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E-JCRT CORRESPONDENCE
Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 666

Synchronous carcinosarcoma of the lower ureter and renal pelvis with heterologous chondrosarcomatous elements: An unusual case report


Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Santosh Menon
Department of Pathology, Tata Memorial Centre, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.143358

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 > Abstract 

Carcinosarcoma of the ureter is a rare biphasic neoplasm, with distinct malignant epithelial and mesenchymal components. The mesenchymal component may show heterologous chondrosarcomatous or osteosarcomatous elements. To the best of our knowledge, there have been only 13 cases reported in the literature. Synchronous malignancy in the renal pelvis is even rarer and this is the first case of synchronous carcinosarcoma of the ureter and renal pelvis to be reported in English literature, which revealed heterologous chondrosarcomatous areas at both sites.

Keywords: Carcinosarcoma, pelvis, renal, ureter


How to cite this article:
Parikh P, Menon S, Desai SB. Synchronous carcinosarcoma of the lower ureter and renal pelvis with heterologous chondrosarcomatous elements: An unusual case report. J Can Res Ther 2015;11:666

How to cite this URL:
Parikh P, Menon S, Desai SB. Synchronous carcinosarcoma of the lower ureter and renal pelvis with heterologous chondrosarcomatous elements: An unusual case report. J Can Res Ther [serial online] 2015 [cited 2019 Nov 15];11:666. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/666/143358


 > Introduction Top


Carcinosarcoma of urothelial tract is a rare but aggressive malignant tumor. They are believed to be metaplastic carcinomas, which show dedifferentiated sarcomatous components. They most commonly occur in the urinary bladder followed by ureter and renal pelvis. [1],[2] Late clinical presentation and diagnosis makes ureteric carcinosarcoma a difficult management challenge in a clinical setting. Synchronous occurrence of malignancies in the ureter and renal pelvis is not an uncommon occurrence, especially as regards to urothelial carcinomas. [3],[4] However, to the best of our knowledge, this is the first case of a synchronous carcinosarcoma of the ureter and pelvis demonstrating heterologous mesenchymal differentiation.


 > Case report Top


A 45-year-old male patient presented with the complaints of painless hematuria with the passage of blood clots in urine since 4 months and sudden retention of urine since 2 days. His physical examination did not reveal any significant findings. Routine hematologic examinations were within normal range. However, renal function tests revealed raised serum creatinine (2.2 mg%) and raised serum blood urea nitrogen - 54 mg%. On computed tomography scan, a 5.2 cm × 6.3 cm × 5.5 cm heterogeneous, exophytic tumor was identified involving left lower end of ureter extending to vesicoureteric junction. Left kidney measured 10.4 cm and radiologically no mass lesion was seen. Right kidney measured 8.4 cm and showed 7 mm calculus at the lower pole of the kidney. Patient was operated for left radical nephroureterectomy with cystoprostatectomy with the creation of an ileal conduit. On gross examination, an infiltrative tumor was identified measuring 7 cm × 5 cm × 4 cm involving lower end of left ureter just extending onto vesicoureteric junction. The tumor showed white, firm cut surface. The left kidney showed multiple cysts with thinned out cortex and showed changes of hydronephrosis. Glistening white firm area measuring 1.5 cm × 1 cm × 1 cm was observed within the hydronephrotic cysts [Figure 1]. On microscopy, sections from both the left ureteric mass and the pelvis of the left kidney showed a biphasic tumor comprised of urothelial carcinoma component of polygonal cells with high nucleocytoplasmic ratio, hyperchromatic nucleus and scanty cytoplasm in sheets and islands admixed with chondrosarcomatous component comprised of lobules of cellular malignant cartilage.[Figure 2]a-c In addition focally the stroma had extensive myxoid change. Mitotic activity were frequent both in epithelial and sarcomatous areas. Multiple sections were studied from the renal pelvis to carefully look for urothelial carcinoma in situ in the native urothelium of pelvis. However, we could not demonstrate the same. However, the pelvic tumor was seen replacing the lining of the pelvic urothelium with invasion of renal parenchyma.
Figure 1: Gross photograph of radical nephroureterectomy and cystoprostatectomy specimen showing the tumor at lower end of ureter and glistening tumor in dilated renal pelvis (arrow)

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Figure 2: Carcinosarcoma of ureter with admixture of malignant epithelial (a, thick arrow) and chondrosarcomatous elements (a, thin arrow) (H and E, ×40). Malignant epithelial component in myxoid stroma (b, H and E, ×100). Synchronous carcinosarcoma of pelvis infiltrating the renal cortex (c, arrow showing glomeruli, H and E, ×40). Cytokeratin 7 positivity in malignant urothelial component (d, thin arrow, indirect immunoperoxidase, ×40) with native urothelium as internal control (d, thick arrow)

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On immunohistochemistry (IHC), the carcinomatous component was highlighted by cytokeratin (CK)-7 [Figure 2]d, high molecular weight CK, whereas the chondrosarcomatous component was highlighted by S100 protein IHC. The renal pelvis tumor invaded the peripelvic fat and renal parenchyma (pT3; tumor node metastasis staging) and the ureteric tumor was invading beyond the muscularis propria in periureretic fat (pT3; TNM staging). Lymphovascular emboli were not demonstrated and perinerial invasion was also not seen. The urinary bladder and prostate were uninvolved.


