplasms (NEN) of the pancreas (40% of patients), duodenum, lung, and thymus. Increased MEN1-related mortality is mainly related to duodenal-pancreatic and thymic NEN.
View Article and Find Full Text PDFEndocr Oncol
January 2024
Centro de Investigación Biomédica en Red, CIBERONC, ISCIII, Madrid, Spain.
While the role of cancer stem cells (CSCs) in tumorigenesis, chemoresistance, metastasis, and relapse has been extensively studied in solid tumors, such as adenocarcinomas or sarcomas, the same cannot be said for neuroendocrine neoplasms (NENs). While lagging, CSCs have been described in numerous NENs, including gastrointestinal and pancreatic NENs (PanNENs), and they have been found to play critical roles in tumor initiation, progression, and treatment resistance. However, it seems that there is still skepticism regarding the role of CSCs in NENs, even in light of studies that support the CSC model in these tumors and the therapeutic benefits of targeting them.
View Article and Find Full Text PDFJ Surg Case Rep
January 2025
Department of Gastroenterological and Transplant Surgery, Graduate School of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 734-8551, Japan.
Neuroendocrine tumors (NENs) originate from neuroendocrine cells and predominantly occur in the gastrointestinal tract, lungs, and pancreas. Although the liver is commonly involved in NEN metastasis, primary hepatic neuroendocrine tumors (PHNETs) are rare. Herein, we report a case of a 52-year-old female who presented with slowly enlarging, cystic, multiple PHNETs.
View Article and Find Full Text PDFIntroduction: Although neuroendocrine neoplasms (NENs) have a good prognosis, distant metastasis remains a crucial prognostic factor. Survivin, a tumor-associated antigen, is overexpressed in several solid tumors, indicating poor prognosis. We aimed to evaluate the clinical significance and role of survivin as a therapeutic target for NEN.
View Article and Find Full Text PDFEndocrine
December 2024
Εndocrinology Unit, First Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Background: Neuroendocrine neoplasms (NEN) are rare tumors arising from neuroendocrine cells most commonly in the gastrointestinal-tract. In recent years, advancements in therapeutics have increased survival rates in patients with NEN leading to a greater clinical burden compared to the general population.
Methods: The aim of this single-center case-control study was to investigate the incidence of low bone mass and changes in body composition in adult patients diagnosed with gastroenteropancreatic neuroendocrine tumors (GEPNET).
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