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Primary Small Cell Neuroendocrine Carcinoma of the Kidney: Morphological, Immunohistochemical, Ultrastructural, and Cytogenetic Study of a Case and Review of the Literature

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Abstract

Poorly differentiated neuroendocrine carcinomas (PDNECs) of the kidney are extremely rare high-grade cancers accounting for only 42 cases reported in the literature. In this paper, we describe the morphological, immunohistochemical, ultrastructural, and for the first time, cytogenetic features of a renal PDNEC. In addition, we have reviewed the literature and compared the published clinicopathological data with our morphological and genetic results. The tumor arose within the kidney parenchyma and showed the typical histological features of a pure small cell PDNEC. Fluorescence in situ hybridization study demonstrated a complex chromosomal assessment indicative of a high degree of chromosome instability with gain of multiple chromosomes, loss of p53, and amplification of myc gene. These results suggest that renal PDNEC has a different genetic background to renal clear cell carcinoma, mainly characterized by the loss of the short arm of chromosome 3. Conversely, genetic alterations seem to resemble those of type 2 papillary renal cell carcinoma. The review of the literature demonstrated that PDNECs are associated with poor prognosis and that parenchymal tumors show some differences from those arising in the pelvis, in that parenchymal tumors are purely neuroendocrine while pelvic tumors are mostly mixed neuroendocrine–exocrine neoplasms.

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Acknowledgments

This study was supported in part by a grant from the University of Insubria, Varese.

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Authors and Affiliations

  1. Department of Pathology, Ospedale di Circolo, Viale Borri 57, 21100, Varese, Italy

    Stefano La Rosa & Giovanna Finzi

  2. Department of Human Morphology, Anatomic Pathology Unit, University of Insubria, Varese, Italy

    Barbara Bernasconi, Donata Micello & Carlo Capella

  3. Centro Insubre di Biotecnologie per la Salute Umana, Varese, Italy

    Carlo Capella

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  1. Stefano La Rosa
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  2. Barbara Bernasconi
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  3. Donata Micello
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Correspondence to Stefano La Rosa.

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La Rosa, S., Bernasconi, B., Micello, D. et al. Primary Small Cell Neuroendocrine Carcinoma of the Kidney: Morphological, Immunohistochemical, Ultrastructural, and Cytogenetic Study of a Case and Review of the Literature. Endocr Pathol 20, 24–34 (2009). https://doi.org/10.1007/s12022-008-9054-y

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