TY - JOUR
T1 - Incidence, clinical management and prognosis of patients with small intestinal adenocarcinomas from 1999 through 2019
T2 - A nationwide Dutch cohort study
AU - de Back, Tim R.
AU - Linssen, Jasmijn D.G.
AU - van Erning, Felice N.
AU - Verbakel, Caitlin S.E.
AU - Schafrat, Pascale J.M.
AU - Vermeulen, Louis
AU - de Hingh, Ignace
AU - Sommeijer, Dirkje W.
N1 - Funding Information:
This work is supported by The New York Stem Cell Foundation ( NYSCF-I-R43 ), grants from the European Research Council (ERC-CoG 101045612 - NIMICRY), ZonMw (Vici 09–15018-21–10029 ) and Dutch Cancer Society grant ( KWF 14182 ). L.V. is a New York Stem Cell Foundation —Robertson Investigator.
Funding Information:
This work is supported by The New York Stem Cell Foundation ( NYSCF-I-R43 ), grants from the European Research Council ( ERC-CoG 101045612 - NIMICRY), ZonMw ( Vici 09–15018-21–10029 ) and Dutch Cancer Society grant ( KWF 14182 ). L.V. is a New York Stem Cell Foundation —Robertson Investigator. Funder had no role in study design or manuscript.
Publisher Copyright:
© 2024 The Authors
PY - 2024/3/1
Y1 - 2024/3/1
N2 - Background: Small intestinal adenocarcinomas (SIAs) are rare. Hence, randomized controlled trials are lacking and understanding of the disease features is limited. This nationwide cohort investigates incidence, treatment and prognosis of SIA patients, to improve disease outcome. Patients and methods: Data of 2697 SIA patients diagnosed from January 1999 through December 2019 were retrieved from the Netherlands Cancer Registry and Pathology Archive. Incidence was calculated using the revised European Standardized Rate. The influence of patient and tumor characteristics on overall survival (OS) was studied using survival analyses. Results: The age-standardized incidence rate almost doubled from 0.58 to 1.06 per 100,000 person-years, exclusively caused by an increase in duodenal adenocarcinomas. OS did not improve over time. Independent factors for a better OS were a younger age, jejunal tumors, Lynch syndrome and systemic therapy. Only 13.8% of resected patients was treated with adjuvant chemotherapy, which improved OS compared to surgery alone in stage III disease (HR 0.47 (0.35-0.61)), but not in the limited group of deficient mismatch repair (MMR) patients (n = 53, HR 0.93 (0.25-3.47)). In the first-line setting, CAPOX was associated with improved OS compared to FOLFOX (HR 0.51 (0.36-0.72)). For oligometastatic patients, a metastasectomy significantly improved OS (HR 0.54 (0.36-0.80)). Conclusions: The incidence of SIAs almost doubled in the past 20 years, with no improvement in OS. This retrospective non-randomized study suggests the use of adjuvant chemotherapy for stage III disease and first-line CAPOX for metastatic patients. For selected oligometastatic patients, a metastasectomy may be considered. MMR-status testing could aid in clinical decision-making.
AB - Background: Small intestinal adenocarcinomas (SIAs) are rare. Hence, randomized controlled trials are lacking and understanding of the disease features is limited. This nationwide cohort investigates incidence, treatment and prognosis of SIA patients, to improve disease outcome. Patients and methods: Data of 2697 SIA patients diagnosed from January 1999 through December 2019 were retrieved from the Netherlands Cancer Registry and Pathology Archive. Incidence was calculated using the revised European Standardized Rate. The influence of patient and tumor characteristics on overall survival (OS) was studied using survival analyses. Results: The age-standardized incidence rate almost doubled from 0.58 to 1.06 per 100,000 person-years, exclusively caused by an increase in duodenal adenocarcinomas. OS did not improve over time. Independent factors for a better OS were a younger age, jejunal tumors, Lynch syndrome and systemic therapy. Only 13.8% of resected patients was treated with adjuvant chemotherapy, which improved OS compared to surgery alone in stage III disease (HR 0.47 (0.35-0.61)), but not in the limited group of deficient mismatch repair (MMR) patients (n = 53, HR 0.93 (0.25-3.47)). In the first-line setting, CAPOX was associated with improved OS compared to FOLFOX (HR 0.51 (0.36-0.72)). For oligometastatic patients, a metastasectomy significantly improved OS (HR 0.54 (0.36-0.80)). Conclusions: The incidence of SIAs almost doubled in the past 20 years, with no improvement in OS. This retrospective non-randomized study suggests the use of adjuvant chemotherapy for stage III disease and first-line CAPOX for metastatic patients. For selected oligometastatic patients, a metastasectomy may be considered. MMR-status testing could aid in clinical decision-making.
KW - Incidence
KW - Molecular characteristics
KW - Prognosis
KW - Small intestinal adenocarcinoma
KW - Systemic therapy
U2 - 10.1016/j.ejca.2024.113529
DO - 10.1016/j.ejca.2024.113529
M3 - Article
SN - 0959-8049
VL - 199
JO - European Journal of Cancer
JF - European Journal of Cancer
M1 - 113529
ER -