Apostolidis Leonidas, Schrader Jörg, Jann Henning, Rinke Anja, Krug Sebastian
Department of Medical Oncology, National Center for Tumor Diseases, University Hospital Heidelberg, 69120 Heidelberg, Germany.
I. Medical Department-Gastroenterology and Hepatology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.
Biology (Basel). 2021 Mar 28;10(4):277. doi: 10.3390/biology10040277.
Central nervous system (CNS) involvement by paraneoplastic syndromes, brain metastases, or leptomeningeal carcinomatosis (LC) in patients with neuroendocrine neoplasms (NEN) has only been described in individual case reports. We evaluated patients with LC in four neuroendocrine tumor (NET) centers (Halle/Saale, Hamburg, Heidelberg, and Marburg) and characterized them clinically. In the study, 17 patients with a LC were defined with respect to diagnosis, clinic, and therapy. The prognosis of a LC is very poor, with 10 months in median overall survival (mOS). This is reflected by an even worse course in neuroendocrine carcinoma (NEC) G3 Ki-67 >55%, with a mOS of 2 months. Motor and sensory deficits together with vigilance abnormalities were common symptoms. In most cases, targeted radiation or temozolomide therapy was used against the LC. LC appears to be similarly devastating to brain metastases in NEN patients. Therefore, the indication for CNS imaging should be discussed in certain cases.
神经内分泌肿瘤（NEN）患者出现副肿瘤综合征、脑转移或软脑膜癌病（LC）累及中枢神经系统（CNS）的情况仅在个别病例报告中有描述。我们评估了四个神经内分泌肿瘤（NET）中心（哈雷/萨勒、汉堡、海德堡和马尔堡）的LC患者，并对其进行了临床特征分析。在该研究中，17例LC患者在诊断、临床和治疗方面得到了明确界定。LC的预后非常差，中位总生存期（mOS）为10个月。神经内分泌癌（NEC）G3 Ki-67>55%的患者病程更差，mOS为2个月。运动和感觉障碍以及警觉异常是常见症状。在大多数情况下，针对LC采用了靶向放疗或替莫唑胺治疗。LC对NEN患者脑转移的破坏性似乎相似。因此，在某些情况下应讨论进行中枢神经系统成像检查的指征。
