Rare vulvar malignancies: sarcomas, melanoma, Paget’s disease


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Abstract

This article describes the clinical presentation, diagnosis, and strategies for the management of rare vulvar malignancies. The anatomical location, depth of invasion, mitotic index, and tumor size for each histological type affect the prognosis and the rates of recurrence and metastatic progression. The difficulty in investigating the etiology, clinical manifestations, course of the disease, and the choice of the optimal treatment modality are associated with the rarity of these tumors. Current recommendations are based on experience with the treatment of squamous cell vulvar cancer and data obtained from published case reports of the treatment of these very rare malignancies. W hile over 90% of vulvar cancers are squamous cell carcinomas, the remaining 10% represents a group of rare histological types of tumors, ranging from malignant melanoma to Bartholin’s gland adenocarcinoma and Paget’s disease.

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About the authors

Kristina V. Safronova

Petrov National Medical Research Center of Oncology of Minzdrav of Russia; Mechnikov North-Western State Medical University of Minzdrav of Russia

Email: kristina-skalli@yandex.ru

Anna S. Artem’eva

Petrov National Medical Research Center of Oncology of Minzdrav of Russia

Email: oinochoya@gmail.com

Ekaterina N. Kozlova

Petrov National Medical Research Center of Oncology of Minzdrav of Russia

Email: enkonco@mail.ru

Anna A. Sidoruk

Petrov National Medical Research Center of Oncology of Minzdrav of Russia; Mechnikov North-Western State Medical University of Minzdrav of Russia

Email: sidoruk_ann@mail.ru

Galia F. Kutusheva

First Pavlov State Medical University of St. Petersburg of Minzdrav of Russia

Elena V. Bakhidze

Petrov National Medical Research Center of Oncology of Minzdrav of Russia; Mechnikov North-Western State Medical University of Minzdrav of Russia

Email: oncl@rion.spb.ru

Igor V. Berlev

Petrov National Medical Research Center of Oncology of Minzdrav of Russia; Mechnikov North-Western State Medical University of Minzdrav of Russia

Email: iberlev@gmail.com

Adiliya F. Urmancheeva

Petrov National Medical Research Center of Oncology of Minzdrav of Russia; Mechnikov North-Western State Medical University of Minzdrav of Russia

Email: adaurm@mail.ru

Elena A. Ul’rikh

Petrov National Medical Research Center of Oncology of Minzdrav of Russia; First Pavlov State Medical University of St. Petersburg of Minzdrav of Russia

Email: elenaulrikh@yandex.ru

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