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Cardiovascular Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
TV disease (tricuspid stenosis or tricuspid regurgitation) is rare in isolation and is usually a result of rheumatic fever in which case left-sided valve disease is usually present. Carcinoid syndrome is another recognized cause. Tricuspid regurgitation is most commonly caused by dilatation of the TV ring as a consequence of RV dilatation brought about by pulmonary hypertension. Tricuspid regurgitation occurs in Ebstein's syndrome when there is congenital displacement of the valve into the RV.
Case 14
Published in Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta, Clinical Cases, 2021
Andrew Solomon, Julia Anstey, Liora Wittner, Priti Dutta
Carcinoid tumoursFlushing (especially facial)Tachycardia and palpitationsBreathlessness and wheezeDiarrhoea, abdominal pain and anorexia
Pediatric Central Nervous System Tumors as Phenotypic Manifestation of Cancer Predisposition Syndromes
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Giorgio Perilongo, Irene Toldo, Stefano Sartori
Prolactin-producing pituitary adenoma, which occurs very rarely in children, may herald the presence of multiple endocrine neoplasia type 1 (MEN1: Wermer syndrome). MEN1 is an autosomal inherited condition predisposing to tumors of the pancreas, pituitary, and parathyroid glands as well as carcinoid tumors and gastrinoma. The disease is linked to a germline mutation in the tumor suppressor gene MEN1 located on the long arm of chromosome 11, encoding a protein called menin.73
Relationship between Primary Tumor Resection for Metastatic Small Intestine Neuroendocrine Tumors and Survival: A Propensity Score-Matched Analysis
Published in Journal of Investigative Surgery, 2022
Haihao Yan, Linlin Yin, Hao Han, Ye Jin, Zheng Liu
PTR is widely used in SI-NETs patients with tumor load symptoms in the common world. As recorded in the SEER database, more than 70% of metastatic SI-NETs patients have received PTR. The 2020 ESMO Clinical practice guidelines, based on the results of the Swedish study [19], recommend only those patients with symptoms associated with tumor load to undergo tumor debulking surgery at the primary site [14]. However, the manifestations of tumor load include carcinoid syndromes such as flushing and diarrhea and intestinal obstruction and colic caused by mesenteric fibrosis. At present, SSA treatment has been proved to control the symptoms of carcinoid syndrome and delay the progression of the disease to avoid surgery and related risks. Therefore, combined with the guidelines and the results of our study after PSM, we believe that carcinoid syndrome is not suitable for active surgical treatment. In the absence of randomized controlled trials, PTR should only be used in related manifestations such as intestinal obstruction or intestinal ischemia caused by primary tumor and surrounding fibrosis that SAA cannot alleviate, consistent with the 2018 NCCN clinical practice guidelines [21]. However, a fact that cannot be ignored is that in our study, the survival of patients in the unmatched cohort was significantly improved after receiving PTR. Even if this may be because surgeons prefer better-performed patients with fewer complications, resulting in higher survival rates. Nevertheless, it also means that properly selected patients can benefit well from PTR.
Ocular adnexal manifestations of neuroendocrine neoplasms: a case report and a major review
Published in Orbit, 2021
Alexander J. Hatsis, Roger K. Henry, Mark T. Curtis, Jurij R. Bilyk, Meera D. Sivalingam, Ralph C. Eagle, Tatyana Milman
The majority of OA NENs (88/94, 94%) were metastases, most commonly from gastrointestinal (GI)-hepatopancreatobiliary (52/88, 59%) tract with predilection for upper GI system (37/88, 42%),10,11,14,15,26,28–35, 40,43,45,46,49,54 followed by lower GI system(12/88, 14%),9,15,23,26,33,47,48 and least frequently hepatopancreatobiliary (3/88, 3%) tract (Tables 1 and 3).11,13 OA NEN metastasis presented following detection of the primary tumor in 73/94 (78%) patients with a median time to metastasis of 24 months (range 0–288 months) (Table 1). Ten NENs (11%) presented with 10-year or longer delayed metastasis to OA, most commonly from the ileum (6/10, 60%) and less frequently from the large intestine (2/10, 20%), lung (1/10, 10%), and testicle (1/10, 10%).9,10,23,29,43,46,48 In 15 (16%) patients, OA NENs presented as the initial manifestation of metastatic disease with a median time to primary tumor detection of 18 months (range 1–108 months).16,18,21,24,26,27,37–42,44, 49,51–53 In two of these patients (2/94, 2%), primary NEN was identified in the ileum and lung, 6 and 9 years after the diagnosis of OA NEN, respectively (Table 1). In 72 patients with detailed information on systemic work-up, the most common extra-OA metastasis site was liver (41/72, 57%).9–15,19,20,22–24,26,28,36,42,44–46 Symptoms of carcinoid syndrome were documented in 28 of 51 (55%) patients (Table 3).10,11,15,20,23,24,26,28–30,32–35,38,44–46,48,49,52
Carcinoid Heart Disease
Published in Structural Heart, 2020
Amin Sabet, Mina Haghighiabyaneh, Chirag Rajyaguru, Ajit Raisinghani, Daniel Kupsky, Anthony N. DeMaria
Carcinoid tumors are rare neuroendocrine malignancies, occurring primarily in the gastrointestinal tract and secondarily in the bronchopulmonary system and gonads.1 They usually grow slowly over years and cause no or minimal symptoms until they have grown to a large size or metastasized. Approximately 40% of patients present with the classic features of the carcinoid syndrome; a symptom complex caused by tumor production of vasoactive substances that are inactivated by the liver. Therefore, carcinoid syndrome usually occurs when the tumor metastasizes to the liver. Clinically, the syndrome is usually manifested by episodes of flushing, hypotension, secretory diarrhea, bronchospasm and less frequently hypertension.2,3 Carcinoid heart disease (CHD), first reported in 1954, develops in more than 50% of patients with the carcinoid syndrome.4–6 It is associated with fibrous plaque–like endocardial thickening that can cause retraction and fixation of the right-sided heart valves. The pathophysiology of carcinoid heart disease is thought to be multifactorial and caused by chronic exposure to excessive circulating vasoactive substances.5–9 The prognosis of patients with CHD treated medically is poor, and surgical mortality is also high.10–13