后腹膜卵黄囊瘤一例

熊茜?古健

【摘要】后腹膜卵黄囊瘤临床罕见，容易漏诊或误诊。该文报道了一例经术后病理检查确诊为后腹膜卵黄囊瘤的年轻女性患者，患者因腹胀1周入院，术前影像学检查提示卵巢来源恶性肿瘤可能，行剖腹探查术中发现肿瘤来源于后腹膜，双侧卵巢未见肿物，予后腹膜肿物切除术，并按卵黄囊瘤的治疗予依托泊苷150 mg、顺铂900 mg、博来霉素15 mg（BEP方案）静脉化学治疗，共化学治疗6次，第3次化学治疗后复查甲胎蛋白降至正常，彩色多普勒超声检查未见明显异常肿物，随访1年未见肿瘤复发。该例诊治过程提示，影像学检查结合甲胎蛋白检测有助于卵黄囊瘤的诊断及预后评估，手术加辅助化学治疗对卵黄素瘤有良好的疗效。

【关键词】后腹膜;卵黄囊瘤;甲胎蛋白;治疗

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

卵黃囊瘤又称内胚窦瘤，是一种由胚外结构卵黄囊发生恶变的原发性生殖细胞肿瘤[1]。腹膜外生殖细胞肿瘤常见于纵隔、后腹膜及骶尾部等部位[2]。生殖腺以外的卵黄囊瘤考虑是胚胎发生过程中生殖细胞沿泌尿生殖脊错误迁移的结果，占所有成人生殖细胞恶性肿瘤的2% ～ 5%[3]。性腺以外的卵黄囊瘤少见，原发于后腹膜的卵黄囊瘤更为罕见。现将近年我科收治的1例后腹膜卵黄囊瘤诊治过程报道如下，以提高临床医师对该病的诊疗水平。

病例资料

一、病史及体格检查

患者女，25岁。因腹胀1周于2018年6月2日收入院。患者既往体健，1周前无明显诱因出现腹胀，为持续性胀痛，休息后明显缓解，曾于当地医院就诊，行盆腔MRI检查提示下腹部巨大占位性病变，为进一步诊治来我院就诊。起病以来患者大小便正常，已婚未育，G0P0，月经规律。

入院体格检查：体温36.8?C，脉搏86次/分，

呼吸16次/分，血压105/68 mm Hg（1 mm Hg = 0.133 kPa）。发育正常，姿势步态正常。妇科检查提示外阴呈已婚未产型，阴道后穹隆膨隆，子宫颈无举痛，子宫左上方可触及一大小约17 cm×15 cm×13 cm实性包块，边界清楚，活动度差，无压痛。

二、实验室及辅助检查

血常规示血红蛋白74 g/L，红细胞2.97×1012/L，

白细胞7.82×109/L，中性粒细胞0.722，淋巴细胞0.202，血小板计数306×109/L。血清癌抗原125（CA125）74.1 kU/L，人附睾蛋白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，血清人绒毛膜促性腺激素（HCG）5 U/L。