Renal Cell Carcinoma with t(X;17): Singular Pediatric Neoplasm with Specific Phenotype/Genotype Features

2003 ◽  
Vol 6 (1) ◽  
pp. 84-87 ◽  
Author(s):  
Eduardo Zambrano ◽  
Miguel Reyes-Múgica

Renal cell carcinomas in children are extremely rare and are usually associated with specific chromosomal rearrangements, different from those seen in adult patients. We present the case of a 9-year-old girl with a renal cell carcinoma with t(X;17) diagnosed at our institution. We also review the pertinent literature, with an emphasis on the genetic and molecular aspects associated with this rare neoplasm.

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Jasper Decoene ◽  
Filip Ameye ◽  
Evelyne Lerut ◽  
Raymond Oyen ◽  
Hein Van Poppel ◽  
...  

Renal cell carcinomas (RCCs) are known for their unpredictable metastatic pattern. We present the case of a 63-year-old woman who initially presented in 1992 with a metastasis in the left calcaneus that led to the discovery of RCC. In 1998, a new metastasis was found in the ovary. In 2008, the diagnosis of a gallbladder metastasis was made. All metastases were surgically removed; no additional systemic therapies were used. Aggressive surgical treatment can prolong the survival of patients with resectable metastases. Patterns of metastasis are discussed, and a brief review of the literature is given regarding each localization.


Author(s):  
Dae Y. Kim ◽  
Christopher G. Wood ◽  
Jose A. Karam

OVERVIEW: The incidental renal mass represents a heterogeneous group that contains both benign and malignant pathologies. The majority of renal cell carcinomas are discovered incidentally, without the presence of symptoms directly related to the mass, and are closely associated with the term small renal masses because of the discovery before the onset of symptoms. In general, small renal masses are defined as 4 cm or smaller, and may account for greater than