 > Discussion Top


Carcinosarcomas of ureter are highly aggressive tumors with poor prognosis. [1],[4],[5] They usually occur in patients of age 60 and above. Ureteral carcinosarcomas preferentially occurs in the distal ureter. [3] A true carcinosarcoma is a biphasic tumor with distinct malignant carcinomatous and sarcomatous elements, believed to be derived from a common pluripotent stem cell. There are controversies regarding the nomenclature and histogenesis of these tumors. In some series, both carcinosarcoma and sarcomatoid carcinoma are included under the rubric "sarcomatoid carcinoma." In others they are thought to be separate entities. Recent molecular studies, however, strongly support a monoclonal origin of both components. [6],[7]

Carcinosarcomas differ from several less aggressive and more common lesions in the region, including carcinomas with osseous or chondroid metaplasia, spindle cell carcinomas, and carcinoma with pseudosarcomatous stroma. Metaplastic stromal changes would not have the histological features of malignancy. Features favoring a sarcomatoid carcinoma include the presence of urothelial carcinoma-in-situ and zones of transition between epithelial and mesenchymal cells. In difficult situations, ancillary immunohistochemical stains for epithelial markers positivity within the spindle cells of sarcomatous stroma would clinch the diagnosis. Pseudosarcomatous stromal reactions lack malignant features in the stromal component, and may only show minimal atypia and mitotic activity, thus distinguishing them from sarcomatous component of a carcinosarcoma. In addition, pseudosarcomatous stromal reactions tend to be highly vascularized with thin capillary sized channels. [1],[3],[5],[8] The fact that these benign mimickers need to be carefully kept in mind by the pathologist so as to avoid misdiagnosis, cannot be overemphasized. Our case had high grade carcinomatous and chondrosarcomatous sarcomatous element, characteristic of carcinosarcomas.

Carcinosarcoma of the ureter is considered to be a highly aggressive tumor, resistant to radiation therapy, and has a poor prognosis. [8],[9] Aggressive surgical treatment in the form of nephroureterectomy is the primary treatment of choice. Adjuvant radiotherapy or chemotherapy has not been shown to significantly improve outcome. [3] Most of the patients recur within a few months and the longest survival period is about 2 years, following radical surgery. [10] Our patient also had a radical nephroureterectomy after which he has been lost to follow-up.


 > Conclusion Top


We have described a rare synchronous malignancy of the ureter and renal pelvis which is important for urologists and pathologists to be aware of, as this is an aggressive neoplasm.

 
 > References Top

1.
Darko A, Das K, Bhalla RS, Heller D. Carcinosarcoma of the ureter: Report of a case with unusual histology and review of the literature. Int J Urol 2006;13:1528-31.  Back to cited text no. 1
    
2.
Orsatti G, Corgan FJ, Goldberg SA. Carcinosarcoma of urothelial organs: Sequential involvement of urinary bladder, ureter, and renal pelvis. Urology 1993;41:289-91.  Back to cited text no. 2
    
3.
Vermeulen P, Hoekx L, Colpaert C, Wyndaele JJ, Van Marck E. Biphasic sarcomatoid carcinoma (carcinosarcoma) of the renal pelvis with heterologous chondrogenic differentiation. Virchows Arch 2000;437:194-7.  Back to cited text no. 3
    
4.
Perimenis P, Athanasopoulos A, Geragthy J, Speakman M. Carcinosarcoma of the ureter: A rare, pleomorphic, aggressive malignancy. Int Urol Nephrol 2003;35:491-3.  Back to cited text no. 4
    
5.
Nagayoshi J, Kawakami T, Maruyama Y. Sarcomatoid carcinoma of the ureter: A case report. Int J Urol 1997;4:618-20.  Back to cited text no. 5
    
6.
Reuter VE. Sarcomatoid lesions of the urogenital tract. Semin Diagn Pathol 1993;10:188-201.  Back to cited text no. 6
    
7.
Gronau S, Menz CK, Melzner I, Hautmann R, Möller P, Barth TF. Immunohistomorphologic and molecular cytogenetic analysis of a carcinosarcoma of the urinary bladder. Virchows Arch 2002;440:436-40.  Back to cited text no. 7
    
8.
Yilmaz E, Birlik B, Arican Z, Guney S. Carcinosarcoma of the renal pelvis and urinary bladder: A case report. Korean J Radiol 2003;4:255-9.  Back to cited text no. 8
    
9.
Johnin K, Kadowaki T, Kushima M, Ushida H, Koizumi S, Okada Y. Primary heterologous carcinosarcoma of the ureter with necrotic malignant polyps. Report of a case and review of the literature. Urol Int 2003;70:232-5.  Back to cited text no. 9
    
10.
Chen KT, Workman RD, Flam MS, DeKlotz RJ. Carcinosarcoma of renal pelvis. Urology 1983;22:429-31.  Back to cited text no. 10
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