Mesonephric-like Carcinoma of the Endometrium
Mesonephric-like Carcinoma of the Endometrium
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子宫内膜中肾样癌
DOI：
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发表时间：
2019
影响因子：
5.6
通讯作者：
A. Malpica
中科院分区：
文献类型：
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作者：
E. Euscher;R. Bassett;D. Duose;Chieh Lan;I. Wistuba;L. Ramondetta;P. Ramalingam;A. Malpica
子宫内膜间肾样癌（MLCa）是罕见的，目前报告的病例少于50例。先前的研究已经描述了MLCa的组织学、免疫组织化学和分子特征；然而，关于结果的信息有限。这项对23例子宫MLCas的单机构研究描述了这种肿瘤的行为特征。回顾了2004年至今子宫MLCas的组织学特征、免疫组化结果、分子谱和临床信息（分期、治疗、随访）。将MLCa的行为与2004年至今在我院治疗的低级别子宫内膜样癌（ECas）和子宫浆液性癌（USCs）进行比较。所有mlca均具有先前描述的结构和细胞学特征，最明显的是导管和/或管状结构（21/23），细胞核类似于甲状腺乳头状癌（18/23），至少有局灶性腔内嗜酸性粒细胞分泌物（20/23）。免疫过氧化物酶研究促进22例诊断：CD10, 10/10；calretinin 5/15;雌激素受体（≥10%细胞核），6/21；孕酮受体，1/15；GATA-3 15/16;TTF-1, 11/16。17例检测病例中有14例发生KRAS突变（7例为唯一突变，7例伴有PIK [n=5]、PTEN [n=2]、CTNNB1 [n=1]等额外突变）。1例PTEN、PIK和CTNNB1突变，无KRAS；2例未检出体细胞突变。总体而言，48%的国际妇产科联合会（FIGO）患者出现3期或4期疾病，伴有以下子宫危险因素：bbb50 %子宫内膜浸润，20/23；淋巴血管浸润，16/23；宫颈间质浸润，7/23。20例患者接受辅助治疗（单纯放疗7例，化疗±放疗13例），3例患者治疗未知或拒绝治疗。21例患者随访：17例患者复发或从未获得缓解，肺是最常见的复发部位（n=9）；7例患者死于疾病。MLCa的中位无进展生存期为18.2个月，ECa为183个月，USC为67.1个月。MLCa的中位总生存期为70.6个月，而USC为139.1个月（ECa的中位生存期未达到）。子宫MLCa并不常见，大多数肿瘤表现为结构不均匀、囊状、核槽重叠、腔内分泌物嗜酸性。典型的免疫图谱包括激素受体的低表达或缺失表达，但至少局灶性表达GATA-3和/或TTF-1。尽管与ECa相关的基因突变并不罕见，但大多数检测病例都有KRAS突变。与更常见的ECa类型相比，MLCa更具侵袭性，倾向于早期和远处复发。
Endometrial mesonephric-like carcinomas (MLCa) are uncommon with <50 reported cases thus far. Previous studies have characterized the histologic, immunohistochemical, and molecular features of MLCa; however, there is limited information with respect to outcome. This single-institution study of 23 uterine MLCas characterizes the behavior of such a neoplasm. Uterine MLCas (2004-present) had review of histologic features, immunohistochemical results, molecular profile, and clinical information (stage, treatment, follow-up). The behavior of MLCa was compared with low-grade endometrioid carcinomas (ECas) and uterine serous carcinomas (USCs) treated at our institution from 2004 to present. All MLCas had a mixture of previously described architectural and cytologic features most notably ductal and/or tubular architecture (21/23), nuclei resembling those of papillary thyroid carcinoma (18/23), and at least focal intraluminal eosinophilic secretions (20/23). Immunoperoxidase studies facilitated diagnosis in 22 cases: CD10, 10/10; calretinin, 5/15; estrogen receptor (≥10% nuclei), 6/21; progesterone receptor, 1/15; GATA-3, 15/16; TTF-1, 11/16. Fourteen of 17 tested cases had a KRAS mutation (7 as the only alteration; 7 with additional mutations including PIK [n=5]; PTEN [n=2], CTNNB1 [n=1]).One case had mutations in PTEN, PIK, and CTNNB1 without KRAS; 2 cases had no detectable somatic mutation. Overall, 48% of patients presented with International Federation of Gynecology and Obstetrics (FIGO) stage 3 or 4 disease with the following uterine risk factors: >50% myometrial invasion, 20/23; lymphovascular space invasion, 16/23; cervical stromal invasion, 7/23. Twenty patients had adjuvant therapy (7 radiation only; 13 chemotherapy±radiation), whereas 3 patients had either unknown or declined therapy. Follow-up was known for 21 patients: 17 patients had recurrences or never achieved remission with the lung being the most common recurrence site (n=9); 7 patients died of disease. The median progression-free survival was 18.2 months for MLCa compared with 183 months for ECa and 67.1 months for USC. The median overall survival for MLCa was 70.6 months compared with 139.1 months for USC (median survival for ECa not reached). Uterine MLCa is uncommon with most tumors recognized by architectural heterogeneity, vesicular, overlapping nuclei with grooves, and eosinophilic luminal secretions. The typical immunoprofile includes low to absent expression of hormone receptors but at least focal expression of GATA-3 and/or TTF-1. Most tested cases had a KRAS mutation although genetic mutations typically associated with ECa are not uncommon. Compared with more commonly encountered types of ECa, MLCa is more aggressive with a tendency towards earlier and distant recurrence.