Journal of Cancer Research and Therapeutics Medknow Publications on behalf of the Association of Radiation Oncologists of India (AROI) ISSN: 0973-1482 EISSN: 1998-4138 Vol. 6, Num. 4, 2010, pp. 578-580

Journal of Cancer Research and Therapeutics, Vol. 6, No. 4, October-December, 2010, pp. 578-580

Case Report

Endometrioid carcinoma of the upper urinary tract

Jagdeesh N Kulkarni¹, Tejal K Gorasia², Jayashree P Choudhary², Pravin P Mahajan²

¹ Department of Urology, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai - 400 020, India
² Department of Urology, S. L. Raheja Hospital, SL Raheja Marg, Mahim, Mumbai 400016, India

Correspondence Address: Jagdeesh N Kulkarni, Department of Urology, Bombay Hospital Institute of Medical Sciences, New Marine lines, Mumbai 400020, India, jnkulkarni@gmail.com

Code Number: cr10144

PMID: 21358108

DOI: 10.4103/0973-1482.77086

Abstract

Keywords: Chemotherapy, endometrioid carcinoma, endometriosis, histopathology, nephro-ureterectomy, ovarian carcinoma, upper urinary tract

Introduction

Endometrial carcinoma is one of the most common malignancies of the gynecological tract in post-menopausal patients. ^{[1],[2],[3],[4]} Late recurrence of endometrial carcinomas after 5-6 years, which usually occurs in the lymph nodes, lung, liver and bones, is well documented. Further, few rare cases of endometrioid carcinoma occurring in the urothelial tract have been reported in association with endometriosis. However, in our case, the endometrioid carcinoma was seen throughout the urothelium of the affected side without any evidence of endometriosis. So far, only a single case of endometrioid carcinoma of bladder without any evidence of endometriosis has been reported. ^[5] We present a second case of endometrioid carcinoma of the upper urinary tract.

Case Report

A 51-year-old nullipara presented to us in December 2009 with history of intermittent painless hematuria of 2 months duration. In 1991, while on treatment for primary infertility, she developed left tubal pregnancy and had a left salphingo-oophorectomy. Later, in 1993, she had RSO+ TAH and omentectomy with diagnosis of right ovarian mass. The histopathological examination showed poorly differentiated adenocarcinoma of possible endometrioid type of right ovary. Later, she received six cycles of taxol plus Carboplatin based chemotherapy. Till recently, she has been in excellent health with no complaints. At presentation, her physical and gynecological evaluation was normal. Her routine hematocrit, biochemical profile and serum CA-125 level were normal. The ultrasonography and computerized tomography studies revealed a mass extending from right kidney pelvis up to the bladder [Figure - 1]. The cystoscopy confirmed a mass popping out of the right ureteric orifice with normal bladder and left ureteric orifice. A formal punch biopsy from the mass raised the suspicion of the endometrioid carcinoma of right urinary tract. The Tc99mDTPA (Diethylene Triamine Penta acetic Acid) scan showed a non-functioning right kidney. Subsequently, she underwent a right radical nephro-ureterectomy with excision of the bladder cuff. Her postoperative recovery was uneventful. She was not given any adjuvant therapy. At 12 months follow-up, she is alive and free of disease.

On gross examination, the right kidney measured 6 cm × 3.5 cm × 3.5 cm in size and the ureter measured 14 cm in length. On opening the ureter, an irregular, polypoidal mass was seen involving the distal ureter. The mass measured 2.5 cm × 0.3 cm × 1 cm [Figure - 2]. The pelvi-calyceal system and most of the proximal ureter up to the intra mural portion showed papillary excrescences [Figure - 3]. The distal cut margin of bladder cuff was 1.5 cm away from the polypoidal tumor. The microscopic examination revealed a high-grade endometrioid carcinoma involving the pelvi-calyceal mucosa and the entire length of ureter, up to the vesico-ureteric junction. The tumor was seen maximum in the distal ureter and had infiltrated the lower ureteric wall and renal medulla [Figure - 4] and [Figure - 5]. In some areas, the normal urothelial lining was replaced by endometrioid epithelium. No areas of endometriosis were evident within the tumor. On immunohistochemistry, the tumor cells expressed CK 7, CA-125, Estrogen Receptors (ER) and Progesterone Receptors (PR) focally. They were negative for CK 20(Cytokeratin 20) and CEA (Carcino Embryonic Antigen). The tumor was seen maximum in the distal ureter and had infiltrated the lower ureteric wall and renal medulla. There was no intestinal metaplasia to suggest origin within the urothelium, and no areas of typical urothelial carcinoma were seen.

Discussion

Our case highlights the diagnostic and the therapeutic dilemma. Our initial impression was a second primary lesion, i.e., urothelial carcinoma. However, biopsy raised the possibility of a non-urothelial tumor which we tried to correlate with previous history of endometrioid carcinoma of the gynecological tract. Review of literature showed that the recurrence of endometrioid carcinoma usually occurs in the first 3 years of excision of the primary. ^[6],[7],[8] Late relapse, either locally or at a distant region after 3 years, is extremely rare and till today only a single case has been reported by Tsurumaki and colleagues. ^[9] Further, we were debating whether this is a late recurrence (after 17 years) of the endometrioid carcinoma in the urinary tract. Women with endometriosis have urinary tract involvement in 1-2% of cases, the bladder being most commonly affected. Malignant transformation is rare at extragonadal sites. ^[10]

Several features have been suggested for diagnosing carcinoma in extragonadal endometriosis. These include endometriosis in proximity to the tumor, no other primary tumor present, histological appearance in keeping with endometrial origin and a dysplastic phase between endometriosis and frank malignancy. ^[10] No evidence of endometriosis was seen anywhere in our tumor or in adjacent tissues of resection specimen. Although our case does not fulfill all the criteria for the histological diagnosis of malignancy arising in endometriosis as suggested above, we made the diagnosis of endometrioid carcinoma on the basis of these features: histological findings of atypical endometrioid type glands replacing the urothelium at places as well as infiltrating the ureteric wall, positive nuclear staining for ER and PR within the tumor consistent with endometrioid origin, and no evidence of a urothelial primary or metastasis from another site.

This case is unique in the following ways. The histopathology report of cystoscopic biopsy suggestive of non-urothelial malignancy of endometrioid type re-emphasizes the difficulty faced by pathologists while making the diagnosis in this very rare presentation. Further, endometrioid carcinoma in the urothelial tract can be mistaken for clear cell variant of urothelial carcinoma and diagnosis in this situation may be very difficult without the use of immunohistochemistry. Thirdly, absence of endometriosis in urinary tract or in the vicinity adds to the difficulties. She had already received adequate first-line chemotherapy for ovarian tumor in the past and lack of adequate literature on the therapeutic aspects of this rare tumor occurrence in urothelial tract, the adjuvant therapy was deferred.

References

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