Toro C, Rinaldo A, Silver C E, Politi M, Ferlito A
Department of Surgical Sciences, Maxillofacial Surgery Clinic, University of Udine, Italy.
Auris Nasus Larynx. 2009 Oct;36(5):513-20. doi: 10.1016/j.anl.2008.10.006. Epub 2008 Dec 27.
Paraneoplastic syndromes (PNS) represent the clinical manifestation of the remote and indirect effects produced by tumor metabolites or other products. Paraneoplastic effects are not directly mediated by tumor invasion of normal tissue, or by the disruption of normal function of the involved organ, or by distant metastases. More than 260 cases of nasopharyngeal carcinoma (NPC) associated with PNS have been reported in the literature. These syndromes can be divided into six main groups: cutaneous or dermatologic, endocrine, hematologic, osteoarticular or rheumatologic, neurologic, and ocular. The most common dermatologic manifestation is dermatomyositis, while the syndrome of inappropriate secretion of antidiuretic hormone and occasionally Cushing's syndrome due to ectopic ACTH production are the endocrinologic manifestations. Tumor fever and leukemoid reaction, osteoarticular or rheumatic syndromes, including clubbing of the fingers and toes, sensory neuropathy and demyelinating motor polyneuropathy, and rarely optic neuritis represent the most prominent examples of the other groups of syndromes. PNS may occur before the NPC is manifest, or while it is in an occult stage, and thus the possibility of NPC should be considered in patients with these various disorders. While some PNS will respond to direct treatment, most often the PNS subsides in parallel to response of the NPC, and thus may be useful for monitoring tumor response or recurrence.
副肿瘤综合征(PNS)是由肿瘤代谢产物或其他产物产生的远程和间接效应的临床表现。副肿瘤效应不是由肿瘤侵袭正常组织、或由受累器官正常功能的破坏、或由远处转移直接介导的。文献中已报道了260多例与PNS相关的鼻咽癌(NPC)病例。这些综合征可分为六大类:皮肤或皮肤病学、内分泌、血液学、骨关节或风湿病学、神经学和眼科。最常见的皮肤病学表现是皮肌炎,而抗利尿激素分泌不当综合征以及偶尔因异位促肾上腺皮质激素分泌导致的库欣综合征是内分泌学表现。肿瘤热和类白血病反应、骨关节或风湿综合征,包括手指和脚趾杵状指、感觉神经病变和脱髓鞘运动性多神经病,以及罕见的视神经炎是其他综合征组中最突出的例子。PNS可能在NPC显现之前出现,或在其处于隐匿阶段时出现,因此患有这些各种疾病的患者应考虑NPC的可能性。虽然一些PNS会对直接治疗有反应,但大多数情况下,PNS会与NPC的反应同时消退,因此可能有助于监测肿瘤反应或复发。