Patterns of distant metastases in vulvar cancer

Objective Metastatic vulvar cancer is a rare disease. Information on metastatic patterns and corresponding prognosis or therapeutic approaches is scarce. We therefore analyzed pattern and course of metastatic disease in a large single center cohort. Methods All patients with primary squamous-cell cancer of the vulvar [n = 391, median age: 60 years (range 20–94)] treated at the Gynecological Cancer Center Hamburg-Eppendorf 1996–2013 were retrospectively evaluated for occurrence of distant metastasis. Furthermore, a systematic Medline database search was performed using the terms: ‘vulvar cancer’ AND ‘metastasis’, ‘chemotherapy’, ‘patterns of recurrence’, or ‘prognosis’. Results Out of 391 patients with primary squamous cell vulvar cancer, 20 patients (5.1%) eventually presented with distant metastases. In these 20 patients, median time to first diagnosis of metastasis after primary diagnosis was 13.4 months (range 4–104). Often patients experienced one or more local recurrences before distant spread (12/20, 60.0%). Documented metastatic sites were lung (n = 9), liver (n = 7), bone (n = 5), skin (n = 4) and lymph-nodes (axillary/thoracic/paraaortic, n = 3). The majority of patients presented with unilocal metastases (13/20, 65.0%). In univariate analysis tumor diameter, invasion depth, nodal status and number of metastatic lymph-nodes were identified predictive for occurrence of distant metastases. 2-year-overall-survival-rate after metastases of all metastatic patients was 11.3%; median survival from first diagnosis of metastases was 5.6 months. Conclusion The occurrence of distant metastasis from vulvar cancer is a rare event with very limited prognosis. Further efforts, especially translational research will be crucial to identify prognostic markers as well as therapeutic targets to improve survival in these patients.