Carcinoma Ex Pleomorphic Adenoma-Clinical Experience in Taipei Veteran General Hospital

源自多形性腺瘤之癌—台北榮民總醫院的臨床經驗

陳世翰(Shih-Han Chen) ； 馬旭(Hsu Ma) ； 廖文傑(Wen-Chieh Liao)

《臺灣整形外科醫學會雜誌》 22卷1期 (2013/03) Pp. 20-28

摘要

背景：源自多形性腺瘤之癌（Carcinoma ex pleomorphic adenoma）是一種高惡性度且尚未被完整了解的惡性腫瘤。此腫瘤通常生於唾液腺，與原發或復發的多形性腺瘤具有相關性。而臨床的表現以及其預後，較少有文獻討論。其中更是缺乏亞洲人種的相關數據。目的及目標：藉由台北榮總診治CXPA病人的經驗，了解疾病的表現，並進一步分析診斷及治療上的困難。材料及方法：我們採用回朔性的病例分析，統整了本院2000至2011年，12年期間，總共15個最終病理診斷為CXPA的病人。主要的分析內容針對臨床表現、術前評估工具、治療方式以及預後。結果：臨床症狀方面，所有的15位病人皆在頰部或頸部摸到腫瘤（100%），其中4位 （27%）具有疼痛感，2位（13%）顏面神經麻痺以及1位（7%）皮膚潰瘍。發生症狀至最終診斷的平均時間為8.3年。術前的診斷工具中，細針抽吸的細胞學檢查診斷率為57.1%，而電腦斷層之診斷率為23%。平均的追蹤期為43.5個月，共有7位病人有腫瘤復發的情形，4位則是因此疾病而死亡。三年的生存率為70%，而三年的無復發生存率為58%。手術的邊界是否殘留腫瘤細胞與腫瘤復發率有明顯的相關性（chi-square=6.61; p=0.0102）。而病人的T stage也和死亡率有明顯的相關性 （chi-square=10.64;p=0.013）。結論：要在術前診斷CXPA是很困難的。以電腦斷層及細針抽吸細胞學檢查這兩項常用的診斷工具而言，細針抽吸具有較高的診斷率。因為手術的方式不同，影響手術切除之邊界是否殘留腫瘤細胞，將會明顯的影響腫瘤復發。我們建議在手術前，根據臨床症狀安排適當檢查，以確立正確診斷。

並列摘要

Background:Carcinoma ex pleomorphic adenoma (CXPA) is an aggressive but poorly understood malignancy which usually arises from salivary glands as a primary or recurrent benign pleomorphic adenoma. The clinical presentation and prognosis were not fully investigated, especially in Asian peopleAim and Objectives:To analyze the clinical cases of CXPA in VGHTPE and to investigate the diagnostic difficulties.Materials and Methods:We retrospectively reviewed the medical records over a 12-year period at the academic medical center. 15 patients with the final pathological diagnosis of CXPA were investigated. Special attention was paid to the clinical presentation, preoperative diagnostic tool as well as the survival time.Results:The most common symptom of CXPA was a palpable mass over cheek or neck (100 %). 4 patients (27%) complained of a painful mass, whereas 2 patients (13%) complained of facial nerve palsy and 1 patient (7%) of skin ulceration. The average time from the onset of symptoms to definite diagnosis was 8.3 years. The sensitivity of cytology in detecting CXPA was 57.1% and that of CT was only 23% in our study. During the follow-up time of 43.5 months in average, 7 patients (46.7%) suffered from tumor recurrence and four patients died from the disease. The overall 3-year survival rate was 70% and the 3-year-recurrence-free survival rate was 58%. The malignant surgical margin was related to the tumor recurrence rate. (chi-square = 6.61; p = 0.0102). Patients in tumor stage T1 and T2 have significantly longer survival time compared with those in tumor stage T3 or T4 (chi-square = 10.64; p = 0.013).Conclusion:Preoperative diagnosis of CXPA is very challenging. Compared with computed tomography, fine needle aspiration cytology has higher sensitivity in detecting this tumor. Given that the malignant surgical margin is associated with the tumor recurrence, more accurate preoperative diagnosis assisted by clinical suspicion and FNAC should be advocated.

並列關鍵字

carcinoma ex pleomorphic adenoma ； parotid gland tumor ； fine needle aspiration cytology