| Abstract|| |
Sarcomatoid carcinoma is a rare malignant tumor that has both malignant epithelial and mesenchymal components. We describe a sarcomatoid carcinoma arising in the left renal pelvis of a 49-year-old man. The dominant component of the tumor was chondrosarcomatous, but there were also focal carcinomatous areas. The carcinomatous tumor cells consisted of papillary urothelial carcinoma. Immunohistochemical assay showed that the sarcomatous tumor cells were positive for vimentin and S 100 and negative for cytokeratin. The papillary urothelial carcinoma was positive for cytokeratin and negative for vimentin. The patient underwent neoadjuvant chemotherapy and after downsizing the tumor, radical nephrectomy was performed with excision of the cuff of bladder.
Keywords: Chondrosarcomatous element, renal pelvis, sarcomatoid carcinoma
|How to cite this article:|
Ramakrishnan D, Subachitra T, Padmavathi R, Devi GD. A case report of a urothelial carcinoma arising in the renal pelvis with exuberant chondrosarcomatous element associated with adrenal metastasis. Indian J Pathol Microbiol 2014;57:284-6
|How to cite this URL:|
Ramakrishnan D, Subachitra T, Padmavathi R, Devi GD. A case report of a urothelial carcinoma arising in the renal pelvis with exuberant chondrosarcomatous element associated with adrenal metastasis. Indian J Pathol Microbiol [serial online] 2014 [cited 2022 Jan 20];57:284-6. Available from: https://www.ijpmonline.org/text.asp?2014/57/2/284/134717
| Introduction|| |
Urothelial carcinoma of the renal pelvis makes up to 7% of all renal tumors.  Majority of the upper urinary tract tumors are urothelial with a small percentage being either squamous or glandular.  In a study by Olgae et al. most of the renal pelvis neoplasms were found to be high grade. Urothelial carcinoma is known for its divergent differentiation. Several morphologic variants have been reported in literature and they have been recognized by WHO in the classification of urothelial neoplasms. Here, we describe a case of urothelial carcinoma of renal pelvis with exuberant chondrosarcomatous elements in a 49-year-old man.
| Case report|| |
A 49-year-old male chronic smoker presented with a history of the left flank pain and hematuria for 4 months. Radiological imaging studies revealed a mass lesion in the collecting system of the left kidney infiltrating into left renal cortex with perinephric extension. The mass was found to occupy the entire left ureter and was seen to protrude into bladder [Figure 1]. Cystoscopic biopsy was reported as high grade urothelial carcinoma and a radical nephroureterectomy with excision of cuff of the bladder was performed. Patient was put on neoadjuvant chemotherapy. Radiologically mass had considerably reduced in size on follow-up and the tumor burden reduced.
Gross: Resected nephrectomy specimen measured 13 cm × 10 cm × 4 cm with attached ureter measuring 13 cm. External surface of the kidney was covered with perinephric pad of fat and appeared smooth. Cut surface of the kidney was entirely replaced by a cystic cavity enclosing a nodular growth in the pelvic region measuring 10 cm × 7 cm extending up to perinephric pad of fat. No corticomedullary differentiation was made out in the kidney. The growth was also involving ureter to its entire length with multiple irregularities in the ureter [Figure 2]a].
|Figure 2: (a) Gross picture showing the mass involving renal pelvis. (b) Low power picture showing the sarcomatoid areas. (c) High power picture showing the chondrosarcomatous areas. (d) Adrenal metastasis|
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Microscopic picture showed renal parenchyma with a neoplasm composed predominantly of sheets and clusters of pleomorphic polyhedral to spindle cells with vesicular nuclei [Figure 2]b and c]. Extensive foci of cartilageneous differentiation made out with chondroid matrix showing pleomorphic chondrocytes with bizarre nuclei. Foci of necrosis hemorrhage and inflammatory infiltrate was made out. Multinucleate tumor giant cells and lymphovascular invasion was seen. Tumor cells were seen to infiltrate into the perinephric pad of fat and muscle. Adherent nodule in perinephric pad of fat showed adrenal parenchyma with tumor infiltration [Figure 2]d]. One node retrieved showed reactive hyperplasia. Furthermore seen were xanthogranulomatous changes in the excised bladder cuff (probably as a result of chemotherapy). Immunohistochemistry was done and epithelial elements were found to be positive for cytokeratin and mesenchymal elements were found to be positive for vimentin [Figure 3]a-c].
|Figure 3: (a) Vimentin positive areas. (b) Cytokeratin positive epithelial areas. (c) S 100 positive chondroid areas|
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| Discussion|| |
The first case of sarcomatoid carcinoma of the renal pelvis was reported by Piscioli et al. in 1984. Until date, only about 20 cases of sarcomatoid carcinoma of renal pelvis have been reported [Table 1].
Grossly this tumor is large with lobulated, fleshy grey white cut surface. Microscopically the carcinomatous components may be separate or admixed with sarcomatous areas with patterns reminiscent of leiomyosarcoma, fibrosarcoma, angiosarcoma, or rhabdomyosarcoma which is rarely seen.
The main differential diagnosis includes true sarcoma, pseudosarcomatous mesenchymal proliferations, renal cell carcinoma with a scar and granulation tissue, malignant fibrous histiocytoma, rhabdomyosarcoma, angiosarcoma, fibrosarcoma and mucinous tubular and spindle cell sarcoma.  This tumor has a worse prognosis and is associated with recurrence and frequent metastasis.
There are various theories for development of sarcomatoid element in an urothelial carcinoma. Expression of vimentin intermediate filaments may be responsible for adoption of a mesenchymal growth pattern,  anaplastic changes can be associated with the loss of expression of cell adhesion molecules  and it may be associated with deletions of 9p, 9q, 17p, and p53  mutations.
This case has been reported for its extreme rarity. In cases of sarcomatoid carcinoma demonstration of transition with in situ urothelial carcinoma can be helpful. Extensive sampling from the specimen along with adequate immunohistochemical panel would help in establishing the diagnosis.
| References|| |
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