Selection of Surgical Strategies for Vulvar Paget’s Disease

Vulvar Paget’s disease (VPD) is a rare intraepithelial adenocarcinoma that predominantly affects older individuals, typically aged between 50 and 80 years. It accounts for 1% to 2% of vulvar malignancies and is characterized by chronic itching, vulvar pain, and erythema. The disease often presents with an “icing cake effect” on the skin, making it easily misdiagnosed as eczema. Due to its asymptomatic nature in the early stages, treatment is often delayed by more than two years, leading to high recurrence rates and treatment failures. Complete surgical removal is the treatment of choice for localized VPD, but the disease’s unclear margins, satellite lesions, and unexpected expansion beyond clinical boundaries complicate surgical planning.

This study aimed to evaluate the effectiveness of two surgical strategies for VPD: extended resection and palliative resection. Extended resection involves a wide excision with a resection line more than 2 cm from the tumor edge, often accompanied by inguinal lymph node dissection if metastasis is present. Palliative resection, on the other hand, focuses on preserving normal tissue while removing the tumor within a 2 cm margin, without multiple frozen biopsies or extensive lymph node dissection. The study followed 54 patients diagnosed with VPD from January 2010 to September 2018, comparing outcomes such as recurrence rates, mortality, surgical complications, and quality of life (QOL) between the two groups.

Background and Clinical Challenges

VPD is a challenging condition to manage due to its unclear margins and potential for satellite lesions. The disease often extends beyond visible clinical boundaries, making it difficult to determine the appropriate resection line. Traditional surgical approaches, such as extensive local excision, have been associated with high recurrence rates, even when negative margins are achieved. Morse microsurgery has shown promise in reducing recurrence rates and preserving tissue, but its effectiveness remains debated. This study sought to provide real-world evidence on the outcomes of extended and palliative resection, focusing on elderly patients who often have comorbidities that complicate treatment decisions.

Study Design and Methodology

The study adopted a real-world research approach, which differs from randomized controlled trials in that it does not artificially restrict patient enrollment criteria, age, or medication regimens. This approach provides results that are more reflective of clinical reality. The study was approved by the Ethical Committee of West China Hospital of Sichuan University, and 54 patients with VPD were followed for an average of 4.6 years. Patients were divided into two groups based on surgical strategy: 25 underwent extended resection, and 29 underwent palliative resection. Inclusion criteria for extended resection included a resection line more than 2 cm from the tumor edge, inguinal lymph node dissection if metastasis was present, and negative frozen pathological margins. Palliative resection included patients with a resection line within 2 cm of the tumor edge, no frozen pathological examination during surgery, or positive margins despite multiple resections.

Surgical Outcomes and Recurrence Rates

The study found no significant differences in mortality, survival time, or recurrence rates between the extended resection and palliative resection groups. This suggests that palliative resection, which preserves more tissue and reduces surgical complexity, may be as effective as extended resection in managing VPD. The median number of frozen biopsies in the extended resection group was 2, compared to 1 in the palliative resection group. The median operation time was 3.0 hours for extended resection and 1.5 hours for palliative resection. Additionally, the palliative resection group had significantly lower surgical costs, reduced rates of skin grafting, and shorter hospital stays. Postoperative scar contracture was also less common in the palliative resection group.

Quality of Life Assessment

The European Organization for Research and Treatment of Cancer Quality of Life Questionnaire (EORTC QLQ-C30) was used to assess postoperative QOL. The questionnaire evaluated physical, role, emotional, cognitive, and social functioning, as well as symptoms such as fatigue, pain, nausea, and financial difficulties. In the 50–69-year age group, patients who underwent palliative resection reported better scores in fatigue, pain, appetite loss, and financial status compared to those who underwent extended resection. However, in the over-70 age group, extended resection patients had lower scores for nausea and vomiting, suggesting that older patients may benefit more from extended resection. Overall, palliative resection was associated with better QOL outcomes for younger patients, while extended resection may be more suitable for older patients.

Prognostic Factors and Recommendations

The study also developed a VPD Prognosis Questionnaire to assess postoperative conditions, including pain, itching, urination, bowel movements, daily activities, and overall QOL. No significant differences were found between the two surgical groups using this questionnaire, further supporting the effectiveness of palliative resection for younger patients. The study concluded that palliative resection is a viable option for patients under 70 years old, as it reduces surgical complexity, shortens recovery time, and improves QOL. For older patients, extended resection may be more appropriate due to its potential to address more extensive disease involvement.

Discussion and Implications

VPD is a rare but challenging condition that requires careful surgical planning. The study’s findings challenge the traditional view that extended resection is necessary to achieve negative margins and reduce recurrence rates. Instead, palliative resection offers a less invasive alternative that preserves tissue, reduces surgical complications, and improves QOL for younger patients. The study also highlights the importance of real-world research in providing clinically relevant insights, particularly for elderly patients with comorbidities.

The study’s limitations include its retrospective design and the relatively small sample size. However, the findings provide valuable guidance for clinicians in selecting surgical strategies for VPD, particularly in balancing the need for complete tumor removal with the preservation of tissue and QOL. Future research should focus on larger, prospective studies to further validate these findings and explore additional treatment modalities, such as chemoradiotherapy, in combination with surgical resection.

In conclusion, this study demonstrates that palliative resection is a safe and effective surgical strategy for VPD, particularly for patients under 70 years old. It reduces surgical complexity, shortens recovery time, and improves QOL without compromising survival or recurrence rates. For older patients, extended resection may be more appropriate due to its potential to address more extensive disease involvement. These findings have important implications for clinical practice and highlight the need for individualized treatment strategies based on patient age and disease characteristics.

doi.org/10.1097/CM9.0000000000001803

Was this helpful?

YesNo

0 / 0