J Gynecol Oncol.  2011 Sep;22(3):211-213. 10.3802/jgo.2011.22.3.211.

Squamous cell carcinoma of the vulva in a virgin patient with Turner syndrome

• Tapisiz OL
• Topcu O
• Gungor T
• Ozdal B
• Sirvan L
• Yesilyurt

• KMID: 2055711
• DOI: http://doi.org/10.3802/jgo.2011.22.3.211

Abstract

Two types of gynecologic tumors are commonly described in the Turner syndrome, the first one is gonadoblastoma, which occurs in patients with Y chromosome abnormalities, and the second one is endometrial carcinoma which is mostly related with exogenous estrogen usage. Here, we describe an extremely rare case of squamous cell carcinoma of the vulva in a virgin woman with Turner syndrome. A 35-years old single, virgin woman referred to our Oncology Department with warty, necrotized, exophytic 6-7 cm vulvar mass. She had a history of primary amenorrhea and mosaic Turner syndrome was determined in her karyotype analysis. Biopsy specimen of the vulvar mass revealed squamous cell carcinoma of the vulva, and total vulvectomy with inguinal femoral lymphadenectomy was performed. The postoperative course was uneventful and there has been no recurrence of the disease up to date. Women with Turner syndrome have streak ovaries that produce very low estrogen and the squamous cell carcinoma of the vulva may have developed at an early age with Turner syndrome because of this low estrogen value similar to postmenopausal women. The current case is a special case due to its age of occurrence, virgin and Turner syndrome status.

Keyword

Squamous cell carcinoma; Turner syndrome; Virgin; Vulvar carcinoma

MeSH Terms

Amenorrhea
Biopsy
Carcinoma, Squamous Cell
Endometrial Neoplasms
Estrogens
Female
Gonadoblastoma
Humans
Karyotype
Lymph Node Excision
Ovary
Recurrence
Turner Syndrome
Vulva
Y Chromosome
Estrogens

Figure

• 

  Fig. 1 Microscopic finding of the patient; squamous cell carcinoma of the vulva, tumoral cell groups infiltrate the stroma irregularly (H&E, ×100).

Reference

1. Elsheikh M, Dunger DB, Conway GS, Wass JA. Turner's syndrome in adulthood. Endocr Rev. 2002. 23:120–140.

2. Chaganti RS, Bailey RB, Jhanwar SC, Arlin ZA, Clarkson BD. Chronic myelogenous leukemia in the monosomic cell line of a fertile Turner syndrome mosaic (45,X/46,XX). Cancer Genet Cytogenet. 1982. 5:215–221.

3. Cabanas P, Garcia-Caballero T, Barreiro J, Castro-Feijoo L, Gallego R, Arevalo T, et al. Papillary thyroid carcinoma after recombinant GH therapy for Turner syndrome. Eur J Endocrinol. 2005. 153:499–502.

4. Sybert VP, McCauley E. Turner's syndrome. N Engl J Med. 2004. 351:1227–1238.

5. Gadducci A, Cionini L, Romanini A, Fanucchi A, Genazzani AR. Old and new perspectives in the management of high-risk, locally advanced or recurrent, and metastatic vulvar cancer. Crit Rev Oncol Hematol. 2006. 60:227–241.

6. Madeleine MM, Daling JR, Carter JJ, Wipf GC, Schwartz SM, McKnight B, et al. Cofactors with human papillomavirus in a population-based study of vulvar cancer. J Natl Cancer Inst. 1997. 89:1516–1523.

7. Canavan TP, Cohen D. Vulvar cancer. Am Fam Physician. 2002. 66:1269–1274.

8. Stroup AM, Harlan LC, Trimble EL. Demographic, clinical, and treatment trends among women diagnosed with vulvar cancer in the United States. Gynecol Oncol. 2008. 108:577–583.

9. Jones RW, Baranyai J, Stables S. Trends in squamous cell carcinoma of the vulva: the influence of vulvar intraepithelial neoplasia. Obstet Gynecol. 1997. 90:448–452.

10. Huang FY, Kwok YK, Lau ET, Tang MH, Ng TY, Ngan HY. Genetic abnormalities and HPV status in cervical and vulvar squamous cell carcinomas. Cancer Genet Cytogenet. 2005. 157:42–48.

11. Iwamoto I, Kijima Y, Fujino T, Yoshinaga M, Douchi T. Verrucous carcinoma of the vulva in Turner syndrome. Gynecol Oncol. 2004. 92:380–383.