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Abstract：
BACKGROUND: Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are rare tumors, often diagnosed in an advanced stage when curative treatment is impossible and grueling symptoms related to vasoactive substance release by tumor cells affect patients\' quality of life. Cardiovascular complications of GEP-NENs, primarily tricuspid and pulmonary valve disease, and right-sided heart failure, are the leading cause of death, even compared to metastatic disease.
METHODS: We present a case of a 35-year-old patient with progressive dyspnea, back pain, polyneuropathic leg pain, and nocturnal diarrhea lasting for a decade before the diagnosis of neuroendocrine carcinoma of unknown primary with extensive liver metastases. During the initial presentation, serum biomarkers were not evaluated, and the patient received five cycles of doxorubicin, which he did not tolerate well, so he refused further therapy and was lost to follow-up. After 10 years, he presented to the emergency room with signs and symptoms of right-sided heart failure. Panneuroendocrine markers, serum chromogranin A, and urinary 5-hydroxyindoleacetic acid were extremely elevated (900 ng/mL and 2178 µmol/L), and transabdominal ultrasound confirmed hepatic metastases. Computed tomography (CT) showed liver metastases up to 6 cm in diameter and metastases in mesenteric lymph nodes and pelvis. Furthermore, an Octreoscan showed lesions in the heart, thoracic spine, duodenum, and ascendent colon. A standard transthoracic echocardiogram confirmed findings of carcinoid heart disease. The patient was not a candidate for valve replacement. He started octreotide acetate treatment, and the dose escalated to 80 mg IM monthly. Although biochemical response and symptomatic improvement were noted, the patient died.
CONCLUSIONS: Carcinoid heart disease occurs with carcinoid syndrome related to advanced neuroendocrine tumors, usually with liver metastases, which manifests as right-sided heart valve dysfunction leading to right-sided heart failure. Carcinoid heart disease and tumor burden are major prognostic factors of poor survival. Therefore, they must be actively sought by available biochemical markers and imaging techniques. Moreover, imaging techniques aiding tumor detection and staging, somatostatin receptor positron emission tomography/CT, and CT or magnetic resonance imaging, should be performed at the time of diagnosis and in 3- to 6-mo intervals to determine tumor growth rate and assess the possibility of locoregional therapy and/or palliative surgery. Valve replacement at the onset of symptoms or right ventricular dysfunction may be considered, while any delay can worsen right-sided ventricular failure.
METHODS: We present a case of a 35-year-old patient with progressive dyspnea, back pain, polyneuropathic leg pain, and nocturnal diarrhea lasting for a decade before the diagnosis of neuroendocrine carcinoma of unknown primary with extensive liver metastases. During the initial presentation, serum biomarkers were not evaluated, and the patient received five cycles of doxorubicin, which he did not tolerate well, so he refused further therapy and was lost to follow-up. After 10 years, he presented to the emergency room with signs and symptoms of right-sided heart failure. Panneuroendocrine markers, serum chromogranin A, and urinary 5-hydroxyindoleacetic acid were extremely elevated (900 ng/mL and 2178 µmol/L), and transabdominal ultrasound confirmed hepatic metastases. Computed tomography (CT) showed liver metastases up to 6 cm in diameter and metastases in mesenteric lymph nodes and pelvis. Furthermore, an Octreoscan showed lesions in the heart, thoracic spine, duodenum, and ascendent colon. A standard transthoracic echocardiogram confirmed findings of carcinoid heart disease. The patient was not a candidate for valve replacement. He started octreotide acetate treatment, and the dose escalated to 80 mg IM monthly. Although biochemical response and symptomatic improvement were noted, the patient died.
CONCLUSIONS: Carcinoid heart disease occurs with carcinoid syndrome related to advanced neuroendocrine tumors, usually with liver metastases, which manifests as right-sided heart valve dysfunction leading to right-sided heart failure. Carcinoid heart disease and tumor burden are major prognostic factors of poor survival. Therefore, they must be actively sought by available biochemical markers and imaging techniques. Moreover, imaging techniques aiding tumor detection and staging, somatostatin receptor positron emission tomography/CT, and CT or magnetic resonance imaging, should be performed at the time of diagnosis and in 3- to 6-mo intervals to determine tumor growth rate and assess the possibility of locoregional therapy and/or palliative surgery. Valve replacement at the onset of symptoms or right ventricular dysfunction may be considered, while any delay can worsen right-sided ventricular failure.
