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      Sarcomatoid Carcinoma of Renal Pelvis Involving Ureter and Renal Parenchyma with Heterologous Osteosarcomatous Differentiation: A Case Report and Review of Literature

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          Abstract

          Sarcomatoid carcinoma is a highgrade rare malignant tumor with both epithelial and mesenchymal components. Sarcomatoid carcinoma in the upper urinary tract is very rare. We reported here a case of sarcomatoid carcinoma of renal pelvis with osteosarcomatous differentiation, with involvement of the ureter and renal parenchyma in a 68-year-old female. Histologically, predominant pleomorphic spindle cell sarcoma component with osteoid production and urothelial carcinoma component with in situ areas were identified. Immunohistochemical analysis showed vimentin positivity in sarcomatous component and cytokeratin positivity in carcinomatous component.

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          Histogenesis of sarcomatoid urothelial carcinoma of the urinary bladder: evidence for a common clonal origin with divergent differentiation.

          The histogenesis of sarcomatoid urothelial carcinoma, a rare neoplasm with bidirectional epithelial and mesenchymal differentiation, has been a matter of controversy. To clarify its origin, we analysed the status of X-chromosome inactivation in sarcomatoid urothelial carcinomas from 10 female patients and examined losses of heterozygosity (LOH) in these specimens and in additional 20 tumours from male patients. Six polymorphic microsatellite markers where genetic alterations occur frequently in early or advanced stages of urothelial carcinomas, including D3S3050, D8S261, IFNA, D9S177, D11S569 and TP53, were investigated in the current study. The identical pattern of non-random X-chromosome inactivation in both carcinomatous and sarcomatous components was identified in five of eight informative female patients, and the remaining three informative cases showed a random, but concordant, pattern of X-chromosome inactivation. The concordant X-chromosome inactivation results in all eight informative cases support the concept of a monoclonal origin of both components of this biphasic neoplasm. Among the tumours demonstrating loss of heterozygosity, high incidences of an identical pattern of allelic loss between carcinomatous and sarcomatous components were identified in genetic alterations associated with early carcinogenesis: 86% at D8S261, 78% at D11S569, 75% at D9S177 and 57% at IFNA. In contrast, concordant LOH patterns were less frequently observed for microsatellites related to advanced carcinogenesis: only 40% at D3S3050 and 40% at TP53. The significant overlap of loss of heterozygosity supports a monoclonal cell origin and suggests that clonal divergence may occur during tumour progression and differentiation. Divergent patterns of discordant allelic loss of microsatellite markers imply that heterogeneous pathogenetic pathways may exist in the evolution of this enigmatic neoplasm. Copyright (c) 2007 Pathological Society of Great Britain and Ireland.
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            Sarcomatoid carcinoma of transitional cell origin confined to renal pelvis.

            Thirteen reported cases of renal pelvic sarcomatoid carcinoma have been documented since 1961, and all patients presented with metastatic disease or advanced renal parenchyma involvement. The mean survival was shorter than 9 months, and adjuvant therapy appeared to offer no benefit. We present the case of a 61-year-old man with gross hematuria and a large filling defect on computed tomography excretory urography. Surgical pathologic examination after laparoscopic nephroureterectomy was consistent with renal pelvis-confined sarcomatoid carcinoma of transitional cell origin. Adjuvant therapy was not given secondary to the organ-confined nature of disease. The patient was without recurrence for more than 1 year.
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              Use of PAX8 and GATA3 in diagnosing sarcomatoid renal cell carcinoma and sarcomatoid urothelial carcinoma.

              Immunohistochemistry for PAX8 and GATA3 are sensitive markers for renal cell carcinoma and urothelial carcinoma, respectively. However, there are limited data on these markers in sarcomatoid renal cell carcinoma (SARCRCC) and sarcomatoid urothelial carcinoma (SARCUC). Tissue microarrays (TMAs) were constructed from 45 cases of SARCRCC and 45 cases of SARCUC of the lower urinary tract, with an additional 11 SARCUCs of the upper tract. PAX8 and GATA3 were also evaluated in TMAs from 161 sarcomas from other sites, 14 atypical epithelioid angiomyolipomas (AMLs) of the kidney, 23 bladder inflammatory myofibroblastic tumors (IMTs), and 2 bladder and 4 renal leiomyosarcomas. In the SARCRCC, PAX8 and GATA3 were positive in the sarcomatoid areas in 31 (69%) and 0 (0%) of cases, respectively. In the bladder SARCUC, GATA3 and PAX8 were positive in 14 (31%) and 2 (4%) of cases, respectively. Of the 11 SARCUCs of the upper urinary tract, 2 (18%) cases were PAX8 positive and 2 (18%) separate cases were GATA3 positive. Only 1 tumor present on the sarcoma TMAs, a Ewing sarcoma/primitive neuroectodermal tumor, was PAX8 positive, and all sarcomas were GATA3 negative. Of the AMLs, IMTs, and leiomyosarcoma, only 1 case of IMT showed moderate GATA3 positivity, and all were negative for PAX8. PAX8 can be used to distinguish SARCCRCC from atypical epithelioid AMLs and primary renal or retroperitoneal sarcomas. However, in a kidney/renal pelvic tumor, PAX8 shows overlap in staining between SARCUC and SARCRCC. GATA3 lacks sensitivity but is more specific for SARCUC. Copyright © 2013 Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Iran J Pathol
                Iran J Pathol
                IJP
                Iranian Journal of Pathology
                Iranian Society of Pathology (Tehran, Iran )
                1735-5303
                2345-3656
                Winter 2018
                2017
                : 13
                : 1
                : 89-93
                Affiliations
                [1 ] Dept. of Pathology, Government Medical College, Kottayam, Kerala, India
                [2 ] Dept. of Genitourinary Surgery, Government Medical College, Kottayam, Kerala, India
                Author notes
                [* ]Corresponding Information:Dr.Bhavya P.Mohan, Assistant Professor, Pathology, Medical college, Kottayam, E-mail: bhavyasharath80@gmail.com
                Article
                ijp-13-089
                5929394
                29731801
                7997642f-1c93-4b61-9c0e-486770b0fd92
                © 2017, IRANIAN JOURNAL OF PATHOLOGY.

                This is an open-access article distributed under the terms of the Creative Commons Attribution-noncommercial 4.0 International License, ( https://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

                History
                : 31 August 2016
                : 10 October 2017
                Categories
                Case Report

                sarcomatoid carcinoma,renal pelvis,osteosarcoma
                sarcomatoid carcinoma, renal pelvis, osteosarcoma

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