scholarly journals Papillary renal cell carcinoma: what is missing in research? A case report and a review of literature

2019 ◽  
Vol 7 ◽  
pp. 2050313X1986947 ◽  
Author(s):  
Ihab Eldessouki ◽  
Ola Gaber ◽  
Mahmoud A Shehata ◽  
Tariq Namad ◽  
Joseph Atallah ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Growth Factor Receptor ◽  
Papillary Renal Cell Carcinoma ◽  
Type I ◽  
Renal Cell Carcinomas ◽  
Cancer Statistics ◽  
Male Predominance ◽  
Endothelial Growth Factor

The incidence of renal cell carcinomas in adults ranges has been increasing over the past decades in both men and women. Once the incidence was 2.9%, now is reported to have increased to 3%–5% with male predominance according to the most recent reports of cancer statistics. The disease typically describes a group of different histopathological subtypes; the most common is clear cell carcinoma which accounts for 70%–80% of the diagnosed cases, while papillary renal cell carcinoma and chromophobe types represent 20% and 5%, respectively. In 1996, the renal cell carcinomas Heidelberg classification was introduced by Delahunt et al. It divides renal cell tumors into benign and malignant parenchymal neoplasms, excluding Wilm’s tumor and secondary metastases and limiting each subcategory to the most commonly documented genetic abnormalities, if applicable. In this report, we discuss a case of metastatic type I papillary renal cell carcinoma treated with the anti-vascular endothelial growth factor receptor sunitinib and showing marked long-term clinical response. Through this case, we highlight the importance of re-classifying papillary renal cell carcinoma subtypes to prioritize the clinical management of these cases.

scholarly journals Various Histological Types of Renal Cell Carcinoma Associated With Hereditary Papillary Renal Cell Carcinoma (HPRCC): a Case Report

2021 ◽  
Author(s):  
Sophie FERLICOT ◽  
Pierre-Alexandre Just ◽  
Eva Compérat ◽  
Etienne Rouleau ◽  
Frédérique Tissier ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Papillary Renal Cell Carcinoma ◽  
Type I ◽  
Renal Cell Carcinomas ◽  
Genomic Study ◽  
Papillary Rcc ◽  
Clear Cell 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.

scholarly journals The effect of the papillary renal cell carcinoma subtype on oncological outcomes

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Honghong Pan ◽  
Liefu Ye ◽  
Qingguo Zhu ◽  
Zesong Yang ◽  
Minxiong Hu
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Papillary Renal Cell Carcinoma ◽  
Clinicopathological Features ◽  
Type I ◽  
Type Ii ◽  
Oncological Outcomes ◽  
Stage Grouping ◽  
Surgical Methods

AbstractThe study aimed to compare the clinicopathological features and prognosis between type I and type II papillary renal cell carcinoma (PRCC) and to investigate whether the subtypes of PRCC would affect oncological outcomes. A total of 102 patients with PRCC were recruited, of which 42 were type I PRCC and 60 type II. The clinicopathological features and oncologic outcomes of the patients were evaluated. The type II cases had a higher WHO/ISUP grading (P < 0.001), T (P = 0.003), N (P = 0.010) stage and stage grouping (P = 0.011) than the type I. During a median follow-up period of 61.4 months, 1-year cancer specific survival (CSS) of the type I was 100%, 5-year CSS was 95.2%, the 1-year CSS of the type II was 96.2%, and 5-year CSS was 75.7%. The univariate analysis showed that subtype, symptoms, TNM, stage grouping, WHO/ISUP grading and surgical methods appeared to affect prognosis of the patients with PRCC. However, multivariate analysis revealed that only stage grouping was the independent risk factor. After the stage grouping factor was adjusted for the analysis, there were no statistically significant differences in CSS (P = 0.214) and PFS (P = 0.190) between the localized type I and type II PRCC groups. Compared with type I PRCC, type II had higher pathological T, N stage and WHO/ISUP grading. However, it was the Stage grouping that made a great difference to oncological outcomes, rather than the subtype of PRCC.

scholarly journals Clear Cell Papillary Renal Cell Carcinoma: A Potential Mimic of Conventional Clear Cell Renal Carcinoma on Core Biopsy

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Heath Liddell ◽  
Anton Mare ◽  
Sean Heywood ◽  
Genevieve Bennett ◽  
Hin Fan Chan
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Core Biopsy ◽  
Clear Cell ◽  
Incidental Finding ◽  
Papillary Renal Cell Carcinoma ◽  
Renal Cell Carcinomas ◽  
Fuhrman Grade ◽  
Stage Renal Disease

