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远不止于特鲁索综合征。胰腺副肿瘤综合征的广泛谱系。

Much More than Trousseau Syndrome. The Broad Spectrum of the Pancreatic Paraneoplastic Syndromes.

作者信息

Zalatnai Attila, Perjési Eszter, Galambos Eszter

机构信息

First Department of Pathology and Experimental Cancer Research, Faculty of Medicine, Semmelweis University, H-1085 Üllői út 26, Budapest, Hungary.

出版信息

Pathol Oncol Res. 2018 Jan;24(1):1-10. doi: 10.1007/s12253-017-0206-6. Epub 2017 Feb 3.

DOI:10.1007/s12253-017-0206-6
PMID:28160197
Abstract

When 150 years ago Armand Trousseau proposed that some thrombotic events might be the first sign of concealed visceral malignancies, these findings seemed to be just of anecdotal interest. Since then, however, we have learned that adenocarcinomas, including pancreatic cancers could be associated with a wide spectrum of paraneoplastic syndromes. They may precede the detection of the tumor, may occur simultaneously or may develop during its progression. Due to various hematologic, endocrine, cutaneous, articular, neuromuscular, renal or even psychiatric syndromes, their correct interpretation is intriguing, and because their early signs are not necessarily recognized first by oncologists, the paraneoplastic syndromes pose a diagnostic challenge. Unfortunately, we cannot generalize about their mechanisms, because the molecular backgrounds are far-reaching. In most of the cases, the pancreatic cancer cells release various factors into the bloodstream triggering the coagulation cascade. These patients frequently present with venous thromboembolism, and sometimes they are resistant to anticoagulation. The simultaneous thrombotic and bleeding evens do reflect the abnormal hemostasis. In other instances autoantibodies are formed against cutaneous, renal, neuromuscular or nervous tissues, but the mechanism of some syndromes remains unclear. Clinicians should be aware that pancreatic carcinoma may be associated with not just the Trousseau-syndrome.

摘要

150年前,阿尔芒·特鲁索提出某些血栓形成事件可能是隐匿性内脏恶性肿瘤的首发症状,当时这些发现似乎仅具有轶事般的趣味性。然而,从那时起,我们了解到包括胰腺癌在内的腺癌可能与多种副肿瘤综合征相关。它们可能在肿瘤被发现之前出现,可能与肿瘤同时发生,也可能在肿瘤进展过程中出现。由于存在各种血液学、内分泌、皮肤、关节、神经肌肉、肾脏甚至精神方面的综合征,对它们的正确解读颇具挑战性,而且由于其早期症状不一定首先被肿瘤学家识别,副肿瘤综合征带来了诊断难题。不幸的是,我们无法对其机制进行一概而论,因为分子背景极为复杂。在大多数情况下,胰腺癌细胞会向血液中释放各种因子,触发凝血级联反应。这些患者常出现静脉血栓栓塞,有时对抗凝治疗有抵抗性。同时出现的血栓形成和出血事件确实反映了异常的止血情况。在其他情况下,会形成针对皮肤、肾脏、神经肌肉或神经组织的自身抗体,但有些综合征的机制仍不清楚。临床医生应意识到胰腺癌可能不仅与特鲁索综合征相关。

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