Extrapulmonary Neuroendocrine Carcinomas: Current Management and Future Perspectives

Neuroendocrine carcinomas (NECs) are poorly differentiated and highly aggressive epithelial neuroendocrine neoplasms. The most common primary site is the lung, but they may arise in every organ. Approximately 37% of extrapulmonary NECs (EP-NECs) occur in the gastroenteropancreatic (GEP) tract, follo...

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Published in:Journal of clinical medicine 2023-12, Vol.12 (24), p.7715
Main Authors: Stumpo, Sara, Formelli, Maria Giovanna, Persano, Irene, Parlagreco, Elena, Lauricella, Eleonora, Rodriquenz, Maria Grazia, Guerrera, Luigi Pio, Zurlo, Ina Valeria, Campana, Davide, Brizzi, Maria Pia, Cives, Mauro, La Salvia, Anna, Lamberti, Giuseppe
Format: Article
Language:English
Subjects:
Cancer
Care and treatment
Chemotherapy
Clinical medicine
Clinical trials
Epidemiology
Esophagus
Medical prognosis
Metastasis
Mutation
Neuroendocrine tumors
Peptides
Protein expression
Radiation therapy
Surgery
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Summary:Neuroendocrine carcinomas (NECs) are poorly differentiated and highly aggressive epithelial neuroendocrine neoplasms. The most common primary site is the lung, but they may arise in every organ. Approximately 37% of extrapulmonary NECs (EP-NECs) occur in the gastroenteropancreatic (GEP) tract, followed by the genitourinary (GU) system and gynecological tract. As a result of their rarity, there is scant evidence to guide treatment recommendations, and a multidisciplinary approach is essential for the management of such patients. Platinum-based chemotherapy currently represents the standard of care for EP-NECs of any site, mirroring the management of small-cell lung cancer (SCLC), but further approaches are still under investigation. Indeed, ongoing trials evaluating targeted therapies, immune checkpoint inhibitors (ICIs), and radionuclide therapy could provide potentially breakthrough therapeutic options. Given the relative dearth of evidence-based literature on these orphan diseases, the aim of this review is to provide an overview of the pathology and current treatment options, as well as to shed light on the most pressing unmet needs in the field.
ISSN:2077-0383
2077-0383
DOI:10.3390/jcm12247715