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Year : 2011  |  Volume : 54  |  Issue : 2  |  Page : 400-401
Sarcomatoid carcinoma of the urinary bladder: A report of two cases

1 Department of Pathology, Al-Jahra Hospital, Kuwait
2 Department of Urology, Al-Jahra Hospital, Kuwait

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Date of Web Publication27-May-2011

How to cite this article:
Singh NG, Mannan AR, Kahvic M, Sara YA. Sarcomatoid carcinoma of the urinary bladder: A report of two cases. Indian J Pathol Microbiol 2011;54:400-1

How to cite this URL:
Singh NG, Mannan AR, Kahvic M, Sara YA. Sarcomatoid carcinoma of the urinary bladder: A report of two cases. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Jul 2];54:400-1. Available from: http://www.ijpmonline.org/text.asp?2011/54/2/400/81635

Here we discuss two cases of sarcomatoid carcinoma of the urinary bladder. We highlight the cases because of the rarity of the tumor and its ominous prognostic implication, and briefly discuss its differential diagnosis

   Case 1 Top

A 70-year-old man with diabetes presented at the Urology Department with complaint of painless hematuria of 2 months duration. Computed tomography of the pelvis revealed a large soft tissue mass of about 6 cm in diameter in the left lateral wall of the urinary bladder extending into the perivesicular fat. Other laboratory investigation findings were noncontributory. Transurethral resection of the tumor was performed.

Microscopic examination revealed tumor tissue, which was composed of haphazardly arranged sheets of pleomorphic spindle cells [Figure 1]a and b exhibiting hyperchromatic nuclei with moderate amount of cytoplasm. The tumor revealed brisk mitotic rate with infiltration into the muscular propria. No heterologous element was identified. The tumor cells were positive for cytokeratin [Figure 1]c, epithelial membrane antigen (EMA), and vimentin [Figure 1]d and negative for the desmin, smooth muscle actin (SMA), and S100 protein. Expression of both epithelial and mesenchymal markers in the spindle tumor cells implied a diagnosis of sarcomatoid tumor. The patient died of multiple metastatic disease, 2 years after the surgery.
Figure 1: Photomicrograph of haphazardly arranged sheets of pleomorphic spindle cells exhibiting hyperchromatic nuclei with moderate amount of cytoplasm (a and b) (H and E, ×100 and ×200 respectively). The cells are positive for cytokeratin (c) and vimentin (d) (avidin and biotin, ×200)

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   Case 2 Top

A 54-year-old man presented with dysuria and macrohematuria of 25 days duration in Urology Outpatient Department. Pelvic computed tomography revealed a 5 × 4 cm tumor with focal calcification, in the right lateral wall of the bladder. Transurethral resection of the tumor was attempted, which on microscopy displayed sarcomatoid carcinoma with extensive muscle invasion. Five months later, in view of the residual tumor showing involvement of the bladder neck, radical cystoprostatectomy was performed.

Surgical specimen grossly displayed a firm tumor measuring 9 cm in diameter [Figure 2]a occupying the bladder cavity. Histopathology revealed pleomorphic tumor cells arranged in diffuse sheets with vesicular nuclei, some exhibiting prominent nucleoli. There were prominent areas showing spindle cell morphology forming fascicles [Figure 2]b. Mitotic rate was high. There were areas of myxoid changes with foci of necrosis. No heterologous elements noted. The tumor cells were positive for cytokertin [Figure 2]c, and EMA throughout the tumor tissue. Vimentin [Figure 2]d was variably immunoreactive; it was diffusely positive in the spindle cell component and focally in other areas. The tumor was negative for desmin, SMA, chromogranin, HMB45, and S100 protein. The tumor shows transmural infiltration into the perivesical fibroadipose tissue. Right external iliac lymph nodes (3/8) revealed metastatic tumor deposit. A diagnosis of sarcomatoid urothelial carcinoma, (pT4a, pN2, Mx) was made. The patient is now on follow-up for the last 1 year.
Figure 2: Gross picture of the tumor in a cystoprostatectomy specimen (a). Photomicrgraph showing the pleomorphic spindle-shaped tumor cell with frequent mitosis (b) (H and E, ×200). The same tumor cells showing immunoreactive to cytokeratin (c) and vimentin (d) (avidin and biotin, ×200)

