Superior Vena Cava Syndrome in Men with Thymic Atypical Carcinoid (Neuroendocrine Tumor Grade 2)

Nora Ramkita, Suly Auline Rusminan, Ika Kartika, Krisna Murti, S. Nurul Amanah Ratna Sari, Devi Eqtriana Setyaningsih
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Abstract

Introduction: Cases of neuroendocrine tumors (NET) can occur in various organs, generally in the form of gastroenteropancreatic (GEP-NET) and pulmonary tract (PNET). The incidence of thymic neuroendocrine tumors (TNET) is rare, accounting for less than 5% of thymic and mediastinal malignancies, and 0.4% of all neuroendocrine tumors. Case presentation: A 57-year-old man came to RSUP Dr. Mohammad Hoesin Palembang with complaints of coughing, bluish and curved blood vessels in the chest, and shortness of breath during activities that have been getting worse for six months. CT scan showed a tumor measuring 18.6 x 11.7 x 11 cm in the anterior mediastinum and pressing on the superior vena cava and abdominal aorta. TTNA and core biopsy with CT guidance showed NET Grade 2, mitoses 6/2 mm2, positive tumor cells with immunohistochemistry for synaptophysin, chromogranin, CD 56, AE1/AE3, and Ki67 9%. The patient received paclitaxel and carboplatin chemotherapy for 6 cycles. Conclusion: The prognosis for atypical carcinoid patients is 20-80% for a 5-year survival rate. Appropriate patient management is needed to improve the outcome of patients with primary thymic atypical carcinoid (NET, grade 2).
胸腺非典型类癌(2 级神经内分泌肿瘤)男性患者的上腔静脉综合征
导言:神经内分泌肿瘤(NET)的病例可发生在多个器官,一般表现为胃肠胰腺肿瘤(GEP-NET)和肺部肿瘤(PNET)。胸腺神经内分泌肿瘤(TNET)的发病率很低,占胸腺和纵隔恶性肿瘤的不到5%,占所有神经内分泌肿瘤的0.4%。病例介绍:一名 57 岁的男子因咳嗽、胸部血管呈蓝色和弯曲状、活动时气短等主诉来到巴伦邦 Mohammad Hoesin 医生的 RSUP 就诊,症状已持续 6 个月。CT 扫描显示,前纵隔有一个 18.6 x 11.7 x 11 厘米的肿瘤,压迫上腔静脉和腹主动脉。在CT引导下进行的TTNA和核心活检显示,NET 2级,有丝分裂6/2 mm2,肿瘤细胞免疫组化突触素、嗜铬粒蛋白、CD 56、AE1/AE3阳性,Ki67 9%。患者接受了紫杉醇和卡铂化疗,共 6 个周期。结论非典型类癌患者的预后为5年生存率20%-80%。要改善原发性胸腺非典型类癌(NET,2级)患者的预后,需要对患者进行适当的管理。
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