Abstract
We describe the case of a primary cervical tumor in a 6-year-old child that was originally suspected to be an embryonal rhabdomyosarcoma botryoides. Histologic analysis revealed a clear cell adenocarcinoma. Despite a direct search and questioning for maternal exposure to diethylstilbestrol, this was not documented. Clear cell adenocarcinoma is an extremely rare neoplasm that should be kept in the differential diagnosis of cervicovaginal lesions in children, even in the absence of a clinical history of in utero diethylstilbestrol exposure. We discuss the hypothesized pathogenesis and review the literature on this unusual tumor.
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Tavassoli FA, Deville P. WHO Classification Tumours of the Breast and Female Genital Organs, Volume 6. Lyon: IARC Press, 2003;297–301
Blythe JG, Michael H, Hodel KA. Colposcopic and pathologic features in two cases of DES-related vaginal clear-cell adenocarcinoma. J Reprod Med 1983;28:137–145
Waggoner SE, Anderson SM, Van Eyeck S, Fuller J, Luce MC, Herbst AL. Human papillomavirus detection and p53 expression in clear-cell adenocarcinoma of the vagina and cervix. Obstet Gynecol 1994;84:404–408
Greenwald P, Barlow J, Nasca PC, Burnett W. Vaginal cancer after maternal treatment with synthetic estrogens. N Engl J Med 1971;285:390–392
Gilson MD, Dibona DD, Knab DR. Clear cell adenocarcinoma in young females. Obstet Gynecol 1973;41:494–500
Melnick S. Cole P. Anderson D. Herbst A. Rates and risks of diethylstilbestrol-related clear-cell adenocarcinoma of the vagina and cervix. An update. N Engl J Med 1971:316:514–516
Boyd J, Takahashi H, Waggoner SE, Jones LA, Hajek RA, Wharton JT, Liu FS, Fujino T, Barrett JC, McLachlan JA. Molecular genetic analysis of clear cell adenocarcinomas of the vagina and cervix associated and unassociated with diethylstilbestrol exposure in utero. Cancer 1996;77:505–513
Waggoner SE, Baunoch DA, Anderson SA, Leigh F, Zagaja VG. Bcl-2 protein expression associated with resistance to apoptosis in clear cell adenocarcinomas of the vagina and cervix expressing wild-type p53. Ann Surg Oncol 1998;5:544–547
Waggoner SE, Anderson SM, Luce MC, Takahashi H, Boyd J. p53 protein expression and gene analysis in clear cell adenocarcinoma of the vagina and cervix. Gynecol Oncol. 1996;60:339–344
Resnik E, Christopherson WA, Stock R. Clear cell adenocarcinoma of the cervix and vagina in a woman with mixed gonadal dysgenesis. A case report. J Reprod Med 1989;34:981–984
Hanselaar A, Van Loosbroek M,Schuurbiers O,Helmerhorst T, Bulten J, Bernheim J. Clear cell adenocarcinoma of the vagina and cervix. An update of the Central Netherlands Registry showing twin age incidence peaks. Cancer 1997;79:2229–2236
Yavuz H. Arslan A. Avsaz AF. Vaginal clear cell adenocarcinoma in an infant. Pediatr Hematol Oncol 1996;13:577–580
Herbst AL, Scully RE. Adenocarcinoma of the vagina in adolescence. A report of 7 cases including 6 clear-cell carcinomas (so-called mesonephromas). Cancer 1970;25:745–757
Matias-Guiu X, Lerma E, Prat J. Clear cell tumors of the female genital tract. Semin Diagn Pathol 1997;14:233–239
Herbst AL, Ulfelder H, Poskanzer DC. Adenocarcinoma of the vagina. Association of maternal stilbestrol therapy with tumor appearance in young women. N Engl J Med 1971;284:878–881
Mittendorf R. Teratogen update: carcinogenesis and teratogenesis associated with exposure to diethylstilbestrol (DES) in utero. Teratology 1995;51:435–445
Netzloff ML. Induction of urogenital neoplasia and abnormalities from prenatal exposure to diethylstilbestrol. Ann Clin Lab Sci 1979;9:263–267
Monaghan JM, Sirisena L. Stilboestrol and vaginal clear-cell adenocarcinoma syndrome. Br Med J 1978;1:1588–1590
Reich O, Tamussino K, Lahousen M, Pickel H, Haas J, Winter R. Clear cell carcinoma of the uterine cervix: pathology and prognosis in surgically treated stage IB-IIB disease in women not exposed in utero to diethylstilbestrol. Gynecol Oncol 2000;76:331–335
Robboy ST, Kaufman RH, Prat J, Welch WR, Gaffey T, Scully RE, Richart R, Fenoglio CM, Virata R, Tilley BC. Pathologic findings in young women enrolled in the National Cooperative Diethylstilbestrol Adenosis (DESAD) project. Obstet Gynecol 1979;53:309–317
Nappi O, Mills SE, Swanson PE, Wick MR. Clear cell tumors of unknown nature and origin. A systematic approach to diagnosis. Semin Diagn Pathol 1997;14:164–174
Accetta GS, Rivoire AW, Monego IH, Vettori DA, De Oliveira Freitas DM, Edelweiss MA, Capp E. Vaginal adenosis in a non-diethylstilbestrol-exposed 6-year-old patient. Gynecol Obstet Invest 2001;51:271–273
Kurman RJ, Scully RE. The incidence and histogenesis of vaginal adenosis. An autopsy study. Hum Pathol 1974;5:265–276
Doshi N, Fujikura T, Kanbour A. Vaginal adenosis and polycystic ovaries during infancy and childhood. Am J Obstet Gynecol 1977;129:374–878
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Ahrens, W.A., Barrón-Rodriguez, L.P., McKee, M. et al. Clear Cell Adenocarcinoma of the Cervix in a Child without In Utero Exposure to Diethylstilbestrol: A Case Report and Review of the Literature. Pediatr Dev Pathol 8, 690–695 (2005). https://doi.org/10.1007/s10024-005-0047-2
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DOI: https://doi.org/10.1007/s10024-005-0047-2