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

BACKGROUND

Signet Ring Cell Adenocarcinoma (SRCA) of the appendix is a rare tumor with a poor prognosis and limited information to help guide treatment.

METHODS

We reviewed patients diagnosed with SRCA between 1998 and 2024 ​at all Mayo Clinic sites.

RESULTS

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).

CONCLUSIONS

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.