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Case report details long-term survival in appendiceal signet-ring cell carcinoma with uterine involvementA rare cancer found in the uterus and ovaries may be treated with surgery and chemo for over five years

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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.

A 45-year-old woman arrived at the hospital with a swollen uterus and fluid in her belly. Doctors found a rare cancer called signet-ring cell carcinoma, which had spread from her appendix to her ovaries. This specific type of cancer is very uncommon when it first appears in the uterus.

The medical team performed a thorough surgery to remove the cancer and then gave her chemotherapy. Her treatment included drugs like 5-FU, cisplatin, carboplatin, and bevacizumab. These medicines helped stop the cancer from growing and allowed her tumor markers to return to normal levels.

She remained cancer-free for more than five years, with her first signs of the cancer returning only after 32 months. While this story comes from a single patient, it offers hope for others with similar symptoms. Doctors suggest that if someone has an enlarged uterus and fluid in the abdomen, they should suspect this rare cancer. Careful surgery combined with chemotherapy can lead to long-term survival.

What this means for you:
Thorough surgery and chemotherapy helped a woman survive over five years with a rare cancer found in the uterus and ovaries.

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.
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