摘要：
背景：胃肠胰腺神经内分泌肿瘤（GEP-NENs）是一种罕见的肿瘤，通常在无法进行治愈性治疗的晚期诊断，与肿瘤细胞释放血管活性物质相关的剧烈症状会影响患者的生活质量。GEP-NENs的心血管并发症，主要是三尖瓣和肺动脉瓣疾病，和右侧心力衰竭,是导致死亡的主要原因,甚至与转移性疾病相比。
方法：我们介绍一例35岁的进行性呼吸困难患者，背痛,多神经性腿部疼痛，和夜间腹泻持续十年，直到诊断出未知的原发性神经内分泌癌并伴有广泛的肝转移。在最初的演讲中，未评估血清生物标志物，病人接受了五个周期的阿霉素,他没有很好地容忍，所以他拒绝了进一步的治疗，失去了随访。十年后,他带着右侧心力衰竭的体征和症状出现在急诊室。神经内分泌标志物,血清嗜铬粒蛋白A,和尿中5-羟基吲哚乙酸极度升高（900ng/mL和2178µmol/L），经腹超声证实肝转移。计算机断层扫描（CT）显示直径达6cm的肝转移和肠系膜淋巴结和骨盆的转移。此外，Octreoscan显示心脏有病变,胸椎,十二指肠,和升结肠。标准的经胸超声心动图证实了类癌心脏病的发现。该患者不是瓣膜置换的候选人。他开始用醋酸奥曲肽治疗,剂量每月增加至80mgIM。尽管注意到生化反应和症状改善，病人死了。
结论：类癌心脏病的发生与晚期神经内分泌肿瘤相关的类癌综合征，通常伴有肝转移，表现为右侧心脏瓣膜功能障碍导致右侧心力衰竭。类癌心脏病和肿瘤负荷是患者生存不良的主要预后因素。因此,必须通过可用的生化标志物和成像技术积极寻找它们。此外，辅助肿瘤检测和分期的成像技术，生长抑素受体正电子发射断层扫描/CT,和CT或磁共振成像，应在诊断时和在3至6个月的间隔内进行，以确定肿瘤的生长速率并评估局部治疗和/或姑息性手术的可能性。在出现症状或右心室功能障碍时可考虑进行瓣膜置换，而任何延迟都会使右心室衰竭恶化。
方法：我们介绍一例35岁的进行性呼吸困难患者，背痛,多神经性腿部疼痛，和夜间腹泻持续十年，直到诊断出未知的原发性神经内分泌癌并伴有广泛的肝转移。在最初的演讲中，未评估血清生物标志物，病人接受了五个周期的阿霉素,他没有很好地容忍，所以他拒绝了进一步的治疗，失去了随访。十年后,他带着右侧心力衰竭的体征和症状出现在急诊室。神经内分泌标志物,血清嗜铬粒蛋白A,和尿中5-羟基吲哚乙酸极度升高（900ng/mL和2178µmol/L），经腹超声证实肝转移。计算机断层扫描（CT）显示直径达6cm的肝转移和肠系膜淋巴结和骨盆的转移。此外，Octreoscan显示心脏有病变,胸椎,十二指肠,和升结肠。标准的经胸超声心动图证实了类癌心脏病的发现。该患者不是瓣膜置换的候选人。他开始用醋酸奥曲肽治疗,剂量每月增加至80mgIM。尽管注意到生化反应和症状改善，病人死了。
结论：类癌心脏病的发生与晚期神经内分泌肿瘤相关的类癌综合征，通常伴有肝转移，表现为右侧心脏瓣膜功能障碍导致右侧心力衰竭。类癌心脏病和肿瘤负荷是患者生存不良的主要预后因素。因此,必须通过可用的生化标志物和成像技术积极寻找它们。此外，辅助肿瘤检测和分期的成像技术，生长抑素受体正电子发射断层扫描/CT,和CT或磁共振成像，应在诊断时和在3至6个月的间隔内进行，以确定肿瘤的生长速率并评估局部治疗和/或姑息性手术的可能性。在出现症状或右心室功能障碍时可考虑进行瓣膜置换，而任何延迟都会使右心室衰竭恶化。
