1045 Papillary renal cell carcinoma (pRCC) subclassification as a prognostic factor: Comparison of type I and II pRCC with clear cell RCC and chromophobe RCC

Abstract Background: Hereditary papillary renal cell carcinoma (HPRCC) is a rare autosomal dominant disease characterized by the development of multiple and bilateral papillary type I renal cell carcinomas (RCC) and papillary adenomas caused by activating mutations in the MET proto-oncogene. Classically, distinctive histological features of RCC are described according to the familial renal cell carcinoma syndrome. To date, no clear cell RCC has been reported in HPRCC syndrome. Case presentation: We describe the case of a 51-year-old man with a germline MET mutation, who developed numerous papillary tumors but also unexpectedly clear cell renal cell carcinomas. During the follow-up, an adrenal metastasis was observed seven years after the initial diagnosis corresponding to a clear cell RCC metastasis. Using FISH, the metastatic tumor presented a trisomy of chromosomes 7 and 17. These genomic alterations are usually detected in papillary RCC, highlighting the potential link between both histological subtypes of tumors and the HPRCC syndrome.Conclusions: The pathologist must be aware that the presence of a non-papillary RCC associated with numerous papillary tumors should not exclude the diagnostic suspicion of HPRCC and thus to perform a thorough genomic study.

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Comparison of type I and II papillary renal cell carcinoma (RCC) and clear cell RCC

BJU International ◽

10.1111/j.1464-410x.2008.07999.x ◽

2008 ◽

Cited By ~ 6

Author(s):

Matthias Waldert ◽

Andrea Haitel ◽

Michael Marberger ◽

Daniela Katzenbeisser ◽

Mehmet Ozsoy ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Papillary Renal Cell Carcinoma ◽

Type I ◽

Clear Cell Rcc

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Chromophobe Renal Cell Carcinoma With Retrograde Venous Invasion and Gain of Chromosome 21: Potential Harbingers of Aggressive Clinical Behavior

International Journal of Surgical Pathology ◽

10.1177/1066896918763948 ◽

2018 ◽

Vol 26 (6) ◽

pp. 536-541 ◽

Cited By ~ 2

Author(s):

Mohsin Jamal ◽

Kanika Taneja ◽

Sohrab Arora ◽

Ravi Barod ◽

Craig G. Rogers ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Aggressive Behavior ◽

Cell Carcinoma ◽

Renal Cell ◽

Renal Vein ◽

Clear Cell ◽

Venous Invasion ◽

Chromosome 21 ◽

Chromophobe Rcc ◽

Clear Cell Rcc

Occasionally, renal cell carcinoma (RCC) with renal vein extension spreads against the flow of blood within vein branches into the kidney, forming multifocal nodules throughout the renal parenchyma. These foci are not regarded as multiple tumors but rather reverse spread of tumor along the venous system. This intravascular spread has previously been reported in clear cell RCC and RCC unclassified. However, to our knowledge, this has never been reported in chromophobe RCC. Chromophobe RCC is a unique histologic subtype of renal cancer, generally thought to have less aggressive behavior. However, it nonetheless has the potential to undergo sarcomatoid dedifferentiation, which is associated with poor prognosis. We report a unique case of a 65-year-old man with chromophobe RCC (pT3a) showing classic morphology (nonsarcomatoid), yet presenting with retrograde venous invasion and hilar lymph node metastasis at the time of right radical nephrectomy. Fluorescence in situ hybridization revealed gain of chromosome 21 with loss of multiple other chromosomes. Partial hepatectomy was performed to resect metastatic RCC 7 months after nephrectomy, revealing chromophobe RCC with classic morphology. Bone biopsy confirmed skeletal metastases 38 months after initial diagnosis. Although invasion of the renal vein and retrograde venous invasion are characteristically seen in clear cell RCC, this unusual phenomenon may also occur in chromophobe RCC, despite its unique tumor biology. This and gain of chromosome 21, which was postulated to be associated with aggressive behavior in a previous report, were associated with adverse behavior in our patient, who had short-term progression to multi-organ metastatic disease.

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Nivolumab in metastatic nonclear cell renal cell carcinoma: First results of the AcSe prospective study.

