Rare vulvar malignancies: sarcomas, melanoma, Paget’s disease
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.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.
Keywords
sarcoma
melanoma
extramammary Paget disease
vulva
References
- Newman P.L., Fletcher C.D. Smooth muscle tumours of the external genitalia: clinicopathological analysis of a series. Histopathology 1991; 18(6): 523–9. doi: 10.1111/j.1365-2559.1991.tb01479.x
- Salehin D., Haugk C., William M., et al. Leiomyosarcoma of the vulva. Eur J Gynaecol Oncol. 2012; 33: 306–8. PMID: 22873106
- Dewdney S., Kennedy C.M., Galask R.P. Leiomyosarcoma of the vulva: A case report. J Rep Med. 2005; 50: 630–32. PMID: 16220773
- Shankar S., Todd P.M., Rytina E., et al. Leiomyosarcoma of the vulva. J Eur Acad Dermatol Venerol. 2006; 20:116–7. doi: 10.1111/j.1468-3083.2006.01341.x ·
- González-Bugatto F., Añón-Requena M.J., López- Guerrero M.A., et al. Vulvar leiomyosarcoma in Bartholin’s gland area: A case report and literature review. Arch Gynecol Obstet. 2009; 279: 171–4. doi: 10.1007/s00404-008-0652-1.
- Mensch L.S., Trask C.E., Eltabbakh G.H. Leiomyosarcoma of the vulva: A brief communication. Eur J Gynaecol Oncol. 200; 21: 61. PMID: 10726621
- Rawal N., Saridogan E., Khan N., et al. Leiomyosarcoma of the vulva in association with lichen sclerosus. J Obstet Gynaecol. 2005; 25: 87–8. DOI: 10.1080/01443610400025549
- Zucker P.K., Peng T.C. Vulvar leiomyosarcoma in pregnancy. Am J Obstet Gynecol 1990;162: 164–6. doi: 10.1016/0002-9378(90)90841-t
- Lösch A., Joura E.A., Stani J., et al. Leiomyosarcoma of the vulva. A case report. J Rep Med. 2001; 46: 609–12.
- Nielsen G.P., Rosenberg A.E., Koerner F.C., et al. Smooth-muscle tumors of the vulva: A clinicopathological study of 25 cases and review of the literature. Am J Surg Pathol. 1996; 20: 779–93. doi: 10.1097/00000478-199607000-00001
- Otoide V., Okobia M., Aligbe J., et al. (2002)Vulvar leiomyosarcoma: A case report in a Nigerian woman. Surg Tech Int. 2002;10:71–3. PMID: 12384866
- Levy R.A., Winham W.M., Bryant C.S., et al. Smooth muscle neoplasms of the vulva masquerading as Bartholin gland duct cysts. Proceedings. 2014; 27: 25–7. doi: 10.1080/08998280.2014.11929043
- Williams N.P., Williams E., Fletcher H. Smooth muscle tumors of the vulva in Jamaica. West Ind Med J. 2002; 51: 228–31. PMID: 12632638
- Patnayak R., Manjulatha B., Srinivas S., et al. Leiomyosarcoma of the vulva. Ind J Pathol Microbiol. 2008; 51: 448–9. doi: 10.4103/0377-4929.42525
- Kuyumcuoğlu U., Kale A. Homologous type of malignant mixed Mullerian tumor of the uterus presenting as a cervical mass. J Chin Med Assoc. 2009; 72: 533–5. doi: 10.1016/S1726-4901(09)70423-X.
- Guy J.B., Trone J.C., Casteillo F., et al. Carcinosarcomas in female genital tracts: General review. Bulletin du Cancer. 2014; 101: 760–4. doi: 10.1684/bdc.2014.1937
- Grigore M., Ilea C., Terinte C., et al. Heterologous type of malignant mixed Müllerian tumor of the uterus presenting as a vulvar mass. Eur J Gynaecol Oncol. 2014; 35: 458–60. PMID: 25118493
- Bogani G., Cromi A., Uccella S., et al. Dermatofibrosarcoma protuberans of the vulva. J Obstet Gynaecol 2014;14:1–2. doi: 10.3109/01443615.2014.935726.
- Ozmen E., Güney G., Algin O. Magnetic res- onance imaging of vulvar dermatofibrosarcoma pro- tuberans – report of a case. Radiol Oncol. 2013; 47: 244–6. doi: 10.2478/raon-2013-0039.
- Hammonds L.M., Hendi A. Dermatofibrosarcoma protuberans of the vulva treated using mohs micrographic surgery. Dermatol Surg. 2010; 36: 558–63. doi: 10.1111/j.1524-4725.2010.01493.x.
- Neff R., Collins R., Backes F., et. al. Dermatofibrosarcoma protuberans: A rare and devastating tumor of the vulva. Gynecologic Oncology Reports. 2019; 28: 9–11. doi: 10.1016/j.gore.2019.01.006.
