Primary ovarian malignant mixed Müllerian tumor: a rare case report.

Therapeutic advances in rare disease Pub Date : 2022-07-12 eCollection Date: 2022-01-01 DOI:10.1177/26330040221107389
Sunil V Jagtap, Shubham S Jagtap, Rashmi Gudur, Sonam Billawaria
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引用次数: 2

Abstract

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.

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原发性卵巢恶性混合Müllerian肿瘤:一例罕见病例报告。
卵巢原发性恶性混合性米勒氏肿瘤(MMMT)是一种极不常见的肿瘤。与卵巢上皮性肿瘤相比,这些肿瘤表现出非常积极的临床过程和高死亡率。本研究的目的是介绍一例罕见的原发性MMMT同源型卵巢的侵袭性临床过程和免疫组织化学结果。一位48岁的女性主诉下腹疼痛,持续时间为3个月。USG腹部-骨盆显示双侧卵巢实性和囊性肿块,提示有恶性潜能。腹膜液细胞学检查报告为恶性细胞阳性。患者接受了剖腹探查术,发现双侧卵巢有巨大肿块,盆腔腹部器官有大量结节性沉积物。进行了最佳的去毛刺手术,并对标本进行了组织病理学检查。在组织病理学上,报告为双侧卵巢MMMT同源型。免疫组化显示肿瘤细胞CK、EMA、CK7、CA-125和WT1表达阳性。此外,不同的肿瘤细胞群表达细胞周期蛋白D1和CD-10的局灶性和斑片状表达。肿瘤的Desmin、PLAP、Calretin和抑制素均为阴性。患者接受了手术、化疗和辅助治疗,以及广泛的电解质、营养和补充支持。然而,患者病情迅速恶化,在术后9个月内死亡。原发性卵巢MMMT是一种极不常见的肿瘤,它表现出广泛的侵袭性临床过程,即使进行手术、化疗和辅助治疗,患者的预后也很差。
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