Rare tumor-like masses in the vulva

Zharov A.V., Kolesnikova E.V., Penzhoyan G.A., Dryaeva L.G.

1) Kuban State Medical University, Ministry of Health of the Russian Federation, Krasnodar, Russia; 2) Regional Clinical Hospital No 2, Ministry of Health of the Krasnodar Region, Krasnodar, Russia
Background. The reason for the increase in the rare pathology of the vulva is, firstly, the accumulation of genetic breakdowns in the population, the study of which has recently intensified, and, secondly, the possibilities of modern diagnosis, which allow for verifying the process, have improved. However, information about the rare tumor-like pathology of the vulva is still insufficient.
Objective. To optimize the diagnosis of rare tumor-like processes in the female external genitalia.
Case report. Based on international experience, the authors analyze their two own clinical cases. One patient had Behcet’s disease with a classic triad of symptoms; the other had aberrant breast tissue in the vulva. In both cases, the final diagnosis was made with a significant delay after the first signs of the disease appeared. The history data, that had not taken into account during the patients’ first visit and served as a reason for late diagnosis, were clarified. Complaints, the type of a pathological focus in the vulvar area, and clinical features are described.
Conclusion. The accumulation of genetic abnormalities in the population increasingly leads to their diverse phenotypic manifestations, inter alia with the localization in the vulva. For timely diagnosis and selection of the optimal treatment option, there is a need for the further accumulation of clinical experience and its coverage in the specialized medical literature.

