輻射誘導肉瘤樣癌－單一醫學中心回顧

背景：頭頸部輻射誘導肉瘤樣癌（radiation-induced sarcomatoid carcinoma of head and neck, RISHN）通常在受輻射照射的骨骼或軟組織中經過一段時間的潛伏期後出現。Cahan等於1948年首先提出的診斷標準，歷年來基本保持不變。由於近年來，我們觀察病人潛伏期似乎有比較早產生的趨勢，另外RISHN的預後也需要進一步探討，故提出此研究。方法：從2000年12月到2017年9月，回顧本院病理資料庫診斷有spindle cell之發現，如病理確診為肉瘤樣癌sarcomatoid carcinoma（SAC）則納入回顧分析，而SAC又分為頭頸癌放射線治療（RT）後引發的肉瘤樣癌（radiation induced sarcomatoid carcinoma, RIS）與原發性肉瘤樣癌（de novo SAC），針對潛伏期及預後進一步進行分析。結果：共有55個病人病理資料庫診斷有spindle cell之發現，22例病患經病理檢查確診為頭頸部SAC納入回顧，平均年齡59.40+/-14.20歲，女性2例，男性20例。15例為RIS，7例為De Novo SAC，回顧RIS原發部位及De Novo SAC發生部位，RIS原發部位有10例於口腔、2例於口咽、2例於鼻咽及1例於下咽。放射線治療後至RIS發生時間中位數為3.88年（四分位距：1.51年），de novo SAC有7例，原發部位5例於口腔、1例於鼻腔、1例於頸部。RT結束後之累積RIS發生率，80%的病人在RT 5年後發生；診斷SAC後5年之存活率約為25%（95%信賴區間：8－44%）；RIS的5年存活率20%（95%信賴區間：3%－45%），de novo SAC 5年存活率=29%（95%信賴區間：4%－61%），其差別並沒有達統計差異（p=0.5597，log-rank test）。在診斷SAC之後的治療方式中，接受手術的病人相較於沒有手術的病人有較好的存活趨勢（hazard ratio=0.43，95%信賴區間：0.15－1.19），但沒有達到顯著統計差異，放射線治療及化療有無對於存活也沒有達到統計顯著差異。結論：回顧本院頭頸區域之SAC，診斷SAC後5年之存活率約為25%，RIS的潛伏期80%在RT結束後5年內發生，RISHN發生的時間中位數為3.88年，相對於文獻提早，推測與本研究RIS病人全部在原發癌症診斷後都接受過化療有關，接受手術對於長期存活有較好的趨勢，有無接受化療或放療對於存活率都沒有達到統計差異，手術治療對於頭頸部肉瘤樣癌是目前較好的治療方式。

關鍵字

head and neck cancer ； radiation-induced sarcomatoid carcinoma ； survival analysis ； sarcomatoid carcinoma（頭頸部腫瘤；輻射誘導肉瘤樣癌；存活分析；肉瘤樣癌）

並列摘要

BACKGROUND: Radiation-induced sarcomatoid carcinoma of the head and neck region (RISHN) arises from previously irradiated bone and soft tissue after a latency period. The diagnostic criteria were proposed by Cahan et al. in 1984 and remain the current mainstay of diagnosis. However, the latency period should be modified for the tendency of shortened latency based on our observation. In addition, the purpose of this article is to analyze the prognosis of RISHN. METHODS: From Dec 2000 to Sep 2017, all cases with histological findings of spindle cells and a pathological diagnosis of sarcomatoid carcinoma in the head and neck region were included in our retrospective analysis. Furthermore, we classified our cases into two groups: RIS and de novo SAC. Survival analysis of the latency period and prognosis was performed. RESULTS: There were 55 cases with histological findings of spindle cells from the database of our hospital. Twenty-two patients with a pathological diagnosis of sarcomatoid carcinoma in the head and neck region were included in our retrospective analysis, and the mean age was 59.40 +/- 14.20 years old. There were 2 females and 20 males. Fifteen cases had RIS, and 7 cases had de novo SAC. Primary cancers of RISHN arose from the oral cavity in 10 cases, the oropharynx in 2 cases, the nasopharynx in 2 cases, and the hypopharynx in one case. The median latency from radiotherapy to the occurrence of RIS was 3.88 years (IQR: 1.51 years). De novo SAC arose from the oral cavity in 5 cases, the nasal cavity in one case, and the neck region in one case. Eighty percent of patients developed RIS 5 years after prior radiotherapy, according to the cumulative incidence rate. The 5-year survival rate after the initial diagnosis of SAC was approximately 25% (95% CI: 8-44%). The 5-year survival rates of RIS and de novo SAC were 20% (95% CI: 3% - 45%) and 29% (95% CI: 4% - 61%), respectively, but these differences were not statistically significant (p=0.56, log-rank test). Among the treatment modalities of SAC, surgery is seemingly superior to non-surgery. Better survival was observed in those with surgery than in those without surgery (hazard ratio=0.43, 95% CI: 0.15-1.19), but these differences were not statistically significant. Radiotherapy and chemotherapy were not statistically associated with survival. CONCLUSIONS: By analyzing the cases of RISHN in our hospital, the 5-year survival rate after the initial diagnosis of sarcomatoid carcinoma was approximately 25% (95% CI:8-44%). Eighty percent of RISs occurred within 5 years. The latency of RIS was shorter in this study compared to that reported in previous literature, and we speculate that this is due to the chemotherapeutic treatment of primary cancers of RISHN. Surgery is seemingly superior to non-surgery. Radiotherapy and chemotherapy were not statistically associated with survival. Surgical intervention for head and neck sarcomatoid carcinoma is currently the preferred treatment modality.

並列關鍵字

head and neck cancer ； radiation-induced sarcomatoid carcinoma ； survival analysis ； sarcomatoid carcinoma

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