医学
神经内分泌肿瘤
重症监护医学
泌尿生殖系统
临床试验
肿瘤科
病理
内科学
作者
Sara Stumpo,Maria Giovanna Formelli,Irene Persano,Elena Parlagreco,E Lauricella,Maria Grazia Rodriquenz,Luigi Pio Guerrera,Ina Valeria Zurlo,Davide Campana,Maria Pia Brizzi,Mauro Cives,Anna La Salvia,Giuseppe Lamberti
摘要
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.
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