Germ Cell Tumors of the Vulva
Synonyms
Endodermal sinus tumor; Yolk sac tumor.
Definition
Germ cell tumors are a group of tumors that arise from the primitive germ cell elements with yolk sac tumor being the most common variant.
Clinical Features
Incidence
Primary germ cell tumors of the vulva are extremely rare. They are believed to arise from the germ cells that persist during embryonic life.
Age
Patients range in age from 1 to 52 with a mean age of 19 years.
Site
Vulva.
Treatment
Local excision followed by adjuvant chemotherapy.
Outcome
Vulvar yolk sac tumor is a rare and aggressive form of germ cell tumor which recurs usually within 12 months of initial diagnosis (Mochizuki et al. 2012) and has a high risk of distant spread (Flanagan et al. 1997). Inguinal lymph nodes are the most common site of recurrence, and lungs are the most common site of distant metastasis (Xu et al. 2015). Tumor size of 5 cm or less is considered favorable prognostic (Basgul et al. 2006).
Macroscopy
Tumors...
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References and Further Reading
Basgul, A., Gokaslan, H., Kavak, Z. N., Eren, F. T., & Bozkurt, N. (2006). Primary yolk sac tumor (endodermal sinus tumor) of the vulva: Case report and review of the literature. European Journal of Gynaecological Oncology, 27(4), 395–398. Retrieved from https://www.ncbi.nlm.nih.gov/pubmed/17009634
Buras, A. L., McSwain, A. N., Qin, Y., & Meunier, J. K. (2015). Primary mycosis fungoides of the vulva: The first reported case. Gynecologic Oncology Reports, 12, 7–8. https://doi.org/10.1016/j.gore.2015.01.006.
Clemente, N., Alessandrini, L., Rupolo, M., Bulian, P., Lucia, E., Canzonieri, V., & Sopracordevole, F. (2016). Primary non-Hodgkin’s lymphoma of the vulva: A case report and literature review. Medicine (Baltimore), 95(10), e3041. https://doi.org/10.1097/MD.0000000000003041.
Flanagan, C. W., Parker, J. R., Mannel, R. S., Min, K. W., & Kida, M. (1997). Primary endodermal sinus tumor of the vulva: A case report and review of the literature. Gynecologic Oncology, 66(3), 515–518. https://doi.org/10.1006/gyno.1997.4759.
Geller, S., Pitter, K., Moskowitz, A., Horwitz, S. M., Yahalom, J., & Myskowski, P. L. (2018). Treatment of vulvar mycosis fungoides tumors with localized radiotherapy. Clinical Lymphoma, Myeloma & Leukemia, 18(7), e279–e281. https://doi.org/10.1016/j.clml.2018.04.010.
Khunamornpong, S., Siriaunkgul, S., Suprasert, P., & Chitapanarux, I. (2005). Yolk sac tumor of the vulva: A case report with long-term disease-free survival. Gynecologic Oncology, 97(1), 238–242. https://doi.org/10.1016/j.ygyno.2004.12.054.
Mochizuki, K., Obatake, M., Taura, Y., Inamura, Y., Takatsuki, M., Nagayasu, T., & Eguchi, S. (2012). Yolk sac tumor of the vulva: A case report with recurrence after long-term follow-up. Pediatric Surgery International, 28(9), 931–934. https://doi.org/10.1007/s00383-012-3153-z.
Winnicki, M., Gariepy, G., Sauthier, P. G., & Funaro, D. (2009). Hodgkin lymphoma presenting as a vulvar mass in a patient with Crohn’s disease: A case report and literature review. Journal of Lower Genital Tract Disease, 13(2), 110–114. https://doi.org/10.1097/LGT.0b013e318187a7ee.
Xu, W., Moon, A., Chetty, N., Lourie, R., & Shannon, C. (2015). Vulvar yolk sac tumor diagnosed during pregnancy, with recurrence during subsequent second pregnancy. Gynecologic Oncology Reports, 12, 67–71. https://doi.org/10.1016/j.gore.2015.03.006.
Ye, A. L., Willis, M. S., Link, B. K., Naridze, R. L., Syrbu, S. I., & Liu, V. (2018). Primary diffuse large B cell lymphoma of the vulva-two new cases of a rare entity and review of the literature. JAAD Case Reports, 4(9), 962–967. https://doi.org/10.1016/j.jdcr.2018.06.023.
Yin-Yi Chang, C., Wu, K. H., Lin, T. Y., Chang, W. C., Lin, W. C., Hung, Y. C., & Yeh, L. S. (2011). Vulvar yolk sac tumor mixed with embryonal carcinoma in a peri-pubertal girl: A case report. Taiwanese Journal of Obstetrics & Gynecology, 50(4), 503–505. https://doi.org/10.1016/j.tjog.2011.10.018.
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Andea, A., Chand, M. (2023). Germ Cell, Lymphoid, Myeloid, and Secondary Tumors, Pathology of the Vulva. In: Stolnicu, S., Ali-Fehmi, R. (eds) Gynecologic Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-97378-0_5591
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