How is ovarian carcinoma with periportal disease staged and treated?

Staging and Treatment of Ovarian Carcinoma with Periportal Disease

Ovarian carcinoma with periportal disease is classified as Stage IIIC according to FIGO staging and requires aggressive surgical cytoreduction followed by platinum-based chemotherapy for optimal outcomes.

Staging Classification

Periportal disease in ovarian carcinoma represents advanced disease with metastatic spread beyond the pelvis. According to the FIGO staging system:

• Stage IIIC is defined as peritoneal metastasis beyond the pelvis >2 cm in diameter and/or positive regional lymph nodes 1
• Periportal disease (metastasis to the porta hepatis region) falls under this classification as it represents macroscopic peritoneal metastasis beyond the pelvis

The staging process requires:

• Comprehensive surgical exploration via laparotomy
• Careful examination of the entire abdominal cavity
• Collection of peritoneal washings
• Multiple biopsies of peritoneal surfaces
• Assessment of lymph nodes 1

Surgical Management

The cornerstone of treatment for ovarian carcinoma with periportal disease is:

1. Primary Debulking Surgery (PDS) with the goal of complete cytoreduction:

   • Total abdominal hysterectomy and bilateral salpingo-oophorectomy
   • Complete infragastric omentectomy
   • Resection of all visible disease including periportal metastases
   • Pelvic and para-aortic lymphadenectomy when feasible 1

2. Neoadjuvant Chemotherapy (NACT) followed by interval debulking surgery when:

   • Complete resection seems unlikely based on disease extent
   • Patient has poor performance status or significant comorbidities
   • Extensive periportal involvement that would make primary surgery technically challenging 1, 2

Critical Considerations for Periportal Disease

Periportal disease presents specific surgical challenges:

• Proximity to major vascular structures (portal vein, hepatic artery)
• Risk of significant bleeding
• Technical complexity requiring advanced surgical expertise 3

Implants larger than 2 cm in the porta hepatis may represent potentially unresectable disease and should prompt consideration of NACT rather than primary surgery 3.

Systemic Treatment

After surgical management, systemic treatment includes:

1. First-line chemotherapy:

   • Carboplatin (AUC 5-7.5) plus paclitaxel (175 mg/m²/3 hours) every 3 weeks for 6 cycles 1, 2, 4

2. Maintenance therapy options to consider:

   • Bevacizumab (anti-angiogenic agent)
   • PARP inhibitors (especially for patients with BRCA mutations) 5

Response Evaluation and Follow-up

• CA125 levels should be monitored prior to each chemotherapy cycle 1
• CT scan should be performed after completion of chemotherapy or earlier if disease progression is suspected 1
• Regular follow-up examinations every 3 months for the first 2 years, then less frequently 2

Prognostic Factors

The prognosis for ovarian carcinoma with periportal disease depends on:

1. Residual disease after surgery (most important factor)
2. Response to platinum-based chemotherapy
3. Histological subtype and grade
4. Patient's performance status 2, 5

The 5-year survival rate for Stage IIIC ovarian cancer is approximately 35-45% 1, 5.

Important Caveats

• Complete tumor resection is the most important prognostic factor and should be the primary goal of surgery 2
• Treatment should be carried out in specialized ovarian cancer centers with experienced gynecologic oncologists 2
• The decision between primary debulking surgery and neoadjuvant chemotherapy should be made by a multidisciplinary team based on imaging findings, disease extent, and patient factors 1, 2
• Periportal disease may be detected preoperatively with CT, MRI, or PET/CT, which can help guide the surgical approach 1, 3