Somatically Derived Yolk Sac Tumor of the Ovary in a Young Woman
Somatically Derived Yolk Sac Tumor of the Ovary in a Young Woman
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DOI：
10.1097/pgp.0000000000000673
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发表时间：
2021-05-01
影响因子：
2.4
通讯作者：
Parra-Herran, Carlos
中科院分区：
文献类型：
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作者：
Hodgson, Anjelica;Ghorab, Zeina;Parra-Herran, Carlos
卵巢癌合并体细胞来源的卵黄囊肿瘤是一种常见于绝经后妇女的现象。在此，我们报告一位年轻女性，在低级别卵巢样腺癌的背景下发生卵巢囊肿相关的体细胞卵黄囊肿瘤。一位27岁女性因腹痛就诊，随后被诊断为右侧卵巢肿块扭转所致。手术治疗后，输卵管卵巢切除术标本的显微镜检查显示子宫内膜异位症和一个主要的囊性卵巢肿瘤，具有2个不同的表型区域：（1）含有Schiller-Duval小体的卵黄囊肿瘤成分和（2）伴有鳞状化生的低度恶性卵巢样癌成分。免疫组织化学评价显示卵黄囊肿瘤（雌激素受体/孕激素受体/PAX 8阴性，SALL 4/磷脂酰肌醇蛋白聚糖3阳性）和类胰蛋白酶（雌激素受体/孕激素受体/PAX 8阳性，SALL 4/磷脂酰肌醇蛋白聚糖3阴性）组分的不同特征。鉴于这些发现，诊断为卵巢癌相关的卵巢样腺癌伴体细胞来源的卵黄囊肿瘤。由于术中溢出，肿瘤分期为pT 1c 1。患者接受化疗治疗，随访15个月后，她还活着，没有复发的证据。这个例子表明，卵巢上皮性瘤变中的体细胞卵黄囊肿瘤分化可以发生在年轻患者中;意识到这种现象是很重要的，因为体细胞和生殖细胞卵黄囊瘤变有不同的行为和治疗。
Ovarian carcinoma with a somatically derived yolk sac tumor component is a phenomenon known to mostly occur in postmenopausal women. Herein, we report an ovarian endometriosis-associated somatic yolk sac tumor arising in the background of a low-grade endometrioid adenocarcinoma in a young woman. A 27-yr-old woman presented with abdominal pain, subsequently recognized to be caused by a right ovarian mass undergoing torsion. Following operative management, microscopic examination of the salpingo-oophorectomy specimen showed endometriosis and a predominantly cystic ovarian neoplasm with 2 distinct phenotypic areas: (1) a yolk sac tumor component containing Schiller-Duval bodies and (2) a low-grade endometrioid carcinoma component with squamous metaplasia. Immunohistochemical evaluation showed distinct profiles in the yolk sac tumor (estrogen receptor/progesterone receptor/PAX8 negative, SALL4/Glypican 3 positive) and endometrioid (estrogen receptor/progesterone receptor/PAX8 positive, SALL4/Glypican 3 negative) components. Given these findings, the diagnosis of an endometriosis-associated endometrioid adenocarcinoma with a somatically derived yolk sac tumor was rendered. The tumor was staged as pT1c1 due to intraoperative spillage. The patient underwent chemotherapeutic treatment and after 15 mo of follow-up, she was alive with no evidence of recurrence. This example demonstrates that somatic yolk sac tumor differentiation in ovarian epithelial neoplasia can occur in young patients; awareness of this phenomenon is important as somatic and germ cell yolk sac neoplasia have different behavior and therapy.