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

Safronova K.V., Artem’eva A.S., Kozlova E.N., Sidoruk A.A., Kutusheva G.F., Bakhidze E.V., Berlev I.V., Urmancheeva A.F., Ul’rikh E.A.

1) Petrov National Medical Research Center of Oncology of Minzdrav of Russia, St. Petersburg, Russia; 2) Mechnikov North-Western State Medical University of Minzdrav of Russia, St. Petersburg, Russia; 3) First Pavlov State Medical University of St. Petersburg of Minzdrav of Russia, St. Petersburg, Russia
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. While 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.

Keywords

sarcoma
melanoma
extramammary Paget disease
vulva

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Received 10.12.2019

Accepted 24.12.2019

About the Authors

Kristina V. Safronova, graduate student of the surgical oncogynecological department of the Federal State Budgetary Institution Scientific Research Center for Oncology named after N.N. Petrova of the Ministry of Health of Russia, Department of Oncology, FSBEI HE North-West State Medical University named after I.I. Mechnikov Ministry of Health of Russia (St. Petersburg, Russia). Contact phone: 8 (906) 400-27-91 E-mail: kristina-skalli@yandex.ru
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.
Ekaterina N. Kozlova, MD, PhD, Oncologist, Clinical and Diagnostic Department, Federal State Budgetary Institution Oncology named after N.N. Petrova Ministry of Health of Russia E-mail: enkonco@mail.ru
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.
Anna S. Artemyeva, candidate of medical sciences, head of the scientific laboratory of tumor morphology, head of the pathology department with the prosecture of the National Medical Research Center for Oncology named after N.N. Petrova Ministry of Health of Russia (St. Petersburg). Email: oinochoya@gmail.com
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.
Anna A. Sidoruk, MD, PhD, Oncologist, Surgical Oncogynecological Department, Federal State Budgetary Institution Oncology Research Center named after N.N. Petrova of the Ministry of Health of Russia, Assistant of the Department of Obstetrics and Gynecology, FSBEI HE Northwest State Medical University named after I.I. Mechnikov Ministry of Health of Russia (St. Petersburg, Russia).
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.
Galia F. Kutusheva, MD, professor, professor of the Department of Obstetrics and Gynecology FSBEI HE First St. Petersburg State Medical University named
after Acad. I.P. Pavlova Ministry of Health of Russia. Russia, 197022 St. Petersburg, ul. Lev Tolstoy, d. 6-8.
Elena V. Bakhidze, MD, Leading Researcher, Department of Oncogynecology, FSBI National Medical Research Center for Oncology named after N.N. Petrova of the Ministry of Health of Russia (St. Petersburg), associate professor of the Department of Obstetrics and Gynecology, FSBEI HE Northwest State Medical University named
after I.I. Mechnikov Ministry of Health of Russia.
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.
Igor V. Berlev, MD, professor, head of the scientific department of oncogynecology, Federal State Budgetary Institution National Medical Research Center for Oncology named after N.N. Petrova Ministry of Health of Russia (St. Petersburg), head of the Department of Obstetrics and Gynecology North-West State Medical University named
after I.I. Mechnikov Ministry of Health of Russia (St. Petersburg, Russia). Email: iberlev@gmail.com
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.
Adiliya F. Urmancheeva, MD, professor, leading researcher of the surgical oncogynecological department of the Federal State Budgetary Institution of Oncology named after N.N. Petrova, Ministry of Health of Russia (St. Petersburg), Professor, Department of Oncology, FSBEI HE Northwest State Medical University named after I.I. Mechnikov Ministry of Health of Russia. E-mail: adaurm@mail.ru
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68
Elena A. Ulrich, MD, professor, oncologist of the Department of Oncogynecology of the National Medical Research Center of Oncology named after N.N. Petrova of the Ministry of Health of Russia (St. Petersburg), professor at the Department of Obstetrics and Gynecology with a course of pediatric gynecology SBEI HPE FSBEI HE First
St. Petersburg State Medical University named after Acad. I.P. Pavlova (St. Petersburg, Russia) of the Ministry of Health of Russia. E-mail: elenaulrikh@mail.ru
197758 Russia, St. Petersburg, pos. Pesochny, st. Leningradskaya 68.

For citation: Safronova K.V., Artem’eva A.S., Kozlova E.N., Sidoruk A.A., Kutusheva G.F., Bakhidze E.V., Berlev I.V., Urmancheeva A.F., Ul’rikh E.A. Rare vulvar malignancies: sarcomas, melanoma, Paget’s disease.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2020; 1(Suppl): 121-130. (in Russian)
https://dx.doi.org/10.18565/aig.2020.1suppl.121-130

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