Primary Pulmonary Sarcomatoid Carcinoma: A Case Series in a Single Medical Center During a 10-year Period

前言：原發性肺肉瘤樣癌於WHO肺癌分類中屬於非小細胞肺癌之一，是屬於非常罕見的肺部惡性腫瘤。其主要由類肉瘤的或肉瘤的成分之上皮癌類腫瘤成分所組成。我們藉由病例的回顧探討原發性肺肉瘤樣癌之臨床病理特徵，影像學表現，分析病人癒後及其相關因子。方法：我們回溯性地從2004年1月1日到2013年6月期間，針對高雄醫學大學附設中和醫院病理部中2214例肺癌病患之檢體，做一次回溯性分析。其檢體中共包含2029例非小細胞肺癌和185例小細胞肺癌，非小細胞肺癌中則再進一步區別出8例原發性肺肉瘤樣癌。藉由個案病歷資料回顧，我們收集個案相關的流行病學資料，影像學表現，臨床病理特徵含免疫染色資料，治療情形，存活時間，及其預後相關之危險因子分析。結果：經病理分析篩選過後，一共有8例原發性肺肉瘤樣癌個案，佔非小細胞肺癌之0.4%。其中含有5位男性個案和3位女性個案，其中五個男性個案皆有抽菸，而女性個案則無。年齡分布43至76歲。全部個案皆無肺部疾病相關之家族病史。這些個案於診斷前之常見初始症狀包含咳嗽有痰，血痰，局部性胸痛。在腫瘤指數上來說，所有個案之血清組織多肽抗原（TPA）皆呈陽性反應。影像學之檢查，多呈現周邊型之實質性腫塊型態，直徑大小分布從1.5公分至16.9公分，左下肺葉有四例，右上肺葉有兩例，左上及右上肺葉各一例。有五例侵犯肋膜或胸壁組織，6例可見肺門和/或縱膈腔淋巴結腫大。顯微病理分類，根據世界衛生組織（WHO）2004年之肺癌分類，有五例個案被分類為多型性肉瘤(其中四例包含上皮組織成分，其中一例由巨大細胞和梭狀細胞組成)。另外三例則被分類為單純性梭狀細胞瘤。於診斷之際，我們所有個案皆已有遠端轉移之情形，轉移之部位包含骨頭，腦部，及肝臟，因此這些個案在臨床分期上皆為第四期。在治療方面，只有一位病人接受手術切除併術後化學治療，五位病人只接受化學治療（其中有兩人同時接受肺部放射線治療），另外兩例則因臨床狀況不佳，採取支持性療法。這些個案的追蹤時間介於4天至62個月，平均生存時間約21.08±8.47個月，中位數約10個月，1年的存活率約38%，其中有兩例病人於存活超過4年。結論：我們藉由回朔性分析一系列本醫學中心10年內之罕見個案病歷研究，期待對原發性肺肉瘤樣癌可以獲得更多了解。但是，我們的研究個案數受限於疾病本身之稀少性，並無法做大規模之統計分析。但經由本研究可發現，年齡和適當治療是決定病人癒後之關鍵因素。

關鍵字

原發性肺肉瘤樣癌

並列摘要

Background: Pulmonary sarcomatoid carcinoma is a rare histologic subtype of non-small cell lung cancer and is difficult to diagnose. In this study, we share our experience in exploring the clinico-pathologic features, clinical characteristics, outcome and prognostic factors of this rare tumor. Method: This study examined 2214 lung cancer samples stored at Kaohsiung Medical University Hospital during a 10-year period (January 2004 to June 2013), including 2029 cases of non-small cell lung cancer (NSCLC) and 185 cases of small cell lung cancer. The medical records of patients with pulmonary sarcomatoid carcinoma were reviewed. The incidence, risk factors, clinical features, imaging characteristics, immuno-histochemical features, treatments, and prognoses were analyzed. Results: Eight cases of sarcomatoid carcinoma (5 males and 3 females) were identified in our study, accounting for 0.4% of NSCLC samples and 0.36% of all lung cancer samples reviewed. The mean age of the patients was 58.4 years (range, 43-76 years). None of the patients had a remarkable medical or family history. Five patients were smokers and the others were ex-smokers. The main initial presenting symptoms included productive cough, hemoptysis, and localized chest pain. All patients had high levels of serum tissue polypeptide antigen on initial diagnosis. On chest radiograph, the most common findings were peripheral nodular opacity or mass (size range, 1.5-16.9 cm in diameter). Using the World Health Organization 2004 criteria, 5 cases were diagnosed as pleomorphic carcinoma, and the other 3 were spindle cell carcinoma. All patients had an aggressive clinical course and distant metastases on initial diagnosis. One patient underwent surgical resection with postoperative chemotherapy, 3 had chemotherapy only, 2 had concurrent chemoradiotherapy, and 2 received supportive care only, due to a poor performance status. The average survival time was 21.08±8.47 months (median, 10 months) and the 1-year overall survival rate was 38%, although 2 patients were alive after 3 years of follow-up. Conclusions: Primary pulmonary sarcomatoid carcinoma is a rare but aggressive malignancy. Based on our review of 8 cases in a 10-year period, age and proper management may be the keys to a better outcome.

並列關鍵字

sarcomatoid carcinoma ； lung

國際替代計量

Primary Pulmonary Sarcomatoid Carcinoma: A Case Series in a Single Medical Center During a 10-year Period

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