Journal of Clinical Oncology ◽

10.1200/jco.2020.38.6_suppl.699 ◽

2020 ◽

Vol 38 (6_suppl) ◽

pp. 699-699

Author(s):

Laurence Albiges ◽

Damien Pouessel ◽

Marie Beylot-Barry ◽

Guido Bens ◽

Diane Pannier ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Prospective Study ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Type I ◽

Cell Renal Cell Carcinoma ◽

Duct Carcinoma ◽

Clear Cell Rcc

699 Background: AcSe Nivolumab (N), is a non-randomised, open-label, multicentric study to investigate the efficacy and safety of nivolumab monotherapy in patients (pts) with specific rare cancers (NCT03012581). We report on the non-clear cell renal cell carcinoma (RCC) cohort. Methods: Primary endpoint was objective response rate (ORR) at 12 weeks according to RECIST1.1. All pts receives N at 240mg IV every 2 weeks. Secondary endpoints included progression free survival (PFS), overall survival (OS), best response, and safety. Results: Between 07/2017 and 02/2019, 50 pts have been enrolled across 13 institutions. Median age was 61.4 years old, 70% were male. ECOG PS was 0, 1, 2, in 29%, 63% and 8% of pts respectively. Histological types were papillary (pRCC) type 2 (41%), chromophobe (18%), pRCC type I (10%), pRCC unclassified (8%), collecting duct carcinoma (CDC) (8%), and others (including predominant sarcomatoid, renal medullary carcinoma, MITF associated RCC, unclassified RCC). N was used in first line in 16%, second line in 54% and third line or beyond in 30%. IMDC risk group was 14%, 70% and 16% for good, intermediate and poor risk respectively. With a median follow up of 10.4 months (mo), 42 pts had discontinued N. The 12 weeks-ORR was 6% (3 PR), with stable disease in 49% and PD in 44% of pts. The best ORR was 10%. Median PFS was 3.9 mo (IC95% [2.9; 8.3]). At time of analysis, 25 pts (50%) had died and 12-months OS rate was 47.7% (IC95% [33.5; 67.8]). Overall, 31 pts (62%) have presented at least one grade ≥ 3 AE. No new safety signal with N was reported. 12 weeks-ORR and best ORR according to distinct histology are presented in table 1. Pts with PR were 1pRCC type 2, 1pRCC type 1, 1 CDC, 1 MITF RCC and 1 unclassified. Conclusions: We report the first prospective study of N single agent in non-clear cell RCC. N demonstrates limited activity in a pretreated and heterogeneous non- clear cell RCC population. Interestingly 1/4 CDC developed PR while no response was noted in chromophobe RCC. Clinical trial information: NCT03012581. [Table: see text]

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Prognostic value of gain of chromosome 5q in localized renal cell carcinoma.

Journal of Clinical Oncology ◽

10.1200/jco.2019.37.7_suppl.623 ◽

2019 ◽

Vol 37 (7_suppl) ◽

pp. 623-623 ◽

Cited By ~ 1

Author(s):

Cedric Michel Lebacle ◽

Aydin Pooli ◽

Nagesh Rao ◽

Erika Louise Wood ◽

Nils Kroeger ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Prognostic Factor ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Independent Prognostic Factor ◽

Risk Of Recurrence ◽

T Stage ◽

Chromosome 5Q ◽

Clear Cell Rcc

623 Background: While gain of chromosome 5q is a frequently seen cytogenetic abnormality noted to occur in patients with renal cell carcinoma (RCC), little is known about its prognostic significance. We investigated the association of gain of 5q with disease-free survival (DFS) in patients with localized (non-metastatic T1-2) RCC. Methods: All patients from UCLA with primary tumor stage T1-2 RCC who had tumor cytogenetic analysis on tumor specimen were included. Alterations in chromosome 5q was specifically reviewed in this study. Logistic regression analyses were used to assess association of gain of 5q with final histopathology, ISUP grading, and T-stage. Cox proportional hazard modeling and Kaplan-Meier analyses were used to assess the impact of gain of 5q on DFS. Recurrence was defined as any local recurrence or development of new metastasis. Results: A total of 676 patients were included in this study. Gain of 5q occurred in 108 (16%) patients and was more commonly seen in clear cell versus non-clear cell tumors (19% vs. 9%, p = .002). Gain of 5q was associated with low grade ISUP (1 or 2) of clear-cell RCC (p = 0.011). On survival analysis, there was a 67% decreased risk of RCC recurrence for patients with gain of 5q (HR = 0.33, p = 0.018). The 5- and 10-year risk of recurrence was 2% vs. 16% and 14% vs. 28% for patients with and without gain of 5q, respectively (p = 0.013). In multivariable Cox analysis, the gain of 5q was an independent prognostic factor (p = .026 with ISUP grade and p = .025 with T-stage). These findings were confirmed among clear-cell RCC subgroup. Conclusions: Gain of chromosome 5q is an independent prognostic factor associated with decreased risk of recurrence in patients with localized T1-2 renal cell carcinoma. Identifying patients with a gain of 5q will improve the stratification of the risk of recurrence, could allow to adapt the follow-up protocols and avoid adjuvant treatment.

