A PAX-8-Positive Female Urethral Adenocarcinoma, Intestinal-Type: A Case Report with Diagnostic Challenges and a Review of the Literature.

IF 0.7 Q4 PATHOLOGY Case Reports in Pathology Pub Date : 2023-01-01 DOI:10.1155/2023/8323821
Mary M Torrez, Frances M Alba, Jain Zhou
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引用次数: 1

Abstract

Female urethral adenocarcinoma (FUA) is extremely rare. It is an aggressive malignancy, and clear cell and columnar/mucinous ("intestinal") represent the two primary histologic subtypes. Diagnosis is often delayed in patients because of their vague symptomatology; hence, they present with an advanced disease and a poor prognosis. The rarity of FUA brings challenges when determining treatment and management, and treatment guidelines for various stages are lacking. We report an intestinal-type FUA that developed from inflammation-related metaplasia in urethral diverticulum with positive paired box 8 (PAX-8) staining. In addition to intestinal-type FUA being extremely rare, this particular entity exhibiting PAX-8 positivity has not been previously described, to the author's best knowledge. The present report highlights the importance of clinical and radiological assessment as well as histomorphologic and immunophenotypic features for an accurate diagnosis of this rare and aggressive malignancy.

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1例pax -8阳性女性尿道腺癌,肠型:1例诊断挑战及文献复习。
女性尿道腺癌(FUA)极为罕见。它是一种侵袭性恶性肿瘤,透明细胞和柱状/黏液(“肠”)是两种主要的组织学亚型。由于患者的症状不明确,诊断常常被延迟;因此,他们表现为疾病晚期和预后不良。FUA的罕见性给确定治疗和管理带来了挑战,并且缺乏不同阶段的治疗指南。我们报告了一例由尿道憩室炎症相关化生引起的肠道型FUA,配对盒8 (PAX-8)染色呈阳性。除了肠道型FUA极为罕见外,据作者所知,这种表现出PAX-8阳性的特殊实体以前没有被描述过。本报告强调临床和放射学评估以及组织形态学和免疫表型特征对于准确诊断这种罕见的侵袭性恶性肿瘤的重要性。
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