Abstract
ObjectiveTo identify clinicopathological characteristics, treatment patterns, clinical outcomes and prognostic factors in patients with vulvar melanoma (VM). Materials & methodsThis retrospective multicentre cohort study included 198 women with VM treated in eight cancer centres in the Netherlands and UK between 1990 and 2017. Clinicopathological features, treatment, recurrence, and survival data were collected. Overall and recurrence-free survival was estimated with the Kaplan-Meier method. Prognostic parameters were identified with multivariable Cox regression analysis. ResultsThe majority of patients (75.8%) had localized disease at diagnosis. VM was significantly associated with high-risk clinicopathological features, including age, tumour thickness, ulceration, positive resection margins and involved lymph nodes. Overall survival was 48% (95% CI 40–56%) and 31% (95% CI 23–39%) after 2 and 5 years respectively and did not improve in patients diagnosed after 2010 compared to patients diagnosed between 1990 and 2009. Recurrence occurred in 66.7% of patients, of which two-third was non-local. In multivariable analysis, age and tumour size were independent prognostic factors for worse survival. Prognostic factors for recurrence were tumour size and tumour type. Only the minority of patients were treated with immuno- or targeted therapy. ConclusionOur results show that even clinically early-stage VM is an aggressive disease associated with poor clinical outcome due to distant metastases. Further investigation into the genomic landscape and the immune microenvironment in VM may pave the way to novel therapies to improve clinical outcomes in these aggressive tumours. Clinical trials with immunotherapy or targeted therapy in patients with high-risk, advanced or metastatic disease are highly needed.
Highlights
Overall survival was 48% and 31% after 2 and 5 years respectively and did not improve in patients diagnosed after 2010 compared to patients diagnosed between 1990 and 2009
Our results show that even clinically early-stage Vulvar melanoma (VM) is an aggressive disease associated with poor clinical outcome due to distant metastases
[5] Most women diagnosed with VM are postmenopausal and presentation is usually delayed due to the anatomic location which contributes to the poor prognosis. [5,7]
Summary
Compared with cutaneous melanomas (CM) (80%), MM have a poor five-year survival of only 25%. [3] Vulvar melanoma (VM) is the second most common malignancy of the vulva, after squamous cell carcinoma, but is still rare with an incidence of 0.1 per 100,000 females per year. Recurrence rates lie between 42 and 70%, with a reported disease-free survival ranging between 12 and 63 months. Surgical treatment in the vulvar area and a high risk of recurrent disease present major clinical challenges in the treatment of patients with VM. The introduction of effective immune- and targeted therapies in 2011 has significantly improved survival in advanced CM, the prognosis of patients with advanced MM has not changed. [14] So far only a few studies describe treatment outcomes of immune- and targeted therapy in VM The introduction of effective immune- and targeted therapies in 2011 has significantly improved survival in advanced CM, the prognosis of patients with advanced MM has not changed. [10] A possible explanation might be the pathogenesis of MM, which seems to differ from that of cutaneous melanoma. [11,12] It has been shown that MM have a different molecular signature than CM by lacking BRAF and NRAS mutations and harbouring KIT mutations. [13,14,15] KIT mutations were shown to be the highest in VM (22%) compared with other MM subtypes (8.8%). [14] So far only a few studies describe treatment outcomes of immune- and targeted therapy in VM
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