Sinonasal Ewing's Sarcoma/Primitive Neuroectodermal Tumor

有病理確定診斷且有病程追蹤記錄的鼻部伊文氏肉瘤／原始神經外皮腫瘤病例在文獻上極少見。本研究報告4名鼻部伊文氏肉瘤／原始神經外皮腫瘤的患者的臨床表現及治療經過。其中l名患者於肝母細胞癌治療後診斷發生此病，此兩種腫瘤發生於同一患者在文獻上尚未有報告。患者以鼻塞、流鼻血、眼球外突、臉部或頸部腫塊表現。影像學檢查可見鼻竇均有巨大腫塊，且有鄰近組織的侵犯，1名患者同時有頸部淋巴結腫大。病理檢查顯示均為小型藍細胞腫瘤，且組織免疫化學祟色均封CD99呈陽性反應。l名患者因個人因素僅接受廣泛手術切除，其餘3名接受化療、手術或治癒性放射治療。l名患者因骨轉移過世，1名患者術後40個月失去追蹤，另兩名患者至今病況穩定。鼻部伊文氏肉瘤／原始神經外皮腫瘤是少見且困難治療的疾病，合併多種治療方式有機會達到較佳的局部控制並減少合併症。

關鍵字

伊文氏肉瘤；伊文氏肉瘤家族腫瘤；原始神經外皮腫瘤；小圓藍細胞瘤；上頷骨

並列摘要

Sinonasal Ewing's sarcoma/ primitive neuroectodermal tumor (PNET) with confirmed diagnosis and follow-up data has rarely been reported in literature. Four patients with Ewing's sarcoma/PNET are presented and their managements are discussed. One of our patients had Ewing sarcoma/PNET as a second tumor after treatment of hepatoblastoma, which has never been reported in the literature. They presented with nasal obstruction, nasal bleeding, proptosis, facial or neck mass. Their image studies all demonstrated huge tumors growing from maxillary or ethmoid sinuses with extension to surrounding tissue, and one demonstrated neck lymph node enlargement. Pathological examination of all tumor showed typical small round cell tumor and had strong immunoreactivity to CD99 immunochemistry staining. One patient only received wide excision, while the rest of them received combined treatment with chemotherapy surgery or radiotherapy. One patient died of bone metastasis; one lost to follow-up at 40 months post operation; the other two remain disease free. Sinonasal Ewing sarcoma/PNET is rare and difficult to treat. Multimodality treatment might provide better disease control and lower morbidity.

並列關鍵字

Ewing's sarcoma ； Ewing sarcoma family of tumors ； primitive neuroectodermal tumor ； small round blue cell tumor ； maxilla

國際替代計量

Sinonasal Ewing's Sarcoma/Primitive Neuroectodermal Tumor

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