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CASE REPORT
Year : 2010  |  Volume : 6  |  Issue : 3  |  Page : 304-306

Endometrial adenocarcinoma involving both horns of a bicornuate uterus


1 Department of Pathology, Cancer Institute (W.I.A.), 38, Sardar Patel Road, Chennai 600 036, India
2 Department of Surgical Oncology, Cancer Institute (W.I.A.), 38, Sardar Patel Road, Chennai 600 036, India
3 Department of Radiation Oncology, Cancer Institute (W.I.A.), 38, Sardar Patel Road, Chennai 600 036, India

Date of Web Publication29-Nov-2010

Correspondence Address:
Sundersingh Shirley
New No. 23, Jambulingam Street, Nungambakkam, Chennai 600 034
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.73322

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 > Abstract 

We report a rare case of endometrial adenocarcinoma involving both horns of a bicornuate uterus in a postmenopausal woman. Patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and bilateral pelvic lymph node dissection following an initial positive diagnosis of well differentiated endometrioid adenocarcinoma on endometrial biopsy. Incidentally, the left ovary revealed a well differentiated sertoli leydig cell tumor. Endometrial carcinoma arising in malformed uterus is rare. Its simultaneous occurrence with an ovarian sertoli leydig cell tumor has not been reported in English literature so far. This case is reported for its rarity.

Keywords: Bicornuate uterus, endometrial adenocarcinoma, ovarian sertoli leydig cell, tumor


How to cite this article:
Shirley S, Devi VS, Krishnamurthy R, Nabhi MV, Majhi U, Selvaluxmy G. Endometrial adenocarcinoma involving both horns of a bicornuate uterus. J Can Res Ther 2010;6:304-6

How to cite this URL:
Shirley S, Devi VS, Krishnamurthy R, Nabhi MV, Majhi U, Selvaluxmy G. Endometrial adenocarcinoma involving both horns of a bicornuate uterus. J Can Res Ther [serial online] 2010 [cited 2021 Jan 24];6:304-6. Available from: https://www.cancerjournal.net/text.asp?2010/6/3/304/73322


 > Introduction Top


Congenital abnormalities of the female genital tract arise from malformation of the paramesonephric (Mόllerian) duct. The true prevalence of mόllerian duct anomalies is difficult to assess due to different populations and differences in data acquisition. [1] Though reported as 4-5% in the general population, [2] the prevalence may be higher as many of them go unnoticed due to their asymptomatic nature. They are usually discovered in patients presenting with infertility. A prevalence of 8-10% in those undergoing investigations for obstetric causes has been reported. [1] Fusion anomalies of the uterus are of various kinds. Bicornuate uterus associated with endometrial carcinoma is rare with only few case reports. [3] Its simultaneous occurrence with ovarian sex-cord stromal tumor has not been reported so far. To the best of our knowledge, this is the first case in the English literature of endometrial adenocarcinoma arising in both horns of a bicornuate uterus with coexisting well differentiated sertoli leydig cell tumor of the ovary.


 > Case Report Top


A 57-years-old, obese, nulliparous woman presented with complaints of postmenopausal bleeding of two years duration. Pervaginal examination revealed healthy cervix and vagina. Uterus was just bulky, mobile and deflected to the left. No adnexal masses were palpable.

Ultrasonography revealed an anteverted uterus measuring 8.2 × 3.8 cm. Endometrial thickness was 1.3 cm. Right ovary measured 2.4 × 1.1 cm and left ovary measured 2.5 × 1.8 cm. No iliac or retroperitoneal lymphadenopathy was detected. Hormone levels were not assessed. Endometrial biopsy was done and showed features of a well differentiated endometrioid adenocarcinoma. Patient was diagnosed to have an endometrial carcinoma, stage I A. One high dose rate intracavitatory application to deliver 4500 cGy to the surface of endometrium as per the institutions protocol was given followed by total abdominal hysterectomy with bilateral salpingo-oophorectomy and bilateral pelvic lymph node dissection.

