What is the management approach for mucinous carcinoma?

Management of Mucinous Ovarian Carcinoma

Primary treatment consists of completion surgery with comprehensive staging including appendectomy, followed by stage-specific postoperative therapy ranging from observation for early-stage disease to chemotherapy (either standard ovarian or gastrointestinal regimens) for advanced stages. 1

Initial Diagnostic Workup

The critical first step is distinguishing primary mucinous ovarian carcinoma from metastatic disease to the ovaries, as metastases are more common than primary tumors:

• Gastrointestinal tract evaluation and CEA level are mandatory to rule out occult GI primary that has metastasized to the ovaries 1
• PAX8 immunostaining may help differentiate primary from metastatic disease 1
• Standard ovarian cancer workup applies, including imaging and tumor markers 1

Surgical Management

Complete surgical resection is the gold-standard treatment for all mucinous ovarian carcinoma cases 2:

• Completion surgery with comprehensive staging including bilateral salpingo-oophorectomy, total hysterectomy, pelvic washings, omentectomy, and peritoneal biopsies 1, 3
• Appendectomy is specifically recommended at primary surgery in all patients with suspected or confirmed mucinous ovarian tumors 1
• Fertility-sparing surgery (unilateral salpingo-oophorectomy) is an option for patients with unilateral mucinous borderline tumors or disease grossly confined to one ovary with normal-appearing contralateral ovary 1, 3
• Lymph node evaluation should be considered in patients with infiltrative tumors 3

Stage-Specific Postoperative Management

Stage IA or IB Disease

• Postoperative observation and monitoring are recommended, as most of these tumors are benign or borderline 1
• No adjuvant chemotherapy needed for these early stages 1

Stage IC Disease

Postoperative options include 1:

1. Observation (acceptable option)
2. Intravenous carboplatin with either paclitaxel or docetaxel (standard ovarian regimen)
3. 5-FU/leucovorin/oxaliplatin (gastrointestinal regimen)
4. Capecitabine/oxaliplatin (gastrointestinal regimen)

Stages II to IV Disease

Postoperative options include 1:

1. Chemotherapy using standard epithelial ovarian cancer regimens (carboplatin/paclitaxel or carboplatin/docetaxel)
2. 5-FU/leucovorin/oxaliplatin (gastrointestinal regimen)
3. Capecitabine/oxaliplatin (gastrointestinal regimen)

Chemotherapy Considerations

Mucinous ovarian carcinoma is resistant to standard platinum-and-taxane regimens used for other ovarian cancers 3:

• Gastrointestinal regimens are considered appropriate because mucinous carcinomas of the ovary are biologically similar to gastrointestinal tumors 1
• For recurrent platinum-sensitive disease with favorable distribution, secondary debulking may be considered 3
• HIPEC may be worth considering for advanced disease, though data are limited 3

Recurrent or Progressive Disease

• Enrollment in clinical trials is strongly advocated for patients with recurrence or progression on first-line chemotherapy 3
• Molecular testing should be performed in all patients with recurrent or progressive disease to identify actionable mutations for targeted therapy 2, 3
• DNA sequencing can identify tumor-specific mutations allowing matching to available targeted agents, including basket trials or off-label use of drugs approved for other cancer types 2

Prognosis

• 5-year disease-free survival is approximately 80% to 90% overall, with excellent prognosis in early-stage disease 1, 4
• Patients typically present at younger age (20-40 years) than high-grade serous ovarian cancer 1, 4
• Advanced-stage disease carries significantly worse outcomes 4, 5

Critical Pitfalls to Avoid

• Failure to perform appendectomy during primary surgery—this is specifically recommended for mucinous tumors 1
• Inadequate workup to exclude metastatic disease—always obtain GI evaluation and CEA level 1
• Treating advanced mucinous carcinoma identically to serous carcinoma—consider GI regimens given poor response to standard platinum/taxane therapy 1, 3
• Missing opportunity for molecular testing in recurrent disease—this can guide targeted therapy options 2, 3