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原发性膀胱神经内分泌-腺癌:一种由大细胞神经内分泌癌和腺癌组成的、具有独特临床行为的未被描述过的实体肿瘤:病例描述及相关文献复习。

Primary MiNEN of the urinary bladder: an hitherto undescribed entity composed of large cell neuroendocrine carcinoma and adenocarcinoma with a distinct clinical behavior : Description of a case and review of the pertinent literature.

机构信息

Department of Medicine and Surgery, Unit of Pathology, University of Insubria, via O. Rossi 9, 21100, Varese, Italy.

Department of Urology, ASST Lariana, Como, Italy.

出版信息

Virchows Arch. 2021 Jul;479(1):69-78. doi: 10.1007/s00428-021-03023-7. Epub 2021 Jan 17.

Abstract

Neuroendocrine carcinomas (NECs) of the urinary bladder are very rare and can be observed in the context of mixed neuroendocrine/non-neuroendocrine neoplasms (MiNENs), most frequently in association with urothelial carcinoma. Small cell NECs are far more common than large cell NECs (LCNECs), which are exceedingly rare. We describe a primary MiNEN of the urinary bladder, composed of a LCNEC and of an adenocarcinoma, in which the neuroendocrine component reached complete pathological regression after neoadjuvant M-VAC chemotherapy, whereas the non-neuroendocrine component of the tumor progressed to metastatic disease. Compared to mixed neuroendocrine/non-neuroendocrine neoplasms described in the literature until now, this appears to be a unique case that expands the spectrum of neuroendocrine neoplasia of the urinary bladder.

摘要

膀胱神经内分泌癌(NEC)非常罕见,可在混合性神经内分泌/非神经内分泌肿瘤(MiNEN)的背景下观察到,最常与尿路上皮癌相关。小细胞 NEC 比大细胞 NEC(LCNEC)更为常见,后者极为罕见。我们描述了一例原发性膀胱 MiNEN,由 LCNEC 和腺癌组成,其中神经内分泌成分在新辅助 M-VAC 化疗后达到完全病理缓解,而肿瘤的非神经内分泌成分进展为转移性疾病。与迄今为止文献中描述的混合性神经内分泌/非神经内分泌肿瘤相比,这似乎是一个独特的病例,扩展了膀胱神经内分泌肿瘤的范围。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/15bb/8298318/5ba47f5c0d4f/428_2021_3023_Fig1_HTML.jpg

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