Multifocal synchronous renal cell carcinoma of three different histologic subtypes: unusual findings and literature review

Chavez, Martha; Dharia, Ashka; Asarian, Armand; Xiao, Philip

doi:10.1093/jscr/rjac452

“…Tumor cells of the papillary subtype showed a large number of eosinophilic, atypical nuclei and prominent nucleoli, whose morphology was not excluded FH-deficient RCC, but no FH protein or molecular associated detection was performed to confirm it. 18 Here, we report a patient with detailed molecular analysis. In our case, clear cell RCC (right) was more likely caused by the VHL somatic frameshift mutation, whereas FH-deficient RCC (left) was caused by the FH germline mutation.…”

Section: Discussionmentioning

confidence: 97%

“…Tumor cells of the papillary subtype showed a large number of eosinophilic, atypical nuclei and prominent nucleoli, whose morphology was not excluded FH-deficient RCC, but no FH protein or molecular associated detection was performed to confirm it. 18…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Synchronous Fumarate Hydratase-Deficient Renal Cell Carcinoma and Clear Cell Renal Cell Carcinoma With Fumarate Hydratase and von Hippel-Lindau Gene Mutations: A Clinicopathologic and Molecular Study

Wen,

Zheng,

Zhang

et al. 2023

Int J Surg Pathol

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Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) is a rare and aggressive tumor characterized by pathogenic alterations in the fumarate hydratase ( FH) gene. Clear cell renal cell carcinoma (clear cell RCC) is a common renal cell carcinoma (RCC) associated with von Hippel-Lindau ( VHL) gene variations. Here, we reported a case of bilateral RCCs. A 60-year-old man was admitted to hospital with a 3.6 cm × 3.3 cm mass in the right kidney and a 2.8 cm × 2.3 cm nodule in the left kidney. Pathologically, the right tumor showed a nested growth pattern of cells with clear cytoplasm and was FH positive and 2-succinylcysteine (2SC) negative. The left tumor demonstrated a high-grade papillary pattern and was FH negative and 2SC positive. Whole-exome sequencing and Sanger sequencing identified a germline FH c.563A > T mutation in both the tumors and an additional somatic VHL c.479_480insA mutation in the right tumor, confirming the diagnosis of clear cell RCC and FH-deficient RCC in the right and left kidneys, respectively. We reported a rare case of synchronous bilateral clear cell RCC (right) and FH-deficient RCC (left) likely driven by somatic VHL mutation and germline FH mutation, respectively.

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“…The life expectancy of the patient with end stage renal disease must be at least 5-years after considering the co-morbidities to include in the wait list for renal subtypes [2][3][4][5]. Martha C, et al described 3 subtypes clear cell, type 1 and type 2 papillary renal cell carcinoma, but not chromophobe renal cell carcinoma [6]. Bilateral radical nephrectomy in renal failure patients who are on dialysis is not commonly done [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

A Rare Presentation of Bilateral Multifocal and Multitype Renal Cell Carcinoma in End Stage Renal Disease

Kabilan,

Edwin,

Seethalakshmi

et al. 2024

Int Arch Urol Complic

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Introduction: Renal cell carcinoma (RCC) is the most common tumour of the kidney. The common histological subtypes are clear cell, papillary and chromophobe renal cell carcinomas. This case has several unique features, which makes it a rare presentation. The most important unique fact is the presence of all the three common histological types of renal cell carcinoma in the same patient, which is a rarity.Presentation: 63-years-old male from Tanzania with End Stage Renal Disease came to hospital for Live Donor renal transplantation. Incidental detection of left renal tumor of size 3 cm diameter in the lower pole of kidney was made. He underwent laparoscopic radical nephrectomy. The histopathology was clear cell renal cell carcinoma. He came back later after 3 years with persistent cough. On evaluation was found to have a 5 cm pulmonary metastasis and two tumors in the upper pole of right kidney. One of the tumours was 3 cm and was solid and the other was 2.5 cm complex cystic tumor. Laparoscopic right radical nephrectomy with partial pneumonectomy was done. Histopathology was papillary renal cell carcinoma in the cystic renal tumor and pulmonary metastasis. Chromophobe renal cell carcinoma reported in the solid renal tumor. The following immunohistochemical markers were used in the sub typing: Cytokeratin 7, vimentin and progesterone receptor. Conclusion:Multitype and multifocal renal cell carcinoma though rare we may come across them. In these situations, it is prudent to suspect HRTS (Hereditary renal tumor syndrome). If these patients are on hemodialysis with ESRD a radical nephrectomy would be better option than partial nephrectomy. B/L radical nephrectomy in ESRD patients can be done in patients with good vascular access.

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“…The life expectancy of the patient with end stage renal disease must be at least 5-years after considering the co-morbidities to include in the wait list for renal subtypes [2][3][4][5]. Martha C, et al described 3 subtypes clear cell, type 1 and type 2 papillary renal cell carcinoma, but not chromophobe renal cell carcinoma [6]. Bilateral radical nephrectomy in renal failure patients who are on dialysis is not commonly done [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Untitled

2024

Int Arch Urol Complic

0

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Introduction: Renal cell carcinoma (RCC) is the most common tumour of the kidney. The common histological subtypes are clear cell, papillary and chromophobe renal cell carcinomas. This case has several unique features, which makes it a rare presentation. The most important unique fact is the presence of all the three common histological types of renal cell carcinoma in the same patient, which is a rarity.Presentation: 63-years-old male from Tanzania with End Stage Renal Disease came to hospital for Live Donor renal transplantation. Incidental detection of left renal tumor of size 3 cm diameter in the lower pole of kidney was made. He underwent laparoscopic radical nephrectomy. The histopathology was clear cell renal cell carcinoma. He came back later after 3 years with persistent cough. On evaluation was found to have a 5 cm pulmonary metastasis and two tumors in the upper pole of right kidney. One of the tumours was 3 cm and was solid and the other was 2.5 cm complex cystic tumor. Laparoscopic right radical nephrectomy with partial pneumonectomy was done. Histopathology was papillary renal cell carcinoma in the cystic renal tumor and pulmonary metastasis. Chromophobe renal cell carcinoma reported in the solid renal tumor. The following immunohistochemical markers were used in the sub typing: Cytokeratin 7, vimentin and progesterone receptor. Conclusion:Multitype and multifocal renal cell carcinoma though rare we may come across them. In these situations, it is prudent to suspect HRTS (Hereditary renal tumor syndrome). If these patients are on hemodialysis with ESRD a radical nephrectomy would be better option than partial nephrectomy. B/L radical nephrectomy in ESRD patients can be done in patients with good vascular access.

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Multifocal synchronous renal cell carcinoma of three different histologic subtypes: unusual findings and literature review

Cited by 3 publications

References 8 publications

A Rare Case of Synchronous Fumarate Hydratase-Deficient Renal Cell Carcinoma and Clear Cell Renal Cell Carcinoma With Fumarate Hydratase and von Hippel-Lindau Gene Mutations: A Clinicopathologic and Molecular Study

A Rare Case of Synchronous Fumarate Hydratase-Deficient Renal Cell Carcinoma and Clear Cell Renal Cell Carcinoma With Fumarate Hydratase and von Hippel-Lindau Gene Mutations: A Clinicopathologic and Molecular Study

A Rare Presentation of Bilateral Multifocal and Multitype Renal Cell Carcinoma in End Stage Renal Disease

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