Keywords

Behcet’s disease
aberrant breasts
vulva

References

  1. Сафронова К.В., Артемьева А.С., Козлова Е.Н., Сидорук А.А., Кутушева Г.Ф., Бахидзе Е.В., Берлев И.В., Урманчеева А.Ф., Ульрих Е.А. Редкие злокачественные опухоли вульвы: саркомы, меланома, болезнь Педжета. Акушерство и гинекология. 2020; 1(Приложение): 121-30. [Safronova K.V., Artem’eva A.S., Kozlova E.N., Sidoruk A.A., Kutusheva G.F., Bakhidze E.V., Berlev I.V., Urmancheyeva A.F., Ulrikh E.A. Rare vulvar malignancies: sarcomas, melanoma, Paget’s disease. Obstetrics and Gynecology. 2020; 1(Suppl): 121-30. (in Russian)]. https://dx.doi.org/10.18565/aig.2020.1suppl.121-130.
  2. Свиридов Е.Г., Кадыкова А.И., Редько Н.А., Дробышев А.Ю., Деев Р.В. Генетическая гетерогенность опухолеподобных поражений костей че-люстно-лицевой области. Гены & Клетки. 2019; 14(1): 49-54. [Sviridov E.G., Kadykova A.I., Redko N.A., Drobyshev A.Yu., Deev R.V. Genetic he­­tero­geneity of tumor-like bone lesions of the maxillofacial area. Genes & Cells. 2019; 14(1): 49-54. (in Russian)]. https://dx.doi.org/10.23868/201903006.
  3. Puccetti A., Fiore P.F., Pelosi A., Tinazzi E., Patuzzo G., Argentino G. et al. Gene expression profiling in Behcet’s disease indicates an autoimmune component in the pathogenesis of the disease and opens new avenues for targeted ther-apy. J. Immunol. Res. 2018; 2018: 4246965. https://dx.doi.org/10.1155/2018/4246965.
  4. Пинский С.Б., Колмаков С.А., Высоцкий В.Ф., Свистунов В.В. Редкие доброкачественные гормонально-неактивные образования надпочечников. Сибирский медицинский журнал. 2007; 71(4): 93-7. [Pinskii S.B., Kolmakov S.A., Vysotskii V.F., Svistunov V.V. Rare benign hormone-inactive formations of the adrenal glands. Siberian Medical Journal. 2007; 71(4): 93-7. (in Russian)].
  5. Behcet H. Uber rezidivierende, aphthose, durch ein virus verursachte Geschwure am Mund, am Auge und an den Genitalien. Dermatol. Wochenschr. 1937; 105: 1152-7.
  6. Kim E.S., Chung W.C., Lee K.M., Lee B.I., Choi H., Han S.W. et al. A case of intestinal Behcet's disease similar to Crohn's colitis. J. Korean Med. Sci. 2007; 22(5): 918-22. https://dx.doi.org/10.3346/jkms.2007.22.5.918.
  7. Кауфман Р.Х. Разнообразные заболевания вульвы. В кн.: Кауфман Р., Фаро С., Браун Д. Доброкачественные заболевания вульвы и влагалища. Пер. с англ. М.: БИНОМ; 2009: 352-97. [Kaufman R.Kh. A variety of vulva diseases. In book: Kaufman R., Faro S., Braun D. Benign diseases of the vulva and vagina. Trans. from English M.: BINOM. 2009: 352-97. (in Russian)].
  8. Хамитов Р.Ф., Пальмова Л.Ю., Якупова З.Н., Хасанова Э.Р. Болезнь Бехчета в терапевтической клинической практике. Казанский медицинский журнал. 2013; 94(4): 545-8. [Khamitov R.F., Palmova L.Yu., Iakupova Z.N., Khasanova E.R. Behchet's disease in therapeutic clinical practice. Kazan Medical Journal. 2013; 94(4): 545-8. (in Russian)].
  9. Голоева Р.Г., Алекберовава З.С., Лапина Т.В. Поражение легких при болезни Бехчета. Научно-практическая ревматология. 2009; 47(4): 90-6. [Goloeva R.G., Alekberovava Z.S., Lapina T.V. Lung damage in Behchet's disease. Scientific and practical rheumatology. 2009; 47(4): 90-6. (in Russian)].
  10. Mat C., Yurdakul S., Sevim A., Özyazgan Y., Tüzün Y. Behcet’s syndrome: facts and controversies. Clin. Dermatol. 2013; 31(4): 352-61. https://dx.doi.org/10.1016/j.clindermatol.2013.01.002.
  11. Алекберова З.С., Измаилова Ф.И., Гусева И.А., Денисов Л.Н., Глухова С.И., Попкова Т.В. HLA-B5/51-генотип: связь с клиническими проявлениями болезни Бехчета. Научно-практическая ревматология. 2015; 53(4): 367-70. [Alekberova Z.S., Izmailova F.I., Guseva I.A. et al. HLA-B5/51-genotype: association with clinical manifestations of Behchet's disease. Scientific and practical rheumatology. 2015; 53(4): 367-70. (in Russian)].
  12. Hamuryudan V., Hatemi G., Sut N., Ugurlu S., Yurdakul S., Yazici H. Freqent oral ulctration during early disease may predict a severe disease course in males with Behcet’s syndrome. al. Clin. Exp. Rheumatol. 2012; 30(3, Suppl. 72): S32-4.
  13. Алекберова З.С. Болезнь Бехчета (лекция). Научно-практическая ревматология. 2013; 51(1): 52-8. [Alekberova Z.S. Behchet's disease (lecture). Sci-entific and practical rheumatology. 2013; 51(1): 52-8. (in Russian)].
  14. Alpsoy E. Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J. Dermatol. 2016; 43(6): 620-32. https://dx.doi.org/10.1111/1346-8138.13381.
  15. Leccese P. Alpsoy E. Behçet’s disease: an overview of etiopathogenesis. Front. Immunol. 2019; 10: 1067. https://dx.doi.org/10.3389/fimmu.2019.01067.
  16. Davatchi F., Assaad-Khalil S., Calamia K.T., Crook J.E., Sadeghi-Abdollahi B., Schirmer M. et al. The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J. Eur. Acad. Dermatol. Venereol. 2014; 28(3): 338-47. https://dx.doi.org/10.1111/jdv.12107.
  17. Hata H., Aoyagi S., Iitani M.M., Homma E., Shimizu H. Squamous cell carcinoma in a chronic genital ulcer in Behçet’s disease. Acta Derm. Venereol. 2010; 90(5): 539-40. https://dx.doi.org/10.2340/00015555-0902.
  18. Germann D., Catarino R., Saiji E., Daikeler T., Petignat P., Vassilakos P. Vulvar intraepithelial neoplasia grade 3 associated with Behçet's disease. BMC Res. Notes. 2016; 9: 172. https://dx.doi.org/10.1186/s13104-016-1985-7.
  19. Hartung E. Ueber einen Fall von Mamma accessoria. Erlangen; 1875. 30p.
  20. Irvin W.P., Cathro H.P., Grosh W.W., Rice L.W., Andersen W.A. Primary breast carcinoma of the vulva: a case report and literature review. Gynecol. Oncol. 1999; 73(1): 155-9. https://dx.doi.org/10.1006/gyno.1998.5269.
  21. Van der Putte S. Anogenital “sweat” glands. Histology and pathology of a gland that may mimic mammary glands. Am. J. Dermatopathol. 1991; 13(6): 557-67.
  22. Van der Putte S. Mammary-like glands of the vulva and their disorders. Int. J. Gynecol. Pathol. 1994; 13(2): 150-60.
  23. Константинова А.М., Белоусова И.Э., Кацеровска Д., Михал М., Шелехова К.В., Казаков Д.В. Морфология аногенитальных маммаро-подобных желез. Вестник Санкт-Петебургского университета. Медицина. 2017; 12(1): 83-92. [Konstantinova A.M. Belousova I.E., Kacerovska D. et al. Mor-phology of anogenital mammary-like glands. Bulletin of St. Petersburg State University. Medicine. 2017; 12(1): 83-92. (in Russian)].
  24. Konstantinova A.M., Kacerovska D., Michal M., Kazakov D.V. A tumoriform lesion of the vulva with features of mammary-type fibrocystic disease. Am. J. Dermatopathol. 2013; 35(7): e124-7. https://dx.doi.org/10.1097/DAD.0b013e318284860c.
  25. Lev-Cohain N., Kapur P., Pedrosa I. Vulvar fibroadenoma with lactational changes in ectopic breast tissue. Case Rep. Obstet. Gynecol. 2013; 2013: 924902: https://dx.doi.org/10.1155/2013/924902.
  26. Castro C.Y., Deavers M. Ductal carcinoma in-situ arising in mammary-like glands of the vulva. Int. J. Gynecol. Pathol. 2001; 20(3): 277-83. https://dx.doi.org/10.1097/00004347-200107000-00012.
  27. Tran T.A., Deavers M.T., Carlson J.A., Malpica A. Collision of ductal carcinoma in situ of anogenital mammary-like glands and vulvar sarcomatoid squamous cell carcinoma. Int. J. Gynecol .Pathol. 2015; 34(5): 487-94. https://dx.doi.org/10.1097/00004347-200107000-00012.
  28. Diniz da Costa A.T., Coelho A.M., Lourenço A.V., Bernardino M., Ribeirinho A.L., Jorge C.C. Primary breast cancer of the vulva: a case report. J. Low. Genit. Tract Dis. 2012; 16(2): 155-7. https://dx.doi.org/10.1097/LGT.0b013e31823b3bc6.
  29. Alligood-Percoco N.R., Kessler M.S., Willis G. Breast cancer metastasis to the vulva 20 years remote from initial diagnosis: a case report and literature review. Gynecol. Oncol. Rep. 2015; 13: 33-5. https://dx.doi.org/10.1016/j.gore.2015.05.002.
  30. Grewal J.K., Eirew P., Jones M. Detection and genomic characterization of a mammary-like adenocarcinoma. Cold Spring Harb. Mol. Case Stud. 2017; 3(6): a002170. https://dx.doi.org/10.1101/mcs.a002170.
  31. Козлов Л.А., Журавлева В.И. Молочная железа в вульве (редкое наблюдение). Казанский медицинский журнал. 2012; 93(5): 838-40. [Kozlov L.A., Zhuravleva V.I. Mammary gland in vulva (rare observation). Kazan Medical Journal. 2012; 93(5): 838-40. (in Russian)].

