Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: A single‐institution study

Abstract

Extramammary Paget's disease (EMPD) often invades the dermis and metastasizes to the lymph nodes. Patients with EMPD associated with lymph node metastases have poor prognosis; to date, effective treatment has not yet been established. Lymph node dissection, aiming to control the local disease, is a standard form of management for EMPD patients with lymph node metastases (LNM). We investigated the clinical and pathological features, treatment strategies and prognostic factors of patients with metastatic EMPD who underwent lymph node dissection. We retrospectively evaluated 38 cases of extramammary Paget's disease with lymph node metastasis over 10 years. All patients underwent wide resection of the primary lesion and lymph node dissection. Univariate analysis revealed the number of metastatic nodes and lymphadenopathy as prognostic factors. In multivariate analysis, the number of metastatic lymph nodes retained statistical significance (hazard ratio, 35.3; 95% confidence interval, 3.23-387.0; P = 0.003). The 5-year survival rate was 100% and 19.1% in patients with two or less LNM and with three or more LNM, respectively. In patients with three or more LNM, the 5-year survival rate after adjuvant radiation therapy was better than that after surgery alone (75% vs 0%). In conclusion, patients with two or less LNM can be expected to have long-term survival with lymph node dissection only, while patients with three or more LNM may require adjuvant radiation therapy to improve prognosis. These results suggest that lymph node dissection may be a strategy to treat EMPD with regional LNM. METHODS PatientsThis retrospective analysis examined a prospectively maintained database of 89 EMPD patients with dermal invasion who were treated at

Postoperative radiation therapy improves prognoses in extramammary Paget's disease presenting with multiple lymph node metastases

“…We also excluded 19 other patients presenting with ≤2 LN metastases. Finally, 26 patients with ≥3 LN metastases were included in the current study due to their poor prognoses, according to the results of previous reports 5–7 . EMPD diagnosis was pathologically confirmed by a board‐certified dermatologist and a board‐certified pathologist.…”

“…Previous reports have also shown that the number of LN metastases predicts survival 4 . For example, the prognoses of patients with ≥3 LN metastases are significantly worse compared to those with fewer metastases; the 5‐year survival rates for patients with 2 and ≥3 LN metastases have been reported to be 77.8%–100% and 0%–20%, respectively 5–7 …”

“…To date, there is no established postoperative treatment to prevent recurrence or metastasis in patients presenting with LN metastases. Two previous studies have evaluated the survival efficacy of postoperative radiotherapy (RT) in treating EMPD presenting with LN metastases; the 5‐year survival rate ranged from 22% to 75% 6,8 . One of these studies was a single‐arm study, while the other included a very small number of patients treated with RT ( n = 5).…”

Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments

“…The 5-year survival rate was 100% and 19.1% in patients with two or less LN metastases and with three or more LN metastases, respectively. They also found that, in patients with three or more LN metastases, the 5-year survival rate after adjuvant radiation therapy was better than that after surgery alone, indicating that adjuvant radiation therapy for EPMD cases with three or more regional LN metastases may improve the prognosis [127].…”

“…LN dissection is a standard form of management for regional LN metastases in EMPD, although there is a lack of evidence showing that the LN dissection significantly improves overall survival in such patients. Tsutsui et al analyzed patients with metastatic EMPD who underwent LN dissection, and their multivariate analysis revealed that the number of metastatic LNs was an independent prognostic factor for overall survival [127]. The 5-year survival rate was 100% and 19.1% in patients with two or less LN metastases and with three or more LN metastases, respectively.…”