聲門上大細胞神經內分泌癌

Supraglottic Large Cell Neuroendocrine Carcinoma

呂存敏(Tsun-Min Lu) ； 呂宜興(Yi-Shing Leu) ； 陳冀寬(Chi-Kuan Chen)

《台灣耳鼻喉頭頸外科雜誌》 50卷2期 (2015/06) Pp. 132-139

https://doi.org/10.6286/2015.50.2.132

摘要

喉部非典型類癌病（LAC, Laryngeal atypical carcinoids）在喉癌中為惡性度較高的族群，但其次分類中的大細胞神經內分泌喉癌之病程進展尤為快速，近來在文獻探討上，傾向將此類病理特徵符合WHO定義中「大細胞神經內分泌肺癌」的喉癌獨立於喉部非典型類癌病外，稱之大細胞神經內分泌喉癌，其病程類似小細胞癌，預後較喉部非典型類癌病差，其細胞分裂活性與組織壞死亦較喉部非典型類癌病來的明顯，在CD56、嗜鉻素A以及突觸素3項免疫組織化學染色中，絕大多數病例中至少有兩項染色呈陽性。1名60歲男性因聲音沙啞及右頸腫塊長達1個月而就診，喉內視鏡發現一聲門上腫瘤，頸部電腦斷層顯示此聲門上腫瘤伴有右側頸部多顆淋巴結腫大，腹部超音波和胸部電腦斷層未發現肝或肺轉移，但病人未規則至門診追蹤，直至5個月後才因喘鳴加劇而回診，此時骨頭掃描未發現骨轉移，術前診斷為聲門上癌，cT4aN2bM0，第IVA期，病人接受全喉切除術及右側頸部淋巴結廓清術，病理診斷為聲門上大細胞神經內分泌癌，病人術後8天出現黃疸、肝功能上升與意識變化等情形，腹部超音波顯示有肝轉移，病人於術後15天死亡；若病患能遵從醫囑盡早接受切片，即可早期診斷此惡性度高的喉癌，亦有機會早期治療，以避免進展至癌症末期。

關鍵字

大細胞神經內分泌癌 ； 喉癌 ； 非典型類癌病 ； 嗜鉻素A ； 突觸素

並列摘要

The prognosis of laryngeal atypical carcinoids is relatively poor in laryngeal cancer. However, one sub-classification of laryngeal atypical carcinoids, called large cell neuroendocrine carcinoma is much more malignant. Based on the recent studies, those cancers which fulfill the criteria of ＂pulmonary large cell neuroendocrine carcinoma＂ of WHO are defined as laryngeal large cell neuroendocrine carcinoma and may be recategorized in the poorly-differentiated neuroendocrine carcinoma, such as small cell carcinoma or parallel to it instead of in the laryngeal atypical carcinoids. The clinical course of laryngeal large cell neuroendocrine carcinoma is more aggressive and is similar to small cell carcinoma. In the aspect of immunohistochemical stain, laryngeal large cell neuroendocrine carcinoma is immunopositve for two or more than two neuroendocrine markers (CD56, chromogranin A and synaptophysin). One 60-yearold male sought for medical help for hoarseness lasting for one month. Laryngoscopy showed a supraglottic mass. Neck computed tomography revealed the supraglottic tumor and multiple neck lymphadenopathy. No hepatic or pulmonary metastasis was found according to the results of abdominal sonography and chest computed tomography. Unfortunately, the patient lost follow-up and came back 5 months later due to his worse stridor. Bone scan did not demonstrate bony metastasis. The pre-operative diagnosis was supraglottic cancer, cT4aN2bMo, stage IVA. Total laryngectomy with right radical neck dissection was performed. The pathological diagnosis disclosed the laryngeal large cell neuroendocrine carcinoma. The patient developed jaundice, elevated liver enzymes and conscious changes eight days after the operation. Liver metastasis was noted by abdominal sonography. The patient died 15 days after the operation.

並列關鍵字

large cell neuroendocrine carcinoma ； laryngeal cancer ； laryngeal atypical carcinoid ； chromogranin A ； synaptophysin