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Review
. 2021 Mar;32(1):192-210.
doi: 10.1007/s12022-020-09660-z. Epub 2021 Jan 12.

Genomics of High-Grade Neuroendocrine Neoplasms: Well-Differentiated Neuroendocrine Tumor with High-Grade Features (G3 NET) and Neuroendocrine Carcinomas (NEC) of Various Anatomic Sites

Silvia Uccella  1 , Stefano La Rosa  2 , Jasna Metovic  3 , Deborah Marchiori  4 , Jean-Yves Scoazec  5 , Marco Volante  3 , Ozgur Mete  6   7 , Mauro Papotti  3
Affiliations
Review

Genomics of High-Grade Neuroendocrine Neoplasms: Well-Differentiated Neuroendocrine Tumor with High-Grade Features (G3 NET) and Neuroendocrine Carcinomas (NEC) of Various Anatomic Sites

Silvia Uccella et al. Endocr Pathol. 2021 Mar.

Abstract

High-grade neuroendocrine neoplasms (HG-NENs) are clinically aggressive diseases, the classification of which has recently been redefined. They now include both poorly differentiated NENs (neuroendocrine carcinoma, NECs) and high proliferating well-differentiated NENs (called grade 3 neuroendocrine tumors, G3 NETs, in the digestive system). In the last decade, the "molecular revolution" that has affected all fields of medical oncology has also shed light in the understanding of HG NENs heterogeneity and has provided new diagnostic and therapeutic tools, useful in the management of these malignancies. Considering the kaleidoscopic aspects of HG NENs in various anatomical sites, this review systematically addresses the genomic landscape of such neoplasm throughout the more common thoracic and digestive locations, as well as it will consider other rare but not exceptional primary sites, including the skin, the head and neck, and the urogenital system. The revision of the available literature will then be oriented to understand the translational relevance of molecular data, by analyzing conceptual issues, clinicopathological correlations, and unmet needs in this field.

Keywords: Genetics; Genomics; Grade 3 neuroendocrine tumor; High-grade neuroendocrine neoplasms; Molecular analysis; Neuroendocrine carcinoma.

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References

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