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      Vulvar malignancies: an interdisciplinary perspective

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          Summary

          Vulvar cancer represents the fourth most common gynecologic malignancy and is often encountered by the general Dermatologist or Gynecologist. Dermatooncologists and Gynecologic Oncologists share expertise in this field and the diagnosis and treatment should ideally be interdisciplinary. All subtypes are typically seen in the later decades of life, although all histologic subtypes have been described in women younger than 30 years. The diagnosis is often delayed. Exact mapping of biopsies is of high importance, as the location and distance from the midline guides the surgical approach depending on the underlying histology. Squamous cell carcinoma accounts for more than 76 % of vulvar cancer with vulvar intraepithelial neoplasia being an important precursor. Basal cell carcinoma is the second most common vulvar malignancy. Melanoma accounts for 5.7 % of vulvar cancer and has a worse prognosis compared to cutaneous melanoma. Most of the trials on checkpoint inhibitors and targeted therapy have not excluded patients with vulvar melanoma and the preliminary evidence is reviewed in the manuscript.

          Surgery remains the primary treatment modality of locally resectable vulvar cancer. In view of the rarity, the procedure should be performed in dedicated cancer centers to achieve optimal disease control and maintain continence and sexual function whenever possible.

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          Most cited references97

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          PD-1 Blockade with Cemiplimab in Advanced Cutaneous Squamous-Cell Carcinoma

          No systemic therapies have been approved for the treatment of advanced cutaneous squamous-cell carcinoma. This cancer may be responsive to immune therapy, because the mutation burden of the tumor is high and the disease risk is strongly associated with immunosuppression. In the dose-escalation portion of the phase 1 study of cemiplimab, a deep and durable response was observed in a patient with metastatic cutaneous squamous-cell carcinoma.
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            Revised FIGO staging for carcinoma of the vulva, cervix, and endometrium.

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              Efficacy and Safety of Nivolumab Alone or in Combination With Ipilimumab in Patients With Mucosal Melanoma: A Pooled Analysis.

              Purpose Mucosal melanoma is an aggressive malignancy with a poor response to conventional therapies. The efficacy and safety of nivolumab (a programmed death-1 checkpoint inhibitor), alone or combined with ipilimumab (a cytotoxic T-lymphocyte antigen-4 checkpoint inhibitor), have not been reported in this rare melanoma subtype. Patients and Methods Data were pooled from 889 patients who received nivolumab monotherapy in clinical studies, including phase III trials; 86 (10%) had mucosal melanoma and 665 (75%) had cutaneous melanoma. Data were also pooled for patients who received nivolumab combined with ipilimumab (n = 35, mucosal melanoma; n = 326, cutaneous melanoma). Results Among patients who received nivolumab monotherapy, median progression-free survival was 3.0 months (95% CI, 2.2 to 5.4 months) and 6.2 months (95% CI, 5.1 to 7.5 months) for mucosal and cutaneous melanoma, with objective response rates of 23.3% (95% CI, 14.8% to 33.6%) and 40.9% (95% CI, 37.1% to 44.7%), respectively. Median progression-free survival in patients treated with nivolumab combined with ipilimumab was 5.9 months (95% CI, 2.8 months to not reached) and 11.7 months (95% CI, 8.9 to 16.7 months) for mucosal and cutaneous melanoma, with objective response rates of 37.1% (95% CI, 21.5% to 55.1%) and 60.4% (95% CI, 54.9% to 65.8%), respectively. For mucosal and cutaneous melanoma, respectively, the incidence of grade 3 or 4 treatment-related adverse events was 8.1% and 12.5% for nivolumab monotherapy and 40.0% and 54.9% for combination therapy. Conclusion To our knowledge, this is the largest analysis of data for anti-programmed death-1 therapy in mucosal melanoma to date. Nivolumab combined with ipilimumab seemed to have greater efficacy than either agent alone, and although the activity was lower in mucosal melanoma, the safety profile was similar between subtypes.
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                Author and article information

                Contributors
                christoph.wohlmuth@outlook.com
                Journal
                J Dtsch Dermatol Ges
                J Dtsch Dermatol Ges
                10.1111/(ISSN)1610-0387
                DDG
                Journal Der Deutschen Dermatologischen Gesellschaft
                John Wiley and Sons Inc. (Hoboken )
                1610-0379
                1610-0387
                12 December 2019
                December 2019
                : 17
                : 12 ( doiID: 10.1111/ddg.v17.12 )
                : 1257-1276
                Affiliations
                [ 1 ] Department of Obstetrics and Gynecology Paracelsus Medical University Salzburg Austria
                [ 2 ] Division of Gynecologic Oncology Department of Surgical Oncology University Health Network Toronto ON Canada
                [ 3 ] Department of Obstetrics and Gynecology University of Toronto ON Canada
                [ 4 ] Department of Dermatology Paracelsus Medical University Salzburg Austria
                Author notes
                [*] [* ] Correspondence to

                Christoph Wohlmuth, MD, PhD

                Department of Obstetrics and Gynecology

                Paracelsus Medical University Salzburg

                Müllner Hauptstrasse 48

                5020 Salzburg, Austria

                E‐mail: christoph.wohlmuth@ 123456outlook.com

                Article
                DDG13995
                10.1111/ddg.13995
                6972795
                31829526
                c66d5346-e886-4f64-819e-eee89a80ec8d
                © 2019 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                History
                : 19 August 2019
                : 30 October 2019
                Page count
                Figures: 4, Tables: 1, Pages: 18, Words: 9455
                Categories
                CME Article
                CME‐Artikel
                Custom metadata
                2.0
                December 2019
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.7.5 mode:remove_FC converted:21.01.2020

                Dermatology
                Dermatology

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