Definition
Urinary tract large cell neuroendocrine carcinoma (LCNEC) is a high-grade carcinoma with large cells showing neuroendocrine differentiation.
Clinical Features
Incidence
LCNEC is a rare tumor. To date, approximately 20 cases of bladder LCNEC of pure and mixed histology have been reported in the literature (Oshiro et al. 2013; Radović et al. 2015), even with small cell neuroendocrine carcinoma.
Age
The tumor occurs mostly in men.
Sex
The age of diagnosis ranges from 61 to 87 years (mean 74 years) (Moch et al. 2016).
Site
LCNEC is mainly reported in the bladder, and single cases have been described in upper urinary tract (Oshiro et al. 2013).
Treatment
Treatment strategies for LCNEC are represented by cystectomy and chemotherapy.
Outcome
Most patients with LCNEC present with high-stage disease and have a poor prognosis. To date, the only reported series of LCNEC of the bladder compared to small cell...
References and Further Reading
Evans, A. E., Al-Maghrabi, J., Tsihlias, J., et al. (2002). Primary large cell neuroendocrine carcinoma of the urinary bladder. Archives of Pathology & Laboratory Medicine, 126, 1229–1232.
Hailemariam, S., Gaspert, A., Komminoth, P., et al. (1998). Primary, pure, largecell neuroendocrine carcinoma of the urinary bladder. Modern Pathology, 11, 1016–11020.
Moch, H., Humphrey, P. A., Ulbright, T. M., & Reuter, V. (2016). Who classification of Tumours of the urinary system and male genital organs. Lyon: IARC Press.
Oshiro, H., Odagaki, Y., Iobe, H., et al. (2013). Primary large cell neuroendocrine carcinoma of the ureter. International Journal of Clinical and Experimental Pathology, 6, 729–736.
Radović, N., Turner, R., & Baralja, F. (2015). Primary “pure” large cell neuroendocrine carcinoma of the urinary bladder: A case report and review of the literature. Clinical Genitourinary Cancer, 13, e375–e377.
Vollbrecht, C., Werner, R., Walter, R. F., et al. (2015). Mutational analysis of pulmonary tumours with neuroendocrine features using targeted massive parallel sequencing: A comparison of a neglected tumour group. British Journal of Cancer, 113, 1704–1711.
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Raspollini, M.R., Cimadamore, A., Montironi, R., Lopez-Beltran, A. (2020). Urinary Tract Large Cell Neuroendocrine Carcinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4972-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_4972-1
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