Gross examination showed a bicornuate uterus with a single cervix and bilateral fallopian tubes and ovaries. The left cornu measured 5.5 × 4 × 3.5 cm and the right cornu measured 5 × 3.5 × 3 cm. On cut section, the left horn revealed a greywhite friable tumor measuring 4 × 1.5 × 1 cm whereas the cavity of the right horn appeared irregular [Figure 1]. Left and right ovaries measured 3.5 × 1.8 × 1 cm and 3 × 1.5 × 1 cm, respectively. Cut surface of both appeared unremarkable grossly.
Figure 1: Bicornuate uterus with grossly visible tumor in the left horn and irregular right horn cavity

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Histopathological examination showed well differentiated endometrioid adenocarcinoma involving both the horns composed of closely packed well defined glands lined by malignant stratified columnar epithelial cells with foci of squamous differentiation [Figure 2]. Areas of necrosis and degeneration were evident in the right horn, probably secondary to intracavitatory application of radiation. Tumor infiltrated less than half of the myometrial thickness of both horns. The tumor was positive for progesterone and estrogen receptors. Left ovary incidentally showed a well differentiated sertoli leydig cell tumor composed of nodular collections of hollow tubules lined by columnar sertoli cells surrounded by fibrous stroma containing aggregates of leydig cells [Figure 3]. Ovarian capsule was free. The tumor was strongly positive for inhibin [Figure 4] and negative for estrogen and progesterone receptors. Both the external iliac and obturator lymph nodes were free. A diagnosis of endometrioid adenocarcinoma, Grade 1 arising in both horns of a bicornuate uterus with coexisting well differentiated sertoli leydig cell tumor of the left ovary was made.
Figure 2: Well differentiated endometrioid carcinoma with foci of squamous differentiation (H and E, 100)

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Figure 3: Well formed tubules lined by sertoli cells surrounded by interstitial leydig cells (H and E, × 100)

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Figure 4: Strong positivity of sertoli and leydig cells for inhibin (DAB, × 100).

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 > Discussion Top


Endometrial carcinoma is the most common malignant tumor of the female genital tract in developed countries. The estrogen dependent endometrioid type accounts for 80-85% of cases and is often related either to exogenous estrogen administration or to endogenous hyperestrinism. [4] Feminizing ovarian disease is an important endogenous risk factor for development of endometrial carcinoma. Sertoli-leydig cell tumors are rare sexcord-stroma derived tumors constituting less than 0.5% of ovarian tumors. [5] Less than 10% occur either prior to menarche or after menopause. [6] Majority of these tumors produce excess of androgen resulting in signs and symptoms of virilization. Rarely sertoli leydig cell tumors may be associated with hyperestrinism. [7] These may occur either because of peripheral conversion of androgens to estrogens or rarely a primary estrogen secreting sertoli leydig cell tumor of the ovary. [8] Estrogenic manifestations in the form of endometrial carcinoma are extremely rare and have not been reported in the literature so far.

The female genital tract develops from two paired paramesonephric (Mόllerian) ducts and develops bidirectionally along the lateral aspects of the gonads. Congenital uterine anomalies known as mόllerian anomalies may manifest as absence of uterus or fusion anomalies. The lesser degree of fusion defect known as the bicornuate uterus accounts for approximately 10% of mόllerian duct anomalies. It is usually discovered in patients presenting with infertility. Endometrial carcinoma arising in bicornuate uterus is rare, the present report being fourth such case [Table 1].
Table 1: Reported cases of endometrial carcinoma in bicornuate uterus

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Mόllerian duct anomalies seem to confer a protective effect against endometrial carcinoma. Estrogens through HOX genes are known to play an important role in the normal development of mόllerian derivatives. Abnormalities in molecular signaling following estrogen receptor activation could result in abnormal HOX gene expression and mόllerian duct abnormalities in mice. [9] The molecular basis of mόllerian duct anomalies has further been characterized by DNA micro array in a recent study. [10] These animal studies can be readily extrapolated to humans. Hence, this abnormal post ER signaling molecular defect could protect the endometrium from carcinogenic effects of estrogen.

