Jagtap Sunil V, Jagtap Shubham S, Gudur Rashmi, Billawaria Sonam
Department of Pathology, Krishna Institute of Medical Sciences Deemed University, Karad 415110, Maharashtra, India.
Department of Medicine, Krishna Institute of Medical Sciences Deemed University, Karad, India.
Ther Adv Rare Dis. 2022 Jul 12;3:26330040221107389. doi: 10.1177/26330040221107389. eCollection 2022 Jan-Dec.
Primary malignant mixed Müllerian tumor (MMMT) of the ovary is an extremely uncommon neoplasm. These tumors show very aggressive clinical course and high mortality as compared to epithelial ovarian neoplasms. The objective of present study is to present a rare case of primary MMMT homologous type of ovary for its aggressive clinical course and immunohistochemistry findings. A 48-year-old woman presented with complaints of lower abdominal pain, dullness of 3 months duration. USG abdomen pelvis revealed bilateral ovarian solid and cystic mass lesion suggestive of malignant potential. Peritoneal fluid cytology reported as positive for malignant cells. Patient underwent exploratory laparotomy which showed large bilateral ovarian masses with extensive nodular deposits all over pelvic-abdominal organs. Optimal debulking surgery was performed and specimen examined for histopathology. On histopathology, it was reported as bilateral ovarian MMMT homologous type. Immunohistochemistry was done which showed the tumor cell expression positive for CK, EMA, CK7, CA-125, and WT1. Also a distinct population tumor cells express Cyclin D1 and focal and patchy expression of CD-10. Tumor was negative for Desmin, PLAP, Calretin, and inhibin. The patient received operative, chemotherapy and adjuvant therapy along with extensive electrolyte, nutritive, and supplementary support. The patient, however, rapidly deteriorated and died within 9 months of postoperative day. Primary ovarian MMMT is an extremely uncommon neoplasm, and it showed extensive aggressive clinical course and even with operative, chemotherapy, and adjuvant therapy, the patient yields poor prognosis.
卵巢原发性恶性苗勒管混合瘤（MMMT）是一种极为罕见的肿瘤。与上皮性卵巢肿瘤相比，这些肿瘤具有非常侵袭性的临床病程和高死亡率。本研究的目的是报告一例罕见的原发性同源性卵巢MMMT病例，阐述其侵袭性临床病程及免疫组化结果。一名48岁女性因下腹部疼痛、隐痛3个月前来就诊。腹部盆腔超声检查发现双侧卵巢实性和囊性肿块病变，提示具有恶性可能。腹水细胞学检查报告恶性细胞阳性。患者接受了剖腹探查术，术中发现双侧卵巢巨大肿块，盆腔和腹部器官广泛存在结节状沉积物。进行了肿瘤细胞减灭术，并对标本进行了组织病理学检查。组织病理学报告为双侧卵巢同源性MMMT。进行了免疫组化检查，结果显示肿瘤细胞对细胞角蛋白（CK）、上皮膜抗原（EMA）、CK7、CA-125和WT1呈阳性表达。此外，有一群独特的肿瘤细胞表达细胞周期蛋白D1以及局灶性和斑片状的CD-10表达。肿瘤对结蛋白、胎盘碱性磷酸酶（PLAP）、钙视网膜蛋白和抑制素呈阴性。患者接受了手术、化疗和辅助治疗，同时给予广泛的电解质、营养和补充支持。然而，患者术后迅速恶化，在术后9个月内死亡。原发性卵巢MMMT是一种极为罕见的肿瘤，它具有广泛的侵袭性临床病程，即使接受了手术、化疗和辅助治疗，患者的预后仍然很差。
