Rare Subentities of Urothelial Bladder Carcinoma

  • Bastian KeckEmail author
  • Simone Bertz
Living reference work entry


Urothelial carcinomas harbor a propensity to divergent histologic differentiation with different clinical behavior and prognosis of the different histologic subtypes. The WHO classification therefore described a variety of distinct histologic variants of infiltrating urothelial carcinomas in 2016. Plasmacytoid urothelial carcinoma is characterized by a single-cell growth pattern that might be caused by loss of cell adhesion due to the lack of E-cadherin. Its clinical course is very aggressive with a high proportion of locally advanced disease and peritoneal spread. Micropapillary carcinomas respond poorly to intravesical BCG and progress rapidly to muscle invasive and metastatic disease, so that radical cystectomy with urinary diversion is regarded as the therapy of choice in this subtype. Nested-type and large nested urothelial carcinomas present tumor cells whose nuclei show only little or no atypia and are arranged in small nests. Treatment options do not differ from conventional urothelial carcinomas. Microcystic urothelial carcinoma shows round to oval microcysts and is a very rare and aggressive variant with hardly no survival of the cases described so far. Giant cell urothelial carcinoma is an extreme form of dedifferentiation with a bizarre and anaplastic appearance and frequent typical or atypical mitotic figures. Sarcomatoid urothelial carcinoma is characterized by spindle cells and has a very bad prognosis; however, it accounts only for a few cases. Lymphoepithelioma-like urothelial carcinoma which harbors a distinct lymphoid infiltrate including T and B lymphocytes, plasma cells, and differential diagnosis to exclude lymphoproliferative diseases is therefore very important in the clinical management.


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Private Practice of UrologyErlangenGermany
  2. 2.Department of UrologyBridge Consortium e.V.MannheimGermany
  3. 3.Department of PathologyUniversity Hospital ErlangenErlangenGermany

Section editors and affiliations

  • Wolfgang Otto
    • 1
  • Shahrokh F. Shariat
    • 2
  1. 1.Department of UrologyUniversity of RegensburgRegensburgGermany
  2. 2.Departement of UrologyWeill Cornell Medical CenterNew YorkUSA

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