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Year : 2021  |  Volume : 64  |  Issue : 2  |  Page : 418-419
Ureteric sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation: A rare entity

1 Department of Uro-oncology, Tata Medical Centre, Kolkata, West Bengal, India
2 Department of Histopathology, Tata Medical Centre, Kolkata, West Bengal, India

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Date of Submission15-Apr-2020
Date of Decision15-Jul-2020
Date of Acceptance15-Jul-2020
Date of Web Publication9-Apr-2021

How to cite this article:
Jindal T, Dey D, Pawar P, Subedi N. Ureteric sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation: A rare entity. Indian J Pathol Microbiol 2021;64:418-9

How to cite this URL:
Jindal T, Dey D, Pawar P, Subedi N. Ureteric sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation: A rare entity. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 May 8];64:418-9. Available from: https://www.ijpmonline.org/text.asp?2021/64/2/418/313284

   Introduction Top

Sarcomatoid urothelial cancers are rare and are associated with aggressive clinical course. The urinary bladder is the usual organ to be affected and description of primary ureteric sarcomatoid urothelial carcinoma is limited to case reports in the literature. We describe a case of a ureteric sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation. We discuss the clinico-pathological findings, differential diagnosis, and management of the rare malignancy.

   Case Presentation Top

A 73-year-old male presented with an intermittent hematuria. The ultrasound revealed right hydroureteronephrosis. MRI revealed a right lower ureteric mass with distension of the ureter and pelvi-calyceal system [Figure 1]a and [Figure 1]b. The metastatic work-up was normal. A right nephroureterctomy was offered but the patient wished for a kidney preserving surgery hence a robotic right lower ureterectomy, excision of bladder cuff along with Boari flap reconstruction was performed [Figure 1]c.
Figure 1: The (a) axial and (b) sagittal T2 weighted images of MRI showing the mass in the lower ureter (arrow), (c) Intra-operative image demonstrating the distended ureter (arrow)

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The excised specimen of the lower ureter was 10.5 cm in length. The tumor was attached focally to the ureteric wall and then extended intraluminally along the entire length of the excised ureter. On microscopic examination, the tumor was polypoidal and predominantly consisted of heterologous cartilage elements showing pleomorphic nuclei and prominent nucleoli. Occasional epithelial cells arranged in papillae were seen. Mitotic figures were present [Figure 2]a and [Figure 2]b. The tumor invaded the muscularis propria of the ureter but the periureteric tissue was free of tumor. The cut end of the ureter and the bladder cuff were free of tumor and dysplasia. The differential diagnosis considered at this point were sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation or high grade sarcoma with chondrosarcomatous differentiation. Immunohistochemistry showed that the epithelial cells were focally positive to cytokeratin, GATA-3 (weak and patchy) and p-63 (patchy) [Figure 2c]. This suggested that the epithelial component seen in the tumor was urothelial carcinoma. The sarcomatoid component was positive for S-100. Desmin was negative. Hence because of the tumor location and immunomorphology, the diagnosis of sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation was favored.
Figure 2: (a) Microscopic images showing the sarcomatoid element with chondromatous differentiation (Hematoxylin and Eosin, 20X) (b) the papillary epithelial component is also seen in chondrosarcomatous background, (c) Immunohistochemistry showing the urothelial component to be positive for cytokeratin

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The patient made an uneventful recovery after surgery. His follow up scan, 4 months post-surgery, revealed loco-regional recurrence as well as systemic metastasis involving the lungs and retroperitoneum. The case was discussed in a multidisciplinary meeting and he was planned for palliative chemotherapy but he succumbed to his illness 5 months post-surgery.

   Discussion Top

Sarcomatoid urothelial carcinomas of ureter are rare and nearly 25 cases have been reported till date in the English literature.[1],[2] The chondrosarcomatous differentiation is even more rare.[1] The patients are usually in the sixth and the seventh decade at the time of presentation. The common symptoms are flank pain, fever, or hematuria.[1] A cross sectional imaging modality like CT or MRI helps in revealing the ureteric mass but there are no specific findings which help in differentiating them from the typical transitional cell carcinoma of the ureter. The diagnosis is usually made only after surgical excision.[1],[2] The demonstration of both, mesenchymal, and epithelial elements help in the pathological diagnosis. There is significant debate regarding the origin of these tumors due to the dual tumor cell lineage. They have been considered to be "collision tumors" in which epithelial and mesenchymal malignancies have occurred simultaneously.[3] The other theory suggests that the origin is from the same set of pluripotent cells which develop into the epithelial and mesenchymal components.[4] At times, the demonstration of the epithelial component may be difficult as in can be scanty and they may be confused with extraskeletal chondrosarcomas. In such cases, immunohistochemistry is a very useful tool which can reveal the expression of both the epithelial and mesenchymal markers, thus facilitating the diagnosis.[1],[2]

As this is a rare tumor, the optimum line of management is still unclear. Multimodal treatment with surgery, adjuvant chemotherapy, or radiotherapy has been tried. Majority of the cases develop early recurrences despite treatment and have a dismal outcome.[1],[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Lu W, Wang Y, Li Y, Cao Y, Han H, Zhou F. Sarcomatoid urothelial carcinoma with chondrosarcomatous differentiation of the ureter: A case report and review of the literature. Oncol Lett 2017;13:1331-7.  Back to cited text no. 1
Wang Y, Liu H, Wang P. Primary sarcomatoid urothelial carcinoma of the ureter: A case report and review of the literature. World J Surg Onc 2018;16,77.  Back to cited text no. 2
Perret L, Chaubert P, Hessler D, Guillou L. Primary heterologous carcinosarcoma (metaplastic carcinoma) of the urinary bladder: A clinicopathologic, immunohistochemical, and ultrastructural analysis of eight cases and a review of the literature. Cancer 1998;82:1535-49.  Back to cited text no. 3
Völker HU, Zettl A, Schön G, Heller V, Heinrich E, Rosenwald A, et al. Molecular genetic findings in two cases of sarcomatoid carcinoma of the ureter: Evidence for evolution from a common pluripotent progenitor cell? Virchows Arch 2008;452:457-63.  Back to cited text no. 4

Correspondence Address:
Tarun Jindal
Department of Uro-oncology, Tata Medical Centre, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_378_20

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