Mόllerian duct anomalies cannot be excluded on the basis of negative ultrasound findings. Pelvic MRI is the investigation of choice for detection of mόllerian duct anomalies with an accuracy of upto 100% being reported. [1] Our patient was nulliparous and the uterine abnormality was detected only after she was operated for an endometrial carcinoma. Since carcinoma involved both horns of the uterus, she had a positive diagnosis of endometrioid carcinoma on the initial biopsy. In the presence of persistent postmenopausal bleeding and a negative biopsy, a pelvic MRI should probably be considered in order to avoid delay in treatment when carcinoma involves only a single horn of a bicornuate uterus.

In our patient, the presence of malignancy in both horns suggests the presence of a systemic risk factor. Apart from the well known risk factors like nulliparity and obesity, the presence of ovarian sertoli leydig cell tumor could also have contributed to the increased estrogen levels leading to the development of endometrial carcinoma in both horns of the bicornuate uterus. The prognosis of endometrial carcinoma that arises as a second cancer appears to be more favorable than for endometrial carcinoma in general.

 
 > References Top

1.Pellerito JS, McCarthy SM, Doyle MB, Glickman MG, DeCherney AH. Diagnosis of uterine anomalies: Relative accuracy of MR imaging, endovaginal sonography, and hysterosalpingography. Radiology 1992;183:795-800.  Back to cited text no. 1
    
2.Elford KJ, Spence JE. The forgotten female: Pediatric and adolescent gynecological concerns and their reproductive consequences. J Pediatr Adolesc Gynecol 2002;15:65-77.  Back to cited text no. 2
    
3.Moore JR, Davidson SA, Singh M. Endometrial carcinoma in one horn of a bicornuate uterus. Gynecol Oncol 2004;95:729-32.  Back to cited text no. 3
    
4.Silverberg SG, Kurman RJ, Nogales F, Mutter GL, Kubik-Huch RA, Tavassoli FA. Tumours of the uterine corpus: Epithelial tumours and related lesions. In: Tavassoli FA, Devilee P, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs. Lyon: IARC Press; 2003. p. 221-32.  Back to cited text no. 4
    
5.Tavassoli FA, Mooney E, Gersell DJ, McCluggage WG, Konishi I, Fujii S, et al. Tumours of the ovary and peritoneum: Sex cord-stromal tumours. In: Tavassoli FA, Devilee P, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs. Lyon: IARC Press; 2003. p. 146-61.  Back to cited text no. 5
    
6.Caringella A, Loizzi V, Resta L, Ferreri R, Loverro G. A case of Sertoli-Leydig cell tumor in a postmenopausal woman. Int J Gynecol Cancer 2006;16:435-8.  Back to cited text no. 6
    
7.Genton CY, Schmid J. Ovarian Sertoli-Leydig cell tumor with hyperoestrinism. Virchows Arch A Pathol Anat Histol 1981;390:243-8.  Back to cited text no. 7
    
8.Appetecchia M, Cela V, Bernardi F, Burelli A, Cionini R, Pucci E. Sertoli-Leydig cell androgens-estrogens secreting tumor of the ovary: Ultra-conservative surgery. Eur J Obstet Gynecol Reprod Biol 2004;116:113-6.  Back to cited text no. 8
    
9.Block K, Kardana A, Igarashi P, Taylor HS. In utero diethylstilbestrol (DES) exposure alters Hox gene expression in the developing mόllerian system. FASEB J 2000;14:1101-8.  Back to cited text no. 9
    
10.Suzuki A, Urushitani H, Sato T, Kobayashi T, Watanabe H, Ohta Y, et al. Gene expression change in the Mόllerian duct of the mouse fetus exposed to diethylstilbestrol in utero. Exp Biol Med (Maywood) 2007;232:503-14.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]


This article has been cited by
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2 Virilizing Leydig-Sertoli cell ovarian tumor associated with endometrioid carcinoma of the endometrium in a postmenopausal patient: Case report and general considerations
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Clinical Medicine Insights: Case Reports. 2012; 5: 149-153
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