- Jemal A., Siegel R., Ward E., et al. Cancer Statistics 2007. Cancer J Clin. 2007; 57(1): 43–66. http://dx.doi.org/10.3322/canjclin.57.1.43
- Behtash N., Mousavi A., Tehranian A., et al. Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature. Gynecol Oncol. 2003; 91(2): 452–5. doi: 10.1016/s0090-8258(03)00539-0
- Reynolds E.A., Logani S., Moller K., Horowitz I.R. Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature. Gynecol Oncol. 2006; 103(2): 736–9. doi: 10.1016/j.ygyno.2006.03.033
- Stang A., Streller B., Eisinger B., et al. Population-based incidence rates of malignant melanoma of the vulva in Germany. Gynecol Oncol. 2005; 96: 216–21. doi: 10.1016/j.ygyno.2004.09.052
- Sugiyama V.E., Chan J.K., Shin J.Y., et al. Vulvar melanoma: a multivariable analysis of 644 patients. Obstet Gynecol. 2007; 110: 296–301. doi: 10.1097/01.AOG.0000271209.67461.91
- Aulmann S., Sinn H.P., Penzel R., et al. Comparison of molecular abnormalities in vulvar and vaginal melanomas. Mod Pathol. 2014: 27: 1386–93.
- Ragnarsson-Olding B., Johansson H., Rutqvist L.E., et al. Malignantmelanoma of the vulva and vagina. Trends in incidence, age distribution, and long-term survival among 245 consecutive cases in Sweden 1960–1984. Cancer. 1993; 71: 1893–7. doi: 10.1002/1097-0142(19930301)71:5<1893::aid-cncr2820710528>3.0.co;2-7
- Pleunis N., Schuurman M.S., VanRossum M.M., et al. Rare vulvar malignancies; incidence, treatment and survival in the Netherlands. Gynecol Oncol. 2016; 142: 440–5. doi: https://doi.org/10.1016/j.ygyno.2016.04.021
- Omholt K., Grafstrom E., Kanter Lewensohn L. KIT pathway alterations in mucosal melanomas of the vulva and other sites. Clin Cancer Res. 2001; 17: 3933–42. doi: 10.1158/1078-0432.CCR-10-2917.
- Aulmann S., Sinn H.P., Penzel R., et al. Comparison of molecular abnormalities in vulvar and vaginal melanomas. Mod Pathol. 2014; 27: 1386–93. http://dx.doi.org/10.1038/modpathol.2013.211
- Kirschner A.N., Kidd E.A., Dewees T., Perkins S.M. Treatment approach and outcomes of vaginal melanoma. Cancer. 2013; 23:1484–9. doi: 10.1097/IGC.0b013e3182a1ced8.
- Buchanan D.J., Schlaerth J., Kurosaki T. Primary vaginal melanoma: thirteen-year disease-free survival after wide local excision and review of recent literature. Am J Obstet Gynecol. 1998;178:1177–84. doi: 10.1016/s0002-9378(98)70320-5
- Leitao Jr.M.M.., Cheng X., Hamilton A.L., et al. Gynecologic Cancer Inter Group (GCIG) consensus review for vulvovaginal melanomas. Int J Gynecol Cancer 2014; 24(9 Suppl 3): S117–22. doi: 10.1097/IGC.0000000000000198.
- Janco J.M., Markovic S.N., Weaver A.L., et al. Vulvar and vaginal melanoma: case series and review of current management options including neoadjuvant chemotherapy. Gynecol Oncol. 2013;129: 533–7. doi: 10.1016/j.ygyno.2013.02.028.
- Gökaslan H., Sişmanoğlu A., Pekin T., et al. Primary malignant melanoma of the vagina: a case report and review of the current treatment options. Eur J Obstet Gynecol Reprod Biol. 2005; 121: 243–8. doi: 10.1016/j.ejogrb.2004.11.048
- Sinasac S.E., Petrella T.M., Rouzbahman M., et al. Melanoma of the vulva and vagina: surgical management and outcomes based on a clinicopathologic review of 68 cases. J Obstet Gynaecol Can. 2019; 41(6): 762–71. doi: 10.1016/j.jogc.2018.07.011.
- Frumovitz M., Etchepareborda M., Sun C.C., et al. Primary malignant melanoma of the vagina. Obstet Gynecol. 2010; 116:1358–65. doi:10.1097/AOG.0b013e3181fb8045
- Huang Q., Huang H., Wan T., et al. Clinical outcome of 31 patients with primary malignant melanoma of the vagina. J Gynecol Oncol. 2013; 24: 330–5. doi: 10.3802/jgo.2013.24.4.330
- Dubreuilh, W. Paget’s disease of the vulva. Br. J. Dermatol. 1901; 13(11): 407–13.
