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Effectiveness of treatment modalities for signet ring cell adenocarcinoma of the appendix
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Effectiveness of treatment modalities for signet ring cell adenocarcinoma of the appendix
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Journal Article
Effectiveness of treatment modalities for signet ring cell adenocarcinoma of the appendix
2025
Overview
Signet Ring Cell Adenocarcinoma (SRCA) of the appendix is a rare tumor with a poor prognosis and limited information to help guide treatment.
We reviewed patients diagnosed with SRCA between 1998 and 2024 at all Mayo Clinic sites.
Among 84 patients, the most common presentation was non-specific abdominal pain (31 %). The majority of tumors were high grade (81 %), and most patients had metastatic disease (69 %). The best overall survival at 5 years was 25 % with complete cytoreduction, which was not significantly improved with the addition of systemic therapy. The addition of HIPEC to cytoreduction surgery showed a clinically significant but not a statistically significant improvement in 5-year survival (20 % vs 5 %, p = 0.059).
Cytoreductive surgery with HIPEC showed the best survival outcomes for SRCA, with no demonstrated efficacy for systemic chemotherapy. Overall outcomes remain poor and new treatment modalities are needed.
•Signet ring cell carcinoma of the appendix is associated with a poor prognosis and low 5-year survival.•The standard treatment approach of cytoreductive surgery with HIPEC (CRS/HIPEC) provided a survival advantage.•Systemic chemotherapy offered no survival benefits in patients with or without CRS/HIPEC.
Publisher
Elsevier Inc,Elsevier Limited
Subject
/ Adenocarcinoma with signet ring cell features
/ Adult
/ Aged
/ Appendiceal Neoplasms - mortality
/ Appendiceal Neoplasms - pathology
/ Appendiceal Neoplasms - therapy
/ Appendix
/ Cancer
/ Carcinoma, Signet Ring Cell - mortality
/ Carcinoma, Signet Ring Cell - pathology
/ Carcinoma, Signet Ring Cell - therapy
/ Cytoreduction Surgical Procedures
/ Female
/ Humans
/ Hyperthermic Intraperitoneal Chemotherapy
/ Male
/ Patients
/ Signet ring cell adenocarcinoma
/ Surgery
/ Survival
/ Tumors
