Case report details long-term survival in appendiceal signet-ring cell carcinoma with uterine involvement.

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Frontiers in Medicine Published April 23, 2026 Medically reviewed April 24, 2026 DOI ↗ By Dr. Julia Lee, PhD · Oncology, Genomics & Drug Development

Key Takeaway

Consider appendiceal SRCC in patients with uterine enlargement and ascites; thorough surgery and chemotherapy may yield long-term survival.

This publication is a case report detailing the management of a 45-year-old woman diagnosed with signet-ring cell carcinoma of the appendix. The disease presented with diffuse uterine involvement and ovarian dissemination. The patient received cytoreductive surgery (CRS) followed by adjuvant chemotherapy consisting of 5-FU plus cisplatin, and subsequent treatment with 5-FU, carboplatin, and bevacizumab. No adverse events, serious adverse events, discontinuations, or tolerability data were reported in this single-patient series.

The primary outcome of overall survival exceeded 52 months. The first recurrence occurred at 32 months. Secondary outcomes included tumor markers, which normalized during the treatment course. The authors highlight that presentation with diffuse uterine involvement as the initial manifestation has not been systematically reported in the literature.

The authors suggest that for patients presenting with uterine enlargement and ascites, appendiceal signet-ring cell carcinoma should be suspected. They identify the enteric immunophenotype and right-sided ovarian dissemination pattern as diagnostic keys. Thorough surgery combined with chemotherapy can achieve long-term survival in this rare presentation. However, the generalizability of findings from a single case report is limited.

Study Details

Study typeSystematic review

EvidenceLevel 1

PublishedApr 2026

View Original Abstract ↓

BackgroundAppendiceal signet-ring cell carcinoma (SRCC) is rarely encountered, and presentation with diffuse uterine involvement as the initial manifestation has not been systematically reported, posing significant risk of misdiagnosis as primary uterine malignancy.Case presentationA 45-year-old woman presented with vaginal bleeding for 4 months. Preoperative CT demonstrated diffuse uterine myometrial thickening and appendiceal wall thickening. Curettage pathology revealed signet-ring cells with immunohistochemistry CK20+++/SATB-2+++/CK7-/PAX-8-, favoring gastrointestinal origin. Intraoperatively, the uterus was 16-week size with a firm, enlarged appendix and right ovarian involvement. Cytoreductive surgery (CRS) confirmed appendiceal SRCC with uterine and mesenteric metastases. Eight cycles of adjuvant 5-FU plus cisplatin were administered. First recurrence occurred at 32 months, treated with six cycles of 5-FU, carboplatin, and bevacizumab. At last follow-up (January 2026), overall survival exceeded 52 months with normalized tumor markers.ConclusionsFor patients with “uterine enlargement and ascites,” appendiceal SRCC should be suspected. The enteric immunophenotype and right-sided ovarian dissemination pattern are diagnostic keys. Thorough surgery combined with chemotherapy can achieve long-term survival.