Clear cell papillary renal cell carcinoma (CCP-RCC) is a recently described, relatively uncommon variant of renal cell carcinoma (RCC) with a reported incidence of 4.1%. Thought to only arise in those with end stage renal disease, CCP-RCC is increasingly identified in those without renal impairment. CCP-RCCs have unique morphologic, genetic, and immunohistochemical features distinguishing them from both conventional clear cell renal cell carcinomas and papillary renal cell carcinomas. Immunohistochemically, these tumors are positive for CK7 and negative for CD10 and racemase. This is in contrast to conventional cell renal cell carcinomas (CK7 negative, CD10 positive) and papillary cell carcinomas (CK7, CD10, and racemase positive). These tumours appear to be indolent in nature, with no current documented cases of metastatic spread. We present the case of a 42-year-old female who presented with an incidental finding of a renal mass that on a core biopsy was reported as clear cell carcinoma, Fuhrman grade 1. She subsequently underwent a radical nephrectomy and further histological examination revealed the tumor to be a clear cell papillary renal cell carcinoma, Fuhrman grade 1.

scholarly journals Integrative Clinical and Molecular Characterization of Translocation Renal Cell Carcinoma

2021 ◽  
Author(s):  
Ziad Bakouny ◽  
Ananthan Sadagopan ◽  
Praful Ravi ◽  
Nebiyou Y. Metaferia ◽  
Jiao Li ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Growth Factor Receptor ◽  
Antioxidant Response ◽  
Vascular Endothelial ◽  
Molecular Features ◽  
Somatic Alterations ◽  
Homozygous Deletions ◽  
Endothelial Growth Factor

Translocation renal cell carcinoma (tRCC) is an aggressive and poorly-characterized subtype of kidney cancer driven by MiT/TFE gene fusions. Here, we define the landmarks of tRCC through an integrative analysis of 152 tRCC patients identified across multiple genomic, clinical trial, and retrospective cohorts. Most tRCCs harbor few somatic alterations apart from MiT/TFE fusions and homozygous deletions at chromosome 9p21.3 (19.2% of cases). Transcriptionally, tRCCs display a heightened NRF2-driven antioxidant response that is associated with resistance to many targeted therapies. Consistently, we find that outcomes for tRCC patients treated with vascular endothelial growth factor receptor inhibitors (VEGFR-TKI) are worse than those treated with immune checkpoint inhibition (ICI). Multiparametric immunofluorescence confirmed the presence of CD8+ tumor-infiltrating T cells compatible with a clinical benefit from ICI and revealed an exhaustion immunophenotype distinct from clear cell RCC. Our findings comprehensively define the clinical and molecular features of tRCC and may inspire new therapeutic hypotheses.

scholarly journals MP80-03 PROGNOSTIC DIFFERENCES AND SURVIVAL OUTCOMES IN PATIENTS WITH PAPILLARY RENAL CELL CARCINOMA SUBTYPES &NDASH; A COMPARISON BETWEEN TYPE I VS. TYPE II

2020 ◽  
Vol 203 ◽  
pp. e1225
Author(s):  
Giuseppe Rosiello* ◽  
Fabio Muttin ◽  
Alessandro Nini ◽  
Gianfranco Baiamonte ◽  
Carlo Andrea Bravi ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Papillary Renal Cell Carcinoma ◽  
Survival Outcomes ◽  
Type I ◽  
Type Ii

scholarly journals Focal Segmental Glomerulosclerosis Preceding Type 2 Papillary Renal Cell Carcinoma

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Dominik Dabrowski ◽  
Ekin Ozluk ◽  
Silvia Barbeito ◽  
Eric X. Wei
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Focal Segmental Glomerulosclerosis ◽  
Paraneoplastic Syndrome ◽  
Renal Cell ◽  
Renal Mass ◽  
Papillary Renal Cell Carcinoma ◽  
Type I ◽  
Type Ii ◽  
Segmental Glomerulosclerosis

Renal cell carcinoma (RCC) is the predominant renal malignancy in adults. Of the four general subtypes, papillary renal cell carcinoma (P-RCC) is the second most common and can be subdivided into type I, type II, and a mixture of type I and II. Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy at all ages, and it can be seen as a paraneoplastic syndrome. RCC, in general, is known to present with many paraneoplastic syndromes, and glomerulopathies are among these. Rarely, RCC and glomerulopathies may overlap in the same patient. Here, we report a 58-year-old male with a past medical history of FSGS and chronic kidney disease (CKD), stage III, who was found to have an incidental renal mass that was later diagnosed as type II P-RCC. The histology showed pseudostratified tumor cells with an eosinophilic cytoplasm that formed papillary configurations and displayed areas of necrosis. The prior FSGS diagnosis exhibited segmental sclerosis, refractory tufts, and capillary membrane wrinkling. A period of 1.5 years elapsed between the diagnosis of the glomerulopathy and the malignancy. The tumor was found to be at stage TIb. To our knowledge, this may be the first reported case of usual-type FSGS as paraneoplastic glomerulopathy (PG) preceding P-RCC. Because FSGS only sparingly affects the kidney and is a common glomerulopathy in adults, it is reasonable to complete comprehensive diagnostic studies and commence medically necessary treatment, especially in the background of other renal comorbidities. These preexisting comorbidities may be associated with malignancy very early in its course. The probability of RCC-associated paraneoplastic glomerulopathy is low, which means an already incidentally found renal mass may conceal a serpentine paraneoplastic syndrome. A more developed understanding of these manifestations can lead experienced clinicians to suspect and possibly uncover an insidious RCC before it advances.

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