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Retroperitoneal Teratoma with Malignant Transformation of Primitive Neuroectodermal Tumor

後腹膜腔畸胎瘤的惡性轉變為原始性神經外皮腫瘤

摘要


一位七個月大的女嬰出現腹脹及食慾不良約一星期。在左腹部有一個10(上標 *)15公分的腫瘤,腹部超音波下可見一巨大囊腫,而血清腫瘤指標顯示甲性胎兒蛋白(α-fetoprotein)為17.92ng/ml,而人類絨毛膜激素(β-hCG)則在正常範圍。腹部電腦斷層發現於後腹腔有一11(上標 *)11(上標 *)14公分多葉性囊腫合併鈣化及脂肪組織,疑似後腹膜腔畸胎瘤。腫瘤完整切除後,病理報告證實為第一級不成熟畸胎瘤(低惡性度)。腫瘤在六個月後復發,經切片證實為畸胎瘤的惡性轉變為原始性神經外皮腫瘤。雖然經過化學治療之後,原發腫瘤縮小,但是已發現多處腫瘤轉移。經多次放射線治療及化學治療後,病患在兩年後死亡。結論:未成熟畸胎瘤可能惡性轉為原始性神經外皮腫瘤,患者接受手術切除後仍需密切追蹤。

並列摘要


A 7-month-old girl suffered from abdominal distention with poor appetite for one week. A palpable mass, around 10(superscript *)15 cm was found over left abdomen. The ultrasonography revealed a huge anechoic cyst with septum occupying whole left abdomen. Serum tumor marker studies revealed α-fetoprotein level of 17.92 ng/ml and normal level of β-hCG. Abdominal computed tomography showed a lobulated cyst, 11(superscript *)11(superscript *)14 cm, over left abdomen with calcified and fatty component. Retroperitoneal teratoma was highly suspected and total excision of retroperitoneal tumor was performed. Pathology report revealed a picture of grade Ⅰ immature teratoma. Retroperitoneal tumor was relapsed 6 months later. Open biopsy was performed and pathology report revealed recurrent teratoma with malignant transformation of primitive neuroectodermal tumor (PNET). The patient was then having chemotherapy with JEB proctocol. Although the tumor shrank after the chemotherapy, multiple metastasis was noted 3 months later after chemotherapy. Although the combination chemotherapy and radiotherapy was given, the patient expired two years later. Conclusion: the immature teratoma may transform into PNET and close follow-up after surgical excision is necessary.

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