Received 08.04.2021

Accepted 12.05.2021

About the Authors

Alexandr V. Zharov, PhD, MD (Medicine), Professor, Department of Obstetrics, Gynecology and Perinatology of the FPC and PPP, Kuban State Medical University, Ministry of Health of Russia, Krasnodar, Russian Federation. E-mail: zharov.1966@yandex.ru. ORCID: 0000-0002-5460-5959. 350063, Russia, Krasnodar, Sedina str., 4.
EkaterinaV. Kolesnikova, PhD (Medicine), Associate Professor, Department of Obstetrics, Gynecology and Perinatology of the FPC and PPP, Kuban State Medical University, Ministry of Health of Russia, Krasnodar, Russian Federation. E-mail: jokagyno@rambler.ru. ORCID: 0000-0002-6537-2572. 350063, Russia, Krasnodar, Sedina str., 4.
Grigory A. Penzhoyan, PhD, MD (Medicine), Professor, Head of the Department of Obstetrics, Gynecology and Perinatology of the FPC and PPP, Kuban State Medical University, Ministry of Health of Russia, Krasnodar, Russian Federation. E-mail: pga05@mail.ru. ORCID: 0000-0002-8600-0532. 350063, Russia, Krasnodar, Sedina str., 4.
Lyudmila G. Dryaeva, PhD (Medicine), Head of the Pathology Department of the Consultative and Diagnostic Center, Regional Clinic Hospital No 2, Krasnodar, Russian Federation. E-mail: Luda_dom@mail.ru. ORCID: 0000-0003-4927-6828. 350012, Russia, Krasnodar, Krasnykh Partizan str., 6/2.

For citation: Zharov A.V., Kolesnikova E.V., Penzhoyan G.A., Dryaeva L.G. Rare tumor-like masses in the vulva.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2021; 6: 192-197 (in Russian)
https://dx.doi.org/10.18565/aig.2021.6.192-197

Similar Articles

By continuing to use our site, you consent to the processing of cookies that ensure the proper functioning of the site.