Effectiveness of treatment modalities for signet ring cell adenocarcinoma of the appendix

Area of Science:

• Gastrointestinal Oncology
• Surgical Oncology
• Rare Cancers

Background:

• Signet Ring Cell Adenocarcinoma (SRCA) of the appendix is a rare malignancy.
• SRCA is associated with a poor prognosis and limited treatment guidelines.
• Existing data on optimal management strategies for appendiceal SRCA is scarce.

Purpose of the Study:

• To evaluate treatment outcomes for patients with appendiceal Signet Ring Cell Adenocarcinoma.
• To identify prognostic factors and optimal surgical and systemic treatment strategies.
• To assess the role of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) in managing SRCA.

Main Methods:

• Retrospective review of 84 patients diagnosed with SRCA between 1998 and 2024.
• Analysis of patient demographics, tumor characteristics, treatment modalities, and survival data.
• Comparison of survival outcomes based on treatment interventions, including cytoreductive surgery and HIPEC.

Main Results:

• The majority of SRCA tumors were high-grade (81%) and presented with metastatic disease (69%).
• Complete cytoreduction offered the best 5-year survival (25%), not significantly improved by systemic therapy.
• Hyperthermic intraperitoneal chemotherapy (HIPEC) added to cytoreductive surgery showed a trend towards improved 5-year survival (20% vs 5%, p=0.059).

Conclusions:

• Cytoreductive surgery combined with HIPEC demonstrated the most favorable survival outcomes for appendiceal SRCA.
• Systemic chemotherapy showed no significant efficacy in improving survival for this patient group.
• Appendiceal SRCA continues to have poor overall outcomes, necessitating the development of novel therapeutic approaches.