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頁籤選單縮合
| 題 名 | Undifferentiated Sarcoma of the Liver=肝未分化肉瘤 |
|---|---|
| 作 者 | 莊文郁; 林哲男; 洪悠紀; 薛純; | 書刊名 | 長庚醫學 |
| 卷 期 | 25:6 2002.06[民91.06] |
| 頁 次 | 頁399-404 |
| 分類號 | 417.5453 |
| 關鍵詞 | 未分化肉瘤; 肝; p53蛋白; Undifferentiated (embryonal) sarcoma; Liver; p53 protein; Ki-67; |
| 語 文 | 英文(English) |
| 中文摘要 | 肝未分化肉瘤是一種主要發生於兒童的罕見原發性肝臟惡性腫瘤。它被認為是一種原始間葉腫瘤,並有高惡性度的表現,過去平均存活不到一年。完全切除腫瘤是治癒的唯一希震,但即使完全切除,仍有許多病人會有腫瘤再發。近年來利用積極性多模式治療,併用外歲女性病患。組織學上,腫瘤由多形性星狀或梭狀細胞和黏液樣間質所組成,並有典型的過碘酸-Schiff陽性,澱粉酵素抗性的透明小球。免疫組織化學方面,腫瘤細胞是vimentin,α1-抗胰蛋白酵素,和α1-抗胰凝乳蛋白酵素陽性。另外,有40%的腫瘤細胞表現p53蛋白,Ki-67則在45%的腫瘤細胞染出。手術後建議病人接受化學治療,夜家屬拒絕。術後第171天發現再發性肝腫瘤,家屬再度拒絕化學治療,並不再回診追蹤。5個月後,病人因腹脹及背痛回到門診,此時再發性腫瘤已達到20公分並壓迫下腔靜脈。病人於住院後接受vincristine, ifosfamide,及cisplatin化學治療。 |
| 英文摘要 | Undifferentiated (embryonal) sarcoma of the live (USL) is a rare primary hepatic malignancy principally affecting patients of pediatric age. It is believed to be a primitive mescenchymal neoplasm, which usually behaves in a highly malignant fashion. The median survival has been less than a year. The only chance for cure appears to be radical excision of the tumor. However, some patients may develop recurrent disease despite complete surgical resection of the tumor. Recently, long-term disease-free survival has been achieved in cases which underwent aggressive multimodal treatment. Herein we report on a 12-year-old girl who underwent surgical excision of USL. Histologically, it was composed of pleomorphic stellate or spindle-shaped cells in a myxoid stroma. Characteristic periodic acid Schiff-postive, diastase-resistant intracytoplasmic hyaline globules were seen. Immunohistochemically, the tumor cells were positive for vimentin, α1-antitrypsin, and α1-antichymotrypsin. In addition, p53 protein was expressed in 40% of tumor cells, and Ki-67 was demonstrated in 45% of tumor cells. Postoperative chemotherapy was recommended, but was refused by the patient’s family. A recurrent liver mass was found 171 days after the operation. Chemotherapy was refused once again by the patient’s family, and she was lost to follow-up for 5 months. The patient was brought to the outpatient clinic again because of abdominal fullness and back pain. The recurrent tumor was 20 cm in size with compression of the inferior vena cava. She was admitted and received chemotherapy with vincristine, ifosfamide, and cisplatin. |
本系統中英文摘要資訊取自各篇刊載內容。