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   Discussion Top

The presence of spindle cell elements in urinary bladder transitional cell carcinoma (TCC) warrants the designation of sarcomatoid carcinoma and some authors define carcinosarcoma as an admixture of malignant epithelial and malignant soft tissue elements. [1] The tumor cells from both the tumor components showed monoclonality and clonal identity in all cases studied, suggesting a monoclonal origin. [2] The predominant location of the sarcomatoid carcinoma was the bladder base, including the trigon and the lateral walls of the bladder. In the present two cases, the tumors were located in the left and the right lateral walls, respectively. Most tumors presented as polypoid or large broadly based mass with ulceration and extension into the muscular layer. [1]

Histologically, the tumor may show a mixture of carcinomatous and sarcomatoid components in varying ratios, but the sarcomatoid component always occupies more than 50% of the tumor area. [1] The cellular arrangement may have various patterns, such as fascicular or storiform appearance, as seen in leiomyosarcoma and malignant fibrous histiocytoma. In sarcomatoid carcinoma loss of cell-cell and cell-matrix adhesion may be associated with dedifferentiation, which thus produces a sarcomtoid pattern, thereby enhancing stromal invasion and enabling the development of distant metastasis. [3]

The histologic differential diagnosis includes various types of sarcoma, reactive pseudosarcomatous mesenchymal proliferations, and malignant melanoma. Distinguishing these masses from sarcomas as leiomyosarcoma, pleomorphic fibrosarcoma, or malignant fibrous histiocytoma may be difficult but sarcomas usually do not stain for epithelial markers and lack desmosomes or tonofilaments on electron microscopy. [4] Benign pseudosarcomatous mesenchymal proliferations in the bladder include pseudosarcomatous myofibroblastic proliferations, postoperative spindle cell nodules, inflammatory pseudotumors, and pseudosarcomatous fibromyxoid tumors. [5] These pseudosarcomatous tumors are immunoractive for vimentin and muscle actin, although aberrant expressions of cytokeratins and EMA have been reported. Histologically, these reactive lesions show neither atypical mitosis nor significant cytologic atypia, whereas sarcomatoid carcinoma exhibits these atypical features with tumor necrosis, invasive tumor margins, and high cellularity. [5]

The tumor has poor prognosis. The carcinomatous component usually exhibits a grade 2 or 3 TCC and most patients suffer from an advanced stage of the tumor invading into or even beyond the muscular wall. [1] The present cases had transmural infiltration of tumor tissue with regional lymph nodes metastasis (2nd case) at the time of diagnosis. Most patients died of local disease or of lymph node, lung, pleura, brain, liver, and bone metastasis.

The recognition of sarcomatoid carcinoma has important therapeutic and prognostic implications since adjuvant radiotherapy or adjuvant chemotherapy is apparently of no effect. Radical cystoprostatectomy should always be considered to bladder sarcomatoid carcinoma.

   References Top

1.Lopez-Beltran A, Pacelli A, Rothenberg HJ, Wollan PC, Zincke H, Blute ML, et al. Carcinosarcoma and sarcomatoid carcinoma of the bladder: Clinicopathological study of 41 cases. J Urol 1998;159:1497-503.   Back to cited text no. 1
2.Armstrong AB, Wang M, Eble JN, MacLennan GT, Montironi R, Tan PH, et al. TP53 mutational analysis supports monoclonal origin of biphasic sarcomatoid urothelial carcinoma (carcinosarcoma) of the urinary bladder. Mod Pathol 2009;22:113-8.   Back to cited text no. 2
3.Behrens J. The role of cell adhesion molecules in cancer invasion and metastasis. Breast Cancer Res Treat 1993;24:175-84   Back to cited text no. 3
4.Lott S, Lopez-Beltran A, Montironi R, MacLennan GT, Cheng L. Soft tissue tumors of the urinary bladder Part II: Malignant neoplasms. Hum Pathol 2007;38:963-77.  Back to cited text no. 4
5.Wick MR, Brown BA, Young RH, Mills SE. Spindle cell proliferations of theurinary tract: An immunohistochemical study. Am J Surg Pathol 1988;12:379-89.  Back to cited text no. 5

Correspondence Address:
Naorem Gopendro Singh
Department of Pathology, Al-Jahra Hospital, P.O. Box 62276, Jahra 02153
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.81635

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