后腹膜卵黃囊瘤一例

熊茜?古健

【摘要】后腹膜卵黃囊瘤臨床罕見，容易漏診或誤診。該文報道了一例經術后病理檢查確診為后腹膜卵黃囊瘤的年輕女性患者，患者因腹脹1周入院，術前影像學檢查提示卵巢來源惡性腫瘤可能，行剖腹探查術中發現腫瘤來源于后腹膜，雙側卵巢未見腫物，予后腹膜腫物切除術，并按卵黃囊瘤的治療予依托泊苷150 mg、順鉑900 mg、博來霉素15 mg（BEP方案）靜脈化學治療，共化學治療6次，第3次化學治療后復查甲胎蛋白降至正常，彩色多普勒超聲檢查未見明顯異常腫物，隨訪1年未見腫瘤復發。該例診治過程提示，影像學檢查結合甲胎蛋白檢測有助于卵黃囊瘤的診斷及預后評估，手術加輔助化學治療對卵黃素瘤有良好的療效。

【關鍵詞】后腹膜;卵黃囊瘤;甲胎蛋白;治療

Primary retroperitoneal yolk sac tumor： a case report Xiong Qian， Gu Jian. Department of Gynecology， the Third Affiliated Hospital of Sun Yat-sen University， Guangzhou 510630， China

【Abstract】Primary retroperitoneal yolk sac tumor is a rare disease in clinical setting， which is likely to be misdiagnosed or miss the diagnosis. In this article， a 25-year-old woman who was pathologically diagnosed with primary retroperitoneal yolk sac tumor was reported. She was admitted to our hospital due to abdominal distention for one week. Preoperative imaging examination suggested the possibility of malignant ovarian tumor. During the exploratory open surgery， it was found that the tumor was derived from the retroperitoneum， and no tumor was found in both ovaries. The tumor was surgically removed. According to the treatment of yolk sac tumor， chemotherapy consisting of etoposide 150 mg + carboplatin 900 mg + bleomycin 15 mg （BEP） was delivered for 6 cycles. After the third cycle of chemotherapy， the alpha-fetoprotein （AFP） level was decreased to normal range. No abnormal tumor was found by color Doppler ultrasound. No tumor recurrence was observed during 1-year follow-up. This case prompts that imaging examination combined with AFP detection contribute to clinical diagnosis and prognosis evaluation of primary retroperitoneal yolk sac tumor. Surgery in combination with chemotherapy yields high clinical efficacy.

【Key words】Retroperitoneum;Yolk sac tumor;Alpha-fetoprotein;Treatment

卵黃囊瘤又稱內胚竇瘤，是一種由胚外結構卵黃囊發生惡變的原發性生殖細胞腫瘤[1]。腹膜外生殖細胞腫瘤常見于縱隔、后腹膜及骶尾部等部位[2]。生殖腺以外的卵黃囊瘤考慮是胚胎發生過程中生殖細胞沿泌尿生殖脊錯誤遷移的結果，占所有成人生殖細胞惡性腫瘤的2% ～ 5%[3]。性腺以外的卵黃囊瘤少見，原發于后腹膜的卵黃囊瘤更為罕見。現將近年我科收治的1例后腹膜卵黃囊瘤診治過程報道如下，以提高臨床醫師對該病的診療水平。

病例資料

一、病史及體格檢查

患者女，25歲。因腹脹1周于2018年6月2日收入院。患者既往體健，1周前無明顯誘因出現腹脹，為持續性脹痛，休息后明顯緩解，曾于當地醫院就診，行盆腔MRI檢查提示下腹部巨大占位性病變，為進一步診治來我院就診。起病以來患者大小便正常，已婚未育，G0P0，月經規律。

入院體格檢查：體溫36.8?C，脈搏86次/分，

呼吸16次/分，血壓105/68 mm Hg（1 mm Hg = 0.133 kPa）。發育正常，姿勢步態正常。婦科檢查提示外陰呈已婚未產型，陰道后穹隆膨隆，子宮頸無舉痛，子宮左上方可觸及一大小約17 cm×15 cm×13 cm實性包塊，邊界清楚，活動度差，無壓痛。

二、實驗室及輔助檢查

血常規示血紅蛋白74 g/L，紅細胞2.97×1012/L，

白細胞7.82×109/L，中性粒細胞0.722，淋巴細胞0.202，血小板計數306×109/L。血清癌抗原125（CA125）74.1 kU/L，人附睪蛋白4 30.1 pmol/L，血清甲胎蛋白10 341.2 μg/L，癌胚抗原（CEA）0.3 μg/L，血清人絨毛膜促性腺激素（HCG）5 U/L。