Moderately Differentiated Neuroendocrine Carcinoma of the Larynx: A Case Report and Review of the Literatures

喉部的中度分化神經內分泌癌（過去曾被稱之為非典型類癌）是一罕見的疾病，臨床多以聲音沙啞與吞嚥困難為表現。此疾病常有淋巴及血管浸潤，一旦擴散至頸部淋巴節往往視為不良預後，隨之容易有遠端轉移而具高致死率。手術切除為多數人採取的治療方式，至於其他治療則因病歷數少而無法有明確的共識。但多數文獻認為此神經內分泌癌為一侵犯性強的腫瘤，積極的治療決策，例如術後放射線治療，可能可以增加病人存活。積極的治療決策有賴於正確診斷此病，然而此病在病理切片下不容易與甲狀腺髓質癌、副神經節瘤做區分，必須藉由特殊染色協助確診。文獻回顧發現：過去由於喉部神經內分泌癌分類命名法紊亂，造成早期文獻所指的「非典型類癌」與現在認為之「中度分化神經分泌癌」可能不是同種疾病，此點在文獻回顧與分析必須相當小心。本文將報告一位59歲男性，在確診為喉部中度分化神經內分泌癌後接受手術治療與術後放射線治療，經過19個月追蹤並無任何復發證據。茲後文獻回顧，將探討此病之分類命名、臨床表現、病理特徵、治療預後與放射線治療所扮演的角色。

關鍵字

神經內分泌癌；非典型類癌；喉癌；放射線治療

並列摘要

The moderately differentiated neuroendocrine carcinomas (MDNC) are rare tumors in the larynx. Most patients present with hoarseness and dysphagia. Commonly, these tumors have vascular or lymphatic invasion. High mitotic rate, necrosis and nuclear pleomorphism are also frequent. Once patients develop regional or distal metastasis, they usually had high mortality with poor prognosis. Unfortunately, these tumors have high tendency of regional or distal metastases, so MDNC is considered as an aggressive tumor in the larynx. Early diagnosis determines the treatment plan and outcome. Surgical removal is generally advised as the treatment of choice, and radiation therapy may have a benefit in survival. We present a 59-year-old non-smoking man with MDNC of the left supraglottic larynx, cT1N0M0, stage Ⅰ. The patient received surgical excision (pT1N0M0, stage Ⅰ) and post-operative radiation therapy (6300 cGy/35 frs). There was no evidence of recurrence for 19 months. Neither dysphasia nor hoarseness was complained. Currently, he had regular follow-up in the out-patient department.

並列關鍵字

Atypical carcinoid ； Moderately differentiated neuroendocrine carcinomas ； Larynx tumors ； Radiation therapy

國際替代計量

Moderately Differentiated Neuroendocrine Carcinoma of the Larynx: A Case Report and Review of the Literatures

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