Renal Pleomorphic Sarcoma in Polycystic Kidney Disease: Case Report

Ganouni, N. Chérif Idrissi El; Idrissi, M. Ouali; Jiddi, Karima; Dahami, Z.; Barjani, F.; Sarf, I.; Belaabidia, B.; Essadki, O.; Ousehal, A.

doi:10.4236/ojrad.2012.23012

Cited by 3 publications

(4 citation statements)

References 9 publications

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Section: Discussionmentioning

confidence: 99%

“…2 But the association of APKD with nonepithelial neoplasms is rare with only a few cases being reported. [3][4][5] The advances in dialysis and management of ESRD has led to increased survival of patients with APKD and consequently the underlying risk of malignant transformation. Our patient had been diagnosed with APKD 20 years back, and had been on maintenance dialysis for the past 6 years.…”

Section: Discussionmentioning

confidence: 99%

“…Even in cases which are clearly neoplastic based on clinical and imaging findings, the tumor characteristics seen on imaging may not be enough to reliably differentiate RCC from other neoplasms. [3][4][5] It is difficult to differentiate HS from other renal malignancies based on clinical presentation, which tend to be nonspecific as patients typically complain with of flank pain or abdominal pain and hematuria. However, systemic B symptoms, such as fever and malaise seen in our patient, are common in hematopoietic malignancies.…”

Section: Discussionmentioning

confidence: 99%

“…2 However, only a few cases of nonepithelial neoplasms arising in APKD have been reported. [3][4][5] Histiocytic sarcoma (HS), a hematopoietic malignant neoplasm accounting for less than 1% of hematologic malignancies, was first described as histiocytic medullary reticulosis by Rappaport et al in 1939. 6 The World Health Organization defines HS as a malignant proliferation of cells with morphologic and immunophenotypic features of mature histiocytes.…”

Section: Introductionmentioning

confidence: 99%

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Primary Histiocytic Sarcoma in Adult Polycystic Kidney Disease: Case Report and Review of Literature

et al. 2020

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Genetically driven tissue destruction followed by remodeling in adult polycystic kidney disease (APKD) raises the possibility of malignant transformation. Renal cell carcinoma (RCC) associated with APKD has been frequently reported in the literature; however, only a few cases of nonepithelial neoplasms arising in APKD have been described so far. Histiocytic sarcoma (HS) is a lymphohematopoietic malignant neoplasm that accounts for less than 1% of hematologic malignancies. In this article, we describe a case of primary HS occurring in a 61-year-old man with end-stage renal disease secondary to APKD. This is the first reported case of primary HS in the setting of APKD. The aberrant h-caldesmon expression seen in this case is another novel finding that has previously not been described. This case highlights the importance of morphology in guiding diagnostic workup and reiterates the necessity of maintaining a high index of suspicion for neoplastic entities in APKD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Primary Histiocytic Sarcoma in Adult Polycystic Kidney Disease: Case Report and Review of Literature

et al. 2020

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Sarcomatoid Urothelial Carcinoma Arising in Autosomal Dominant Polycystic Kidney Disease: A Case Report and Literature Review

Bolous,

Trpkov,

Siadat

et al. 2024

Int J Surg Pathol

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Autosomal dominant polycystic kidney disease (ADPKD) may be associated with various epithelial malignancies. The most reported ones are papillary renal cell carcinoma (RCC) and clear cell RCC. Only one noninvasive urothelial carcinoma arising in the renal pelvis has been previously reported in the setting of ADPKD in the English literature. A 52-year-old patient with ADPKD and a history of renal transplant presented with a poorly differentiated sarcomatoid neoplasm in his native left polycystic kidney. A recognizable urothelial or renal cell carcinoma differentiation was not identified in the resected neoplasm microscopically. The initial diagnosis for this specimen was challenging on morphology and immunohistochemistry, but targeted next-generation sequencing provided molecular evidence in support of urothelial origin, indicating a hotspot mutation −124 C > T in the TERT promoter (C228 T) and loss of heterozygosity on chromosomes 9p and 8p. This tumor is unique because, to our knowledge, this is the first report of upper tract sarcomatoid urothelial carcinoma in a patient with ADPKD.

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Metastatic Undiff erentiated Pleomorphic Sarcoma of Kidney: Presenting as Hematuria - A Case Report

Gurwale,

Iqbal,

Choudhury

et al. 2023

Journal of Medical Sciences

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Pleomorphic undifferentiated sarcoma (PUS) is an extremely aggressive soft-tissue sarcoma that occurs most commonly in the trunk and the extremities but is extremely rare in the kidney. This entity has no distinctive clinical or radiological signs for diagnosis. It has high local recurrence and distant metastasis rates along with poor prognosis. We describe a case of PUS of the left kidney with urinary bladder metastases. Radiological investigations suggested an initial diagnosis of multifocal urothelial carcinoma; however, a definitive and confirmatory diagnosis of PUS was made on the basis of histopathological and immunohistochemical examination. Subsequently, left nephroureterectomy along with bladder cuff excision was performed and postoperative histopathology revealed PUS. Follow-up investigations revealed metastatic deposits of tumor in the base of the urinary bladder for which an endoscopic surgery of transurethral resection of the bladder tumor was performed. The histopathological findings were consistent with metastatic high-grade PUS of the kidney with metastasis to the bladder.

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Renal Pleomorphic Sarcoma in Polycystic Kidney Disease: Case Report

Cited by 3 publications

References 9 publications

Primary Histiocytic Sarcoma in Adult Polycystic Kidney Disease: Case Report and Review of Literature

Primary Histiocytic Sarcoma in Adult Polycystic Kidney Disease: Case Report and Review of Literature

Sarcomatoid Urothelial Carcinoma Arising in Autosomal Dominant Polycystic Kidney Disease: A Case Report and Literature Review

Metastatic Undiff erentiated Pleomorphic Sarcoma of Kidney: Presenting as Hematuria - A Case Report

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