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 Table of Contents  
CASE REPORT
Year : 2012  |  Volume : 1  |  Issue : 4  |  Page : 239-241

Primary clear cell adenocarcinoma of the uterine cervix in a young woman not associated with diethylstilbestrol: A case report and review of literature


Department of Radiotherapy, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India

Date of Web Publication21-Jan-2013

Correspondence Address:
Tamojit Chaudhuri
Department of Radiotherapy, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow- 226014
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.106278

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  Abstract 

We report an extremely rare case of primary clear cell adenocarcinoma (CCA) of the uterine cervix in a 21-year-old Asian female, with no maternal history of diethylstilbestrol ingestion during pregnancy. A review of the relevant literature regarding the possible etiology and treatment of primary CCA of cervix is done.

Keywords: Clear cell adenocarcinoma, diethylstilbestrol, human papillomavirus


How to cite this article:
Chaudhuri T, Sharma D. Primary clear cell adenocarcinoma of the uterine cervix in a young woman not associated with diethylstilbestrol: A case report and review of literature. Clin Cancer Investig J 2012;1:239-41

How to cite this URL:
Chaudhuri T, Sharma D. Primary clear cell adenocarcinoma of the uterine cervix in a young woman not associated with diethylstilbestrol: A case report and review of literature. Clin Cancer Investig J [serial online] 2012 [cited 2020 Apr 6];1:239-41. Available from: http://www.ccij-online.org/text.asp?2012/1/4/239/106278


  Introduction Top


Carcinoma of the uterine cervix is the most common gynecological malignancy worldwide. The most common histological type of malignant cervical neoplasms is squamous cell carcinoma. Adenocarcinomas account only for approximately 15% of malignant cervical tumors, and histologically they are categorized into mucinous, endometrioid, clear cell, serous, and mesonephric subtypes. Clear cell adenocarcinoma (CCA) most commonly occurs in the ovary, followed by endometrium, vagina, and cervix. Primary CCA of the vagina and uterine cervix is a rare neoplastic entity, which occurs in young women exposed to diethylstilbestrol (DES) in utero. Primary CCA of the uterine cervix inyoungwomen without in utero DES exposure is extremely rare. Here, we report a 21-year-old Asian female with primary CCA of the uterine cervix, with no maternal history of DES ingestion during pregnancy. The results of high-risk type of human papillomavirus (HPV-16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, 59, and 68) were negative in this particular case. The patient's unremarkable medical and sexual history prompted us to report this case.


  Case Report Top


A 21-year-old Asian woman, with negative familial cancer history, was presented with complaint of abnormal vaginal bleedingfor last 3 months. Gynecological examination revealed bulky cervix with a 2 × 1.5 cm ulceroproliferative lesion at the anterior lip. A punch biopsy from the lesion was taken. Histopathological examination of the biopsy showed papillary and tubular proliferations of malignant cells with glycogen-rich clear cytoplasm and malignant hobnail cells [Figure 1]. Contrast-enhanced computed tomographic scan of the abdomen and pelvis showed moderately enhanced bulky cervix with paracervical fat stranding and nodularity, without any locoregional lymphadenopathy [Figure 2]. All other hematological, biochemical, and radiological tests were within normal limits. The patient was therefore diagnosed as a case of CCA of cervix, FIGO stage IB1. She had undergone Wertheim's hysterectomy with bilateral pelvic lymph node dissection. Histologically, the initial diagnosis of primary cervical CCA, FIGO stage IB1, was confirmed. After 4 weeks of surgery, she received whole pelvis external beam irradiation upto a total dose of 50.4 Gy in 28 fractions along with concurrent chemotherapy (Cisplatin, 40 mg/m 2 weekly × five cycles), followed by vaginal brachytherapy (6 Gy × two fractions). The patient is still under observation in our department, 6 months after completion of treatment. No signs of recurrence have been detected since then.
Figure 1: Photomicrograph of the histopathology of cervical punch biopsy showing papillary and tubular proliferations of malignant cells with glycogen-rich clear cytoplasm and malignant hobnail cells (H and E, ×200)

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Figure 2: Contrast-enhanced computed tomographic scan of the abdomen and pelvis showing moderately enhanced bulky cervix with paracervical fat stranding and nodularity, without any locoregional lymphadenopathy

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  Discussion Top


The association between CCA of the vagina and cervix and in utero exposure to DES was first described in a case series in 1971, [1] and after that the causal association between in utero DES exposure and CCA of the vagina and cervix has been firmly established through the follow-up of several DES-exposed cohorts. [2],[3] Primary CCA of the vagina and cervix primarily occurs in young women exposed to DES in utero. The median age of diagnosis is 18.9 years, and the estimated risk of developing CCA of the vagina andcervixin an exposedfemaleup to age 24 is between 0.14 and 1.4 in thousand. [4] The national cooperative DES adenosis project estimates the risk to be less than 1 in thousand. [5] Althoughearly analyses described an excess of CCA of the vagina and cervix that peaked during late adolescence and early adulthood, [2],[3],[4] but follow-up of established cohorts suggests an elevation in risk persisting as the cohort aged. [6],[7]

