Non-diethylstilbestrol exposed vaginal adenocarcinoma in young patients associated with unilateral renal agenesis: two case reports and literature review

Background: Adenocarcinoma, accounts for up to14% of all vaginal cancer. In young patients, common histological feature is clear cell adenocarcinoma (CCA) while mesonephric adenocarcinoma (MA) is very rare. The authors report two patients in their early twenties with unilateral renal agenesis and vaginal adenocarcinoma not exposed to diethylstilbestrol (DES). Cases: Two patients with vaginal adenocarcinoma were treated, with external beam radiotherapy of pelvis combined with brachytherapy to a radical dose. In 2000, 25-year-old female, was admitted for radiotherapy after incomplete excision of the tumor localized in left vaginal apex and fornix. Histopathology confirmed CCA and classified as clinical Stage II. CT revealed left renal agenesis.The patient is alive and disease-free 15 years after therapy. Vaginal, urethral stenosis, and hydronephrosis occurred and ureteral stent was inserted. In the second patient, 22- year-old, in 2004, after biopsy of bulky tumor of vagina and histology, revealed MA in Stage III and CT scan also confirmed right renal agenesis. Radiotherapy was followed by chemotherapy. After 11 years, patient is disease-free with vaginal stenosis and incipient renal hydronephrosis. Conclusion: Radiotherapy is effective treatment in advance vaginal adenocarcinoma, however, with high morbidity. The authors advise rigorous gynecologic exams in young females with renal agenesis as there may be a risk of malignant changes in vagina.

Vaginal adenocarcinoma; Renal agenesis; Radiotherapy.

[1] Creasman W.T., Phillips J.L., Menck H.R.: “The national cancer data base report on cancer of vagina”. Cancer, 1998, 83, 1033.

[2] Zeeshan-ud-din, Ahsan A.: “Vaginal clear cell adenocarcinoma with associated Müllerian duct anomalies, renal agenesis and situs inversus: report of a case with no known in-utero exposure with diethyl stilboestrol”. J. Pak. Med. Assoc., 2009, 59, 568.

[3] Ott M.M., Rehn M., Müller J.G., Gruss A., Martius J., Steck T., Müller-Hermelink H.K.: “Vaginal clear cell carcinoma in a young patient with ectopic termination of the left ureter in the vagina”. Virchows. Arch., 1994, 425, 445.

[4] Li S., Qayyum A., Coakley F.V., Hricak H.: “Association of renal agenesis and mullerian duct anomalies”. J. Comp. Assis. Tomography, 2000, 24, 829.

[5] Senekijan E.K., Frey K.W., Stone C., Herbst A.l.: “An evaluation of stage II vaginal clear cell adenocarcinoma according to substages”. Gynecol. Oncol., 1988, 31, 56.

[6] Waggoner S.E., Baunoch D.A., Anderson S.A., Leigh F., Zagaja V.G.: “Bcl-2 protein expression associated with resistance to apoptosis in clear cell adenocarcinomas of the vagina and cervix expressing wild-type p53”. Ann. Sur. Oncol., 1998, 5, 544.

[7] Tewari S.D., McHale M.T., Kuo J.V., Monk B.J., Burger R.A.: “Primary Invasive Vaginal cancer in setting of the Mayer-rokitanskykuster- hauser syndrome”. Gynecol. Oncol., 2002, 85, 384.

[8] Uehara T., Onda T., Sasajima Y., Sawada M., Kasamatsu T.: “A case of vaginal clear cell adenocarcinoma complicated with congenital anomalies of the genitourinary tract and metanephric remnant without prenatal diethylstilbestrol exposure”. J. Obstet. Gynaecol. Res., 2010, 36, 681.

[9] Watanabe Y., Etoh T., Nakai H.: “Adenocarcinoma of the lower female genital tract in patient with Herlyn-Werner-Wunderlich syndrome”. Am. J. Obstet. Gynecol., 2012, 207, e5.

[10] Bifulco G., Mandato V.D., Mignogna C., Giampaolino P., Di Spiezio Sardo A., De Cecio R., et al.: “A case of mesonephric adenocarcinoma of the vagina with a 1-year follow-up”. Int. J. Gynecol. Cancer, 2008, 18, 1127.

[11] Ersahin C., Huang M., Potkul R.K., Hammadeh R., Salhadar A.: “Mesonephric adenocarcinoma of the vagina with a 3-year follow up”. Gynecol. Oncol., 2005, 99, 757.

[12] McNail R.Y., Nowicki P.D., Miller B., Billups C., Liu T., Daw N.C.: “Adenocarcinoma of the cervix and vagina in pediatric patient”. Pediatr. Blood Cancer, 2004, 43, 289.

[13] Perez C.A., Grigsby P.W., Garipagaoglu M.,Lockett M.A.: “Factors affecting long-therm outcome of irradiation in carcinoma of the vagina”. Int. J. Radiat. Oncol. Biol. Phys., 1999, 44, 37.

[14] Frank S.J., Deavers M.T., Jhingran A., Bodurka D.C., Eifel P.J.:”Primary adenocarcinoma of the vagina not associated with diethylstilbestrol (DES) exposure”. Gynecol. Oncol., 2007, 105, 470.

[15] Kurman R.J., Norris H.J., Wilkinson E.: “Tumors of the cervix, vagina and vulva”. In: Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology, 1992.

[16] Kirkbride P., Fyles A., Rawlings A.:”Carcinoma of the vagina – experience at the Princess Mrgaret Hospital (1974-1989)”. Gynecol. Oncol., 1995, 56, 435.

[17] Jones W.B., Tan L.K., Lewis J.L. Jr.: “Late recurrence of clear cell adenocarcinoma of the vagina and cervix: a report of three cases”.Gynecol. Oncol., 1993, 51, 266.