Chivukula Mamatha, Hunt Jennifer, Carter Gloria, Kelley Joseph, Patel Minita, Kanbour-Shakir Amal
Department of Pathology, Magee-Womens Hospital of the UPMC Health System, Pittsburgh, PA, USA.
Int J Gynecol Pathol. 2007 Jan;26(1):30-3. doi: 10.1097/01.pgp.0000225387.48868.39.
Gynandroblastoma is a rare ovarian tumor that is composed of both Sertoli cells and granulosa cells. Only 23 cases have been reported in the literature, and recurrence has never been described. We report the first case of a recurrent gynandroblastoma along with its molecular analysis and immunohistochemical studies. A 49-year-old Gravida 0 woman with a 10-year prior diagnosis of ovarian-mixed stromal tissue tumor (well-differentiated Sertoli cell and granulosa cell tumor) and staging laparotomy, presented now with a retroperitoneal mass and an elevated inhibin level. CT scan was suspicious for recurrence. The patient had no prior adjuvant therapy. The histomorphological features of the recurrent tumor had both Sertoli cell and granulosa cell tumor. The molecular analysis of both primary and recurrent tumor showed minor genetic instability in the 17q12.2 gene locus with no dedifferentiation or progression, which is consistent with a low-grade tumor. The immunohistochemical staining profile showed positivity for CD99, inhibin, calretinin, and vimentin; focal positivity for cytokeratin AE1/AE3 and negative for EMA and melan-A. All the previously mentioned immunostainings support the diagnosis. We report the first case of a recurrent gynandroblastoma 10 years after initial presentation along with its molecular analysis and immunohistochemical studies.
两性母细胞瘤是一种罕见的卵巢肿瘤,由支持细胞和颗粒细胞组成。文献中仅报道了23例,且从未有过复发的描述。我们报告了首例复发性两性母细胞瘤病例及其分子分析和免疫组化研究。一名49岁未孕女性,10年前被诊断为卵巢混合性间质组织肿瘤(高分化支持细胞和颗粒细胞瘤)并接受了分期剖腹手术,现出现腹膜后肿块且抑制素水平升高。CT扫描怀疑为复发。该患者之前未接受辅助治疗。复发性肿瘤的组织形态学特征兼具支持细胞瘤和颗粒细胞瘤。原发肿瘤和复发性肿瘤的分子分析显示17q12.2基因位点存在轻微遗传不稳定,无去分化或进展,这与低级别肿瘤相符。免疫组化染色结果显示CD99、抑制素、钙视网膜蛋白和波形蛋白呈阳性;细胞角蛋白AE1/AE3呈局灶性阳性,上皮膜抗原和黑素A呈阴性。上述所有免疫染色均支持该诊断。我们报告了首例初次发病10年后复发的两性母细胞瘤病例及其分子分析和免疫组化研究。