Primary CCA of cervix is extremely rare in women without in utero DES exposure and in such cases it concerns mostly postmenopausal women. [8] There have been very few case reports of CCA of cervix in young women without in utero DES exposure. [9],[10] Moreover, we have to emphasize the negative family history of cancer and the lack of epidemiological risk factors of cervical cancer, such as HPV infection, multiple sexual partners, smoking, low socioeconomic status, and oral contraceptive use in the presented case. [11],[12] In a retrospective analysis, Liebrich et al. have reported 18 cases of primary cervical cancer, persistently negative for high-risk HPV-DNA, in virgins and very young women. Most of them had rare subtypes of adenocarcinoma, such as clear cell or endometrioid variety, or with unknown histology. Finally, the authors concluded that rare adenocarcinoma of the uterinecervix in virgins and young adolescents may represent a distinct entity unrelated to HPV. [13] This may be a possible explanation of rapid onset cervical cancer in our patient. It is well known that ovarian CCA is closely associated with ovarian endometriosis. [14] There have been a case report of association between CCA of cervix with cervical endometriosis. [15] But the question of whether cervical CCA is related to endometriosis has never been addressed in any study. There is also a reported case of CCA of cervix associated with hemihypertrophy and bilateral Wilms' tumor in a young girl with no maternal history of DES ingestion during pregnancy. [16]

The data from the literature indicate that in primary CCA of cervix, either radiation or radical hysterectomy and bilateral lymph node dissection results in cure rates of 85-90% for patients with small volume disease. [17] The mode of treatment depends on patient factors and available local experience. In smaller foci of disease, as in our patient, upfront surgery followed by radiation therapy is the preferred mode of treatment. [18]

 
  References Top

1.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;284:878-81.  Back to cited text no. 1
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2.Hanselaar A, van Loosbroek M, Schuurbiers O, Helmerhorst T, Bulten J, Bernhelm J. Clear cell adenocarcinoma of the vagina and cervix. An update of the central Netherlands registry showing twin age incidence peaks. Cancer 1997;79:2229-36.  Back to cited text no. 2
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3.Hatch EE, Palmer JR, Titus-Ernstoff L, Noller KL, Kaufman RH, Mittendorf R, et al. Cancer risk in women exposed to diethylstilbestrol in utero. JAMA 1998;280:630-4.  Back to cited text no. 3
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4.Herbst AL. The current status of the DES-exposed population. Obstet Gynecol Annu 1981;10:267-78.  Back to cited text no. 4
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5.Robboy SJ, Szyfelbein WM, Goellner JR, Kaufman RH, Taft PD, Richard RM, et al. Dysplasia and cytologic findings in 4,589 young women enrolled in diethylstilbestrol-adenosis (DESAD) project. Am J Obstet Gynecol 1981;140:579-86.  Back to cited text no. 5
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6.Troisi R, Hatch EE, Titus-Ernstoff L, Hyer M, Palmer JR, Robboy SJ, et al. Cancer risk in women prenatally exposed to diethylstilbestrol. Int J Cancer 2007;121:356-60.  Back to cited text no. 6
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7.Verloop J, van Leeuwen FE, Helmerhorst TJ, van Boven HH, Rookus MA. Cancer risk in DES daughters. Cancer Cause Control 2010;21:999-1007.  Back to cited text no. 7
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8.Schrager S, Potter BE. Diethylstilbestrol exposure. Am Fam Physician 2004;69:2395-400.  Back to cited text no. 8
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9.Stewart J 3rd, Bevans-Wilkins K, Ye C, Kurtycz DF. Clear-cell endocervical adenocarcinoma in a 19-year-old woman. Diagn Cytopathol 2006;34:839-42.  Back to cited text no. 9
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10.Maheshwari V, Sharma R, Mehdi G, Prasad S, Hakim S. Clear cell adenocarcinoma cervix: A diagnostic dilemma. Indian J Pathol Microbiol 2009;52:282-3.  Back to cited text no. 10
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11.Cannistra SA, Niloff JM. Cancer of the uterine cervix. N Engl J Med 1996;334:1030-8.  Back to cited text no. 11
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12.Liu S, Semenciw R, Mao Y. Cervical cancer: The increasing incidence of adenocarcinoma and adenosquamous carcinoma in younger women. CMAJ 2001;164:1151-2.  Back to cited text no. 12
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13.Liebrich C, Brummer O, Von Wasielewski R, Wegener G, Meijer C, Iftner T, et al. Primary cervical cancer truly negative for high-risk human papillomavirus is a rare but distinct entity that can affect virgins and young adolescents. Eur J Gynaecol Oncol 2009;30:45-8.  Back to cited text no. 13
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14.Tanaka YO, Yoshizako T, Nishida M, Yamaguchi M, Sugimura K, Itai Y. Ovarian carcinoma in patients with endometriosis: MR imaging findings. AJR Am J Roentgenol 2000;175:1423-30.  Back to cited text no. 14
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15.Hiromura T, Tanaka YO, Nishioka T, Satoh M, Tomita K. Clear cell adenocarcinoma of the uterine cervix arising from a background of cervical endometriosis. Br J Radiol 2009;82:e20-2.  Back to cited text no. 15
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16.Stalens JP, Maton P, Gosseye S, Clapuyt P, Ninane J. Hemihypertrophy, bilateral Wilms' tumor, and clear-cell adenocarcinoma of the uterine cervix in a young girl. Med Pediatr Oncol 1993;21:671-5.  Back to cited text no. 16
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17.Quinn MA. Adenocarcinoma of the cervix. Ann Acad Med Singapore 1998;27:662-5.  Back to cited text no. 17
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18.Benedet JL, Bender H, Jones H. Staging classifications and clinical practice guidelines of gynaecologic cancers. Elsevier: FIGO Committee on Gynecologic Oncology; 2000. p. 36-58.  Back to cited text no. 18
    


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