- 期刊
Proximal-Type Epithelioid Sarcoma-Clinical Cases Report and Literature Review
近端型上皮樣肉瘤－病例報告以及文獻回顧
摘要
背景：上皮樣肉瘤是一種相當少見且有高復發率的肉瘤，表現出易轉移到淋巴結的特性，特別是近端型上皮樣肉瘤。此腫瘤通常並無特別惡性之外觀，也因此容易造成延遲診斷。目的及目標：報告三例近端型上皮樣肉瘤病例，並作文獻回顧。材料及方法：此三位病患中包含兩位女性以及一位男性，並分析病患背景，腫瘤特性以及治療方式。結果：第一位病患一開始被為認為是惡性橫紋肌樣瘤，之後確診為近端型上皮樣肉瘤，經過多次復發以及手術治療，後來腫瘤仍出現肺部轉移，而最後病患死亡。第二位病患由於第一次手術時邊緣仍有腫瘤細胞浸潤而在同樣部位復發，在經過廣泛腫瘤切除以及淋巴切除手術併術後放射線治療，追蹤十個月無法發之情形。第三位病患一開始即接受完整腫瘤切除手術，由於腫瘤部位接近肋骨，術後病患接受放射線治療，追蹤九個月無復發之情形。結論：上皮樣肉瘤和其他軟組織肉瘤的差異在於容易出現淋巴結轉移，近端型相較於遠端型上皮樣肉瘤預後更差。雖然可以以放射線治療或是化學治療作為輔助之治療，但完整的腫瘤手術切除還是主要的治療方式。
關鍵字
無資料並列摘要
Background: Epithelioid sarcoma (ES), especially the proximal type, is a rare subtype of soft tissue sarcoma with a high recurrence rate and frequent lymph nodes metastases. The tumor often presents as an innocuous soft mass; as result, ES is particularly prone to delayed diagnosis. Aims and Objectives: This study was to present our experience treating the proximal-type epithelioid sarcoma (PES) patients and review the relevant literature. Materials and Methods: We treated three patients, two women and one man, with PES. Patient, tumor, and treatment characteristics were analyzed. Results: The first patient was initially diagnosed as malignant rhabdoid tumor. She died of PES after multiple abdominal wall recurrences and lung metastases. The second patient developed recurrences because of inadequate tumor excision. The third patient was disease free after undergoing appropriate surgical resection and adjuvant radiotherapy. Conclusion: Proximal-type epithelioid sarcoma differs from other soft tissue sarcoma in its propensity for nodal spread and has a much poorer prognosis than distal-type epithelioid sarcoma. Wide excision with tumor-free margins has been the mainstream treatment, and radiation and chemotherapy have been used occasionally as adjuvant but have limited success.
延伸閱讀
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