RT null
T1 Advances in the Treatment of Gastroenteropancreatic Neuroendocrine Carcinomas: Are we Moving Forward?
A1 García Carbonero, Rocío
A1 Anton Pascual, Beatriz
A1 Modrego, Andrea
A1 Riesco Martinez, Maria del Carmen
A1 Lens Pardo, Alberto
A1 Carretero Puche, Carlos
A1 Rubio Cuesta, Beatriz
A1 Soldevilla Navarro, Beatriz
AB Poorly differentiated gastroenteropancreatic neuroendocrine carcinomas are aggressive neoplasms of challenging clinical management. A small proportion of patients with early-stage disease may achieve long-term survival, but the majority of patients present with rapidly lethal metastatic disease. Current standard of care still follows the treatment paradigm of small cell lung cancer, a far more common G3 neuroendocrine neoplasm, although emerging molecular and clinical data increasingly question this approach. In this article, we will briefly summarize epidemiology and prognosis of gastroenteropancreatic neuroendocrine carcinomas to emphasize the very low incidence, aggressive nature, and orphan status of this tumor entity. We will also discuss the current pathological classification and its limitations, as well as recent data on their differential biological background compared with small cell lung cancer, and its potential implications for patients care. Then, we will review the standard of care of systemic therapy, basically focused on platinum-based cytotoxic chemotherapy, including some recent randomized trials providing evidence regarding efficacy of irinotecan vs etoposide platinum doublets. Finally, we will present a comprehensive overview of novel therapeutic strategies in current clinical development, including recently reported data on immunotherapy, tumor-agnostic therapies (microsatellite instability, high tumor mutational burden, NTRK and RET gene fusions, BRAF or KRAS inhibitors), and additional treatment strategies targeting other tumor vulnerabilities (ie, Notch pathway, novel targets for radioligand therapy), and provide some insights regarding unmet needs and future perspectives to improve patient's care and prognosis.
PB Oxford University Press
SN 0163-769X
YR 2023
FD 2023
LK https://hdl.handle.net/20.500.14352/121773
UL https://hdl.handle.net/20.500.14352/121773
LA eng
NO Rocio Garcia-Carbonero, Beatriz Anton-Pascual, Andrea Modrego, Maria del Carmen Riesco-Martinez, Alberto Lens-Pardo, Carlos Carretero-Puche, Beatriz Rubio-Cuesta, Beatriz Soldevilla, Advances in the Treatment of Gastroenteropancreatic Neuroendocrine Carcinomas: Are we Moving Forward?, Endocrine Reviews, Volume 44, Issue 4, August 2023, Pages 724–736, https://doi.org/10.1210/endrev/bnad006
NO M.R. is partially funded by the AECC (CLSEN19003RIES). A.L.P. is funded by the Instituto de Salud Carlos III (PFIS; FI20/00131). C.C.P. was partially funded by CAM (PEJD-2016-PRE/BMD-2666). B.R.C. was partially funded by CAM (PEJD-2017-PRE/BMD-4981). B.S. is partially funded by the AECC (POSTDO46SOLD, Spain).
NO Asociación Española Contra el Cáncer
NO Instituto de Salud Carlos III
NO Comunidad de Madrid
DS Docta Complutense
RD 28 jun 2025