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Comparative Assessment of the WNT/β-Catenin Pathway, CacyBP/SIP, and the Immunoproteasome Subunit LMP7 in Various Histological Types of Renal Cell Carcinoma

Frontiers in Oncology ◽

10.3389/fonc.2020.566637 ◽

2020 ◽

Vol 10 ◽

Author(s):

Żaneta Piotrowska ◽

Michał Niezgoda ◽

Grzegorz Młynarczyk ◽

Magdalena Acewicz ◽

Irena Kasacka

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Kidney Tissue ◽

Renal Tumors ◽

Histological Types ◽

Chromophobe Rcc ◽

Study Results ◽

Clear Cell Rcc

ObjectiveThe Wnt/ß-catenin pathway plays an important role in pathogenesis of variety cancers. Most studies on changes in WNT/β-catenin pathway in renal cell carcinoma (RCC) apply only to clear cell RCC, while there are no comparative assessments of this signaling pathway in various histological types of renal tumors in the available literature. Additionally, considering the close relationship between WNT/β-catenin signaling, CacyBP/SIP and proteasomal activity, it seemed worth comparing WNT/β-catenin pathway, CacyBP/SIP and LMP7 immunoproteasome subunit in human samples of clear cell, papillary, and chromophobe RCC.MethodsTests were performed on sections of three types of kidney tumors together with surrounding unchanged tissue fragments collected from 50 patients. Samples were divided into three groups depending on the histological type of cancer: clear cell, papillary and chromophobe RCC. Immunohistochemistry and PCR methods were used to identify WNT10A, Fzd5, β-catenin, GSK-3ß, CacyBP/SIP, LMP7, and gene expression.ResultsImmunoreactivity and expression of WNT10A, Fzd5, β-catenin, GSK-3ß, CacyBP/SIP, LMP7 in clear cell RCC was markedly increased compared to non-cancerous kidney tissue. In papillary RCC, immunoreactivity and expression of WNT/β-catenin pathway, CacyBP/SIP, LMP7 was also increased compared to non-malignant kidneys, but it was less pronounced than in clear cell RCC. The least substantial increase in immunoreactivity and expression of WNT/β-catenin pathway, CacyBP/SIP, LMP7 was found in chromophobe RCC, compared to other RCC histological subtypes studied.ConclusionsStudy results suggest an important role of WNT/β-catenin pathway, CacyBP/SIP and LMP7 in RCC carcinogenesis, and may indicate new aspects of pathomechanisms leading to differences in the biology of clear cell, papillary and chromophobe RCC.

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Karyopherin Alpha 2 Is an Adverse Prognostic Factor in Clear-Cell and Papillary Renal-Cell Carcinoma

Clinical Genitourinary Cancer ◽

10.1016/j.clgc.2018.10.008 ◽

2019 ◽

Vol 17 (1) ◽

pp. e167-e175 ◽

Cited By ~ 4

Author(s):

Tim Müller ◽

Yuri Tolkach ◽

David Stahl ◽

Susanne Steiner ◽

Stefan Hauser ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Prognostic Factor ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Papillary Renal Cell Carcinoma ◽

Adverse Prognostic Factor

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Clear cell (Tubulo) papillary renal cell carcinoma: Case report

10.26226/morressier.578f37fbd462b8028d8904a2 ◽

2016 ◽

Author(s):

Ulku Kazanci

Keyword(s):

Renal Cell Carcinoma ◽

Case Report ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Papillary Renal Cell Carcinoma ◽

Renal Cell Carcinoma Case

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Two Cases of Sporadic Eosinophilic Solid and Cystic Renal Cell Carcinoma in Manitoba Population

International Journal of Surgical Pathology ◽

10.1177/1066896921993229 ◽

2021 ◽

pp. 106689692199322

Author(s):

Seyed Mohammad Mohaghegh Poor ◽

Shivani Mathur ◽

Karl Kassier ◽

Janetta Rossouw ◽

Robert Wightman ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Clear Cell ◽

Preoperative Imaging ◽

Renal Neoplasm ◽

Renal Masses ◽

Cystic Renal Cell Carcinoma ◽

Eosinophilic Cytoplasm ◽

Clear Cell Rcc

Two sporadic cases of eosinophilic solid and cystic renal cell carcinoma (ESC RCC), at our institution, are presented in this study to contribute to the growing literature on this novel renal neoplasm. The first patient was a 38-year-old female with two synchronous renal masses measuring 3.5 and 1.9 cm on preoperative imaging. The second patient was a 44-year-old female with an incidental renal mass measuring 4 cm. Both patients underwent uncomplicated radical nephrectomies. The 1.9 cm mass in the first patient was consistent with clear cell RCC. The dominant mass in the first patient and the tumor in the second patient had microscopic and macroscopic findings in keeping with ESC RCC including a tan appearance, abundant eosinophilic cytoplasm, and CK20+ and CK7− staining. Both patients had an uncomplicated course following surgery with no evidence of local recurrence or distant metastatic disease for 1 and 2 years for the first and second patient accordingly. These cases contribute to a growing body of literature regarding ESC RCC including, to our knowledge, the first reported case of synchronous ESC RCC and clear cell RCC. Further research about this novel renal neoplasm is needed.

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