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Peptide Receptor Radionuclide Therapy for Neuroendocrine Tumours
Published in Michael Ljungberg, Handbook of Nuclear Medicine and Molecular Imaging for Physicists, 2022
Anna Sundlöv, Katarina Sjögreen Gleisner
NETs that secrete one or more hormones may cause typical hormonal syndromes. The most well-known is the carcinoid syndrome, which is caused by tumoural secretion of vaso-active substances of which the most important is serotonin. The carcinoid syndrome is a triad of flushing (redness and heat in the skin), diarrhea and carcinoid heart disease (fibrosis of the right heart valves causing congestive heart failure). Another very typical, but also very rare, hormonal syndrome is caused by pancreatic insulinomas – NETs that continuously produce insulin – causing life-threatening episodes of hypoglycemia, which worsen as the tumour progresses. Other pancreatic NETs may secrete glucagon or gastrin, among other hormones, each with its specific clinical presentation. Bronchopulmonary NETs sometimes secrete ACTH, which causes the ectopic Cushing syndrome (hyperglycemia, muscle wasting, redistribution of body fat, osteoporosis, etc.) by stimulating the production of cortisol by the adrenal glands.
Non-Obstructing Small Bowel Neuroendocrine Tumor with Liver Metastasis
Published in Savio George Barreto, Shailesh V. Shrikhande, Dilemmas in Abdominal Surgery, 2020
Mufaddal Kazi, Manish S. Bhandare, Vikram A. Chaudhari, Shailesh V. Shrikhande
Patients with advanced and metastatic disease are kept under follow up every three months with serum CgA or 24 hour urine 5-hydroxyindoleacetic acid levels and triphasic CT scans. Follow-up interval can be prolonged for indolent, slow-growing tumors. Somatostatin receptor-based imaging is performed in case of suspected recurrences and before initiating or escalating therapies. Evaluation for carcinoid heart disease is performed by echocardiogram every two or three years. Follow-up continues lifelong, as late recurrences beyond 20 years are also known.
The Carcinoid Tumors
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
Patients with carcinoid syndromes should be carefully studied and probably explored for curative resection of carcinoid tumors outside the GI tract. Hepatic resections are highly recommended not only for curative attempt but also for palliation. Enucleation of liver metastasis and removal of significant tumor bulk may result in amelioration of the symptoms. Hepatic artery infusion and hepatic dearterialization with distal hepatic artery infusion with chemotherapeutic agents are being tried. In addition, patients with carcinoid heart disease may benefit from valve reconstruction or replacement if they are in heart failure.
Peptide receptor radionuclide therapy in neuroendocrine neoplasms and related tumors: from fundamentals to personalization and the newer experimental approaches
Published in Expert Review of Precision Medicine and Drug Development, 2023
Carcinoid Heart Disease: One of the advanced complications, generally seen in high volume functioning disease, typically observed in patients with bulky hepatic involvement. These tumors secrete a large number of vasoactive substances (mainly 5-hydroxytryptamine, tachykinins, and prostaglandins) that reach the right side of the heart through hepatic veins and lead to the deposition of fibrous tissue on the endocardial surfaces of the heart. This results in the pathogenesis of carcinoid heart disease. Though reported incidence is as high as 70% within patients of carcinoid syndrome, its incidence is now much reduced as a result of widespread use of synthetic somatostatin analogues (SSA). Administration of PRRTs (177Lu-DOTATATE) in such patients with resistant disease, helped in substantial reduction of 5-HIAA levels and resulted in symptomatic improvement and improved health-related quality of life (from NYHA grade III at baseline to NYHA grade I after 4–6 cycles) and also enabled taking the patient for corrective valvular surgery [88] which would otherwise have shown high morbidity and mortality.
Serotonin and valvular heart disease
Published in Expert Opinion on Therapeutic Targets, 2022
Helge Waldum, Rune Wiseth, Alexander Wahba
Another rare cause of heart valve disease is small intestinal carcinoid tumors (now called neuroendocrine tumors). Carcinoid valve disease is of great importance because the etiology of the dominantly right-sided valve lesions is linked to serotonin release from the tumor. Small intestine carcinoid valve disease, thus in contrast to most other causes of heart valve diseases, mainly affects the low-pressure side of the heart. Carcinoid heart disease, as a rule, does not develop before the tumor has metastasized to the liver associated with flushing. Rarer carcinoids having the origin outside the portal area may show flushing without metastasis. However, carcinoid heart disease is very rare in patients with carcinoid developing outside the small intestine, possibly because of early symptoms like flushing in such patients allow for diagnosis and surgery at a curable stage.
Fatigue and quality of life in patients with neuroendocrine neoplasia
Published in Scandinavian Journal of Gastroenterology, 2023
Nynne Emilie Hummelshøj, Henning Gronbaek, Palle Bager, Elizaveta Tabaksblat, Gitte Dam
Forty-three percent of the patients had the carcinoid syndrome encompassing either flushing, diarrhea, or carcinoid heart disease. The presence of carcinoid heart disease was extrapolated from the electronic patient file (echocardiography report), and symptoms such as flushing and/or diarrhea were derived from questionnaires as well as recent journal notes regarding patient reported symptoms. HRQoL data for patients with and without the CS are presented in Table 2 and Figure 2. In patients with CS, four out of the five HRQoL parameters; self-care, usual activities, pain/discomfort, and anxiety/depression were significantly worse compared to those without CS. In addition, mobility also tended to be worse (p = .06).