- Fanning, J., Lambert H.C., Hale T.M., Morris P.C., Schuerch C. Paget’s disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget’s disease, and recurrence after surgical excision. Am J Obstet Gynecol. 1999;180(1): 24–7. DOI: 10.1016/s0002-9378(99)70143-2
- Preti M., Micheletti L., Ghiringhello B., et al., [Vulvar paget’s disease. Clinico- pathologic review of the literature]. Minerva Ginecol. 2000; 52(5): 203–11. [Article in Italian]. PMID: 11048477
- Kurman R.J., Carcangiu M.L., Herrington C.S., Young R.H. WHO Classification of Tumours of Female Reproductive Organs. Lyon, 2014; 6: ISBN-13 (PDF)978-92-832-4487-5
- Lynch, P., Moyal-Barracco M., Scurry J., Stockdale C. 2011 ISSVD terminology and classification of vulvar dermatological disorders: an approach to clinical diagnosis. J Low Genit Tract Dis. 2012; 16(4): 339–44.doi: 10.1097/LGT.0b013e3182494e8c.
- De Magnis A., Checcucci V., Catalano C., Corazzesi A., Pieralli A., Taddei G., Fambrini M. Vulvar paget disease: a large single-centre experience on clinical presentation, surgical treatment, and long-term outcomes. J Low Genit Tract Dis. 2013; 17(2): 104e110. doi: 10.1097/LGT.0b013e31826569a9.
- Mendivil A.A., Abaid L., Epstein H.D., Rettenmaier M.A., Brown J.V. 3rd, Micha J.P., Wabe M.A., Goldstein B.H. Paget’s disease of the vulva: a clinicopathologic institutional review. Int J Clin Oncol. 2012; 17(6): 569–74. doi: 10.1007/s10147-011-0325-0.
- van der Linden M., Meeuwis K.A., Bulten J., Bosse T., van Poelgeest M.I., de Hullu J.A., Paget disease of the vulva. Crit Rev Oncol Hematol. 2016; 101: 60e74. https://doi.org/10.1016/j.critrevonc.2016.03.008
- Hatta, N., Yamada M., Hirano T., Fujimoto A., Morita R. Extramammary Paget’s disease: treatment, prognostic factors and outcome in 76 patients. Br J Dermatol. 2008; 158(2): 313–8. doi: 10.1111/j.1365-2133.2007.08314.x
- Ito, Y., Igawa S, Ohishi Y, Uehara J, Yamamoto AI, Iizuka H. Prognostic indicators in 35 patients with extramammary Paget’s disease. Dermatol Surg. 2012; 38(12):1938–44. doi: 10.1111/j.1524-4725.2012.02584.x.
- Gaarenstroom, K.N., Kenter G.G., Trimbos J.B., Agous I., Amant F., Peters A.A., Vergote I. Postoperative complications after vulvectomy and inguinofemoral lymphadenectomy using separate groin incisions. Int J Gynecol Cancer. 2003; 13(4): 522–7. doi: 10.1046/j.1525-1438.2003.13304.x
- Senn B., Mueller M.D., Cignacco E.L., Eicher M. Period prevalence and risk factors for postoperative short-term wound complications in vulvar cancer: a cross-sectional study. Int J Gynecol Cancer. 2010; 20(4): 646–54. doi: 10.1111/IGC.0b013e3181d92723.
- Robison, K., Roque D., McCourt C., Stuckey A., DiSilvestro P.A., Sung C.J., Steinhoff M., Granai C.O., Moore R.G. Long-term follow-up of vulvar cancer patients evaluated with sentinel lymph node biopsy alone. Gynecol Oncol. 2014; 133(3): 416–20. doi: 10.1016/j.ygyno.2014.03.010
- Gunther, V., Malchow B, Schubert M, Andresen L, Jochens A, Jonat W, Mundhenke C., Alkatout I. Impact of radical operative treatment on the quality of life in women with vulvar cancer—a retrospective study. Eur J Surg Oncol. 2014; 40(7): 875–82. doi: 10.1016/j.ejso.2014.03.027
- Perez, D.R., Trakarnsanga A., Shia J., Nash G.M., Temple L.K., Paty P.B., Guillem J.G., Garcia-Aguilar J., Weiser M.R. Management and outcome of perianal Paget’s disease: a 6-decade institutional experience. Dis Colon Rectum. 2014; 57(6): 747–51. doi: 10.1097/DCR.0000000000000100.
- Winters, U., Daayana S., Lear J.T., Tomlinson A.E., Elkord E., Stern P.L., Kitchener H.C. Clinical and immunologic results of a phase II trial of sequential imiquimod and photodynamic therapy for vulval intraepithelial neoplasia. Clin Cancer Res. 2008; 14(16): 5292–9. doi: 10.1158/1078-0432.CCR-07-4760.
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.ru197758 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