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被误诊为尿路上皮乳头状癌的贝利尼管癌

Bellini Duct Carcinoma Misdiagnosed with Urothelial Papillary Carcinoma.

作者信息

El Khoury Joey, Abdessater Maher, Halabi Rami, Nasr Fadi, Boustany Johnny, Kanbar Anthony, El Hachem Charbel, El Khoury Raghid

机构信息

Faculty of Medicine and Medical Sciences, Holy Spirit University of Kaslik (USEK), Jounieh, Lebanon.

Department of Urology, University Hospital Center-Notre Dame des Secours, Byblos, Lebanon.

出版信息

Case Rep Oncol Med. 2020 Feb 10;2020:3174674. doi: 10.1155/2020/3174674. eCollection 2020.

Abstract

BACKGROUND

Collecting (Bellini) duct carcinoma (CDC) or Bellini duct carcinoma (BDC) is a rare subtype of kidney tumors, accounting for less than 3% and known to have the worst prognosis. It is known to have multiple clinical presentations; this is why it can be easily misdiagnosed. The aim of this article is to present a case of CDC that was initially misdiagnosed with urothelial papillary carcinoma (UPC) in a 41-year-old male. . Our patient presented with a left flank pain evolving for one month and one episode of gross macroscopic hematuria. Upon presentation, he had left costovertebral angle tenderness. Initial lab tests were normal. Computed tomography revealed a 5 cm solid mass of the left renal pelvis and multiple infracentimetric perihilar lymph nodes. Subsequently, the patient had left nephroureterectomy. Microscopic examination showed the presence of a high-grade urothelial papillary carcinoma of the renal pelvis' lumen. All four of the dissected lymph nodes showed disease metastasis. Three years after establishing the diagnosis, the patient presented again for chronic abdominal pain, with a recent history of weight loss. CT scan showed a left paraaortic mass infiltrating the left psoas muscle over a length of 12 cm. Immunohistochemical profiling of this mass confirmed the diagnosis of Bellini duct carcinoma, rejecting the initial diagnosis of UPC. Therefore, the patient required a cisplatin-gemcitabine-based chemotherapy regimen.

CONCLUSION

BDC remains one of the rare aggressive subtypes of RCC, having a multitude of initial clinical presentations and an unfavorable prognosis. In this patient, CDC was masquerading as a transitional cell carcinoma that should always be kept in mind as a possible presentation. Corresponding early imaging and histopathology exams are primordial for a correct diagnosis and thus a better prognosis.

摘要

背景

集合管癌(CDC)或贝利尼管癌(BDC)是一种罕见的肾肿瘤亚型,占比不到3%,且已知预后最差。它有多种临床表现,这就是它容易被误诊的原因。本文旨在介绍一例41岁男性患者,其集合管癌最初被误诊为尿路上皮乳头状癌(UPC)。我们的患者出现左侧腰痛1个月,并伴有一次肉眼血尿。就诊时,他左侧肋脊角压痛。初始实验室检查正常。计算机断层扫描显示左肾盂有一个5厘米的实性肿块以及多个小于1厘米的肾门周围淋巴结。随后,患者接受了左肾输尿管切除术。显微镜检查显示肾盂腔内存在高级别尿路上皮乳头状癌。所有四个切除的淋巴结均显示有疾病转移。确诊三年后,患者因慢性腹痛再次就诊,近期有体重减轻史。CT扫描显示左腹主动脉旁有一个肿块,浸润左腰大肌长达12厘米。对该肿块进行免疫组织化学分析确诊为集合管癌,排除了最初的尿路上皮乳头状癌诊断。因此,患者需要接受基于顺铂 - 吉西他滨的化疗方案。

结论

集合管癌仍然是肾细胞癌罕见的侵袭性亚型之一,有多种初始临床表现且预后不良。在该患者中,集合管癌伪装成移行细胞癌,应始终将其视为一种可能的表现形式。相应的早期影像学和组织病理学检查对于正确诊断以及改善预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/41a6/7035507/1d1963209430/CRIONM2020-3174674.001.jpg

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