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探索神经内分泌肿瘤中的类癌综合征:多学科叙述性综述的见解

Exploring Carcinoid Syndrome in Neuroendocrine Tumors: Insights from a Multidisciplinary Narrative Review.

作者信息

Marasco Matteo, Romano Elena, Arrivi Giulia, Prosperi Daniela, Rinzivillo Maria, Caruso Damiano, Mercantini Paolo, Rossi Michele, Faggiano Antongiulio, Panzuto Francesco

机构信息

Digestive Disease Unit, Sant' Andrea University Hospital, ENETS Center of Excellence, 00189 Rome, Italy.

PhD School in Translational Medicine and Oncology, Department of Medical and Surgical Sciences and Translational Medicine, Faculty of Medicine and Psychology, Sapienza University of Rome, 00185 Rome, Italy.

出版信息

Cancers (Basel). 2024 Nov 14;16(22):3831. doi: 10.3390/cancers16223831.

Abstract

Carcinoid syndrome (CS) is a rare condition associated with neuroendocrine tumors (NETs), particularly those originating in the gastrointestinal tract, which secrete bioactive substances like serotonin. The management of CS requires a multidisciplinary approach due to its complex clinical manifestations, including flushing, diarrhea, bronchospasm, and carcinoid heart disease. Optimal care involves collaboration between several professional figures like oncologists, endocrinologists, gastroenterologists, surgeons, and dietitians. Currently, a wide range of treatments are available, focused on both symptom control and tumor burden reduction. Somatostatin analogs (SSAs) are the first-line therapy for symptom relief. Still, in patients with progressive disease or refractory CS, other options include targeted therapies, peptide receptor radionuclide therapy (PRRT), liver-directed therapies, and surgical resection, when feasible. Furthermore, management of complications related to prolonged serotonin release and malnutrition as a result of exocrine pancreatic insufficiency, post-surgical conditions, vitamin deficit, and chronic diarrhea often requires early detection to mitigate symptoms and improve the quality of life in these patients. The complexity of CS necessitates individualized care and continuous coordination among specialists to optimize outcomes and enhance patient well-being.

摘要

类癌综合征(CS)是一种与神经内分泌肿瘤(NETs)相关的罕见病症,尤其是起源于胃肠道的那些肿瘤,这些肿瘤会分泌血清素等生物活性物质。由于其复杂的临床表现,包括潮红、腹泻、支气管痉挛和类癌心脏病,CS的管理需要多学科方法。最佳治疗需要肿瘤学家、内分泌学家、胃肠病学家、外科医生和营养师等多个专业人员之间的协作。目前,有多种治疗方法可供选择,重点是症状控制和减轻肿瘤负担。生长抑素类似物(SSAs)是缓解症状的一线治疗方法。然而,对于疾病进展或难治性CS患者,其他选择包括靶向治疗、肽受体放射性核素治疗(PRRT)、肝脏定向治疗以及在可行时进行手术切除。此外,由于外分泌性胰腺功能不全、术后状况、维生素缺乏和慢性腹泻导致的血清素长期释放和营养不良相关并发症的管理通常需要早期检测,以减轻症状并改善这些患者的生活质量。CS的复杂性需要个性化护理以及专家之间的持续协调,以优化治疗效果并提高患者的健康水平。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7e15/11592972/b53370e439e0/cancers-16-03831-g001.jpg

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