Defining a role for systemic chemotherapy in local and advanced appendix adenocarcinoma

M C Strach; B Chakrabarty; R T Nagaraju; S Mullamitha; M Braun; S T O'Dwyer; O Aziz; J Barriuso

doi:10.1016/j.esmoop.2023.101619

Defining a role for systemic chemotherapy in local and advanced appendix adenocarcinoma

ESMO Open. 2023 Oct;8(5):101619. doi: 10.1016/j.esmoop.2023.101619. Epub 2023 Aug 24.

Authors

M C Strach¹, B Chakrabarty², R T Nagaraju³, S Mullamitha⁴, M Braun³, S T O'Dwyer³, O Aziz³, J Barriuso⁵

Affiliations

¹ Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, Manchester; Division of Cancer Sciences, School of Medical Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK; Faculty of Medicine and Health, The University of Sydney, Darlington, Australia. Electronic address: madeleine@strach.net.
² Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, Manchester; Division of Cancer Sciences, School of Medical Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK; Department of Pathology, The Christie NHS Foundation Trust, Manchester, UK.
³ Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, Manchester; Division of Cancer Sciences, School of Medical Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
⁴ Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, Manchester.
⁵ Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, Manchester; Division of Cancer Sciences, School of Medical Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK. Electronic address: jorge.barriuso@manchester.ac.uk.

Abstract

Background: Appendix adenocarcinomas (AAs) are rare tumours that often present late, with a propensity for peritoneal metastases (PMs). This study aimed to evaluate outcomes of AA patients undergoing cytoreductive surgery (CRS) with curative intent and determine the role of systemic chemotherapy.

Materials and methods: Data were collected from a prospective database and classified according to World Health Organization (WHO) 2019 classification. Tumour clearance from CRS was described using a completeness of cytoreduction (CC) score ranging from 0 [no residual disease (RD)] to 3 (>2.5 cm RD). Patients with CC0-2 CRS received hyperthermic intraperitoneal chemotherapy (HIPEC). Systemic chemotherapy was categorised as 'prior' (>6 months before), 'neoadjuvant' (<6 months before), 'adjuvant' (<6 months after CC0-1 CRS) or 'palliative' (after CC2-3 CRS). Analyses used Kaplan-Meier and Cox regression methods.

Results: Between January 2005 and August 2021, 216 AA patients were identified for inclusion. Median age was 59 years (21-81 years). CRS/HIPEC was carried out in 182 (84%) patients, of whom 164/182 (76%) had mitomycin C HIPEC. CC0-1 was achieved in 172 (80%) patients. Systemic chemotherapy was given to 97 (45%) patients from the whole cohort and to 37/46 (80%) patients with positive nodes. Median overall survival (OS) was 122 months (95% confidence interval 61-182 months). After multivariate analysis, patients with acellular and lower-grade PM had similar OS to those with localised (M0) disease (P = 0.59 and P = 0.19). For patients with positive nodes, systemic chemotherapy was associated with reduced risk of death compared to no chemotherapy (P < 0.0019).

Conclusion: This study identifies AA patients with positive lymph nodes derive the most benefit from systemic chemotherapy. We confirm the prognostic importance of stage and peritoneal grade, with excellent outcomes in patients with acellular mucin and lower-grade PM.

Keywords: appendix adenocarcinoma; appendix cancer; chemotherapy; peritoneal metastases; treatment outcomes.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adenocarcinoma* / drug therapy
Adenocarcinoma* / pathology
Appendiceal Neoplasms* / drug therapy
Appendiceal Neoplasms* / pathology
Appendix* / pathology
Humans
Middle Aged
Peritoneal Neoplasms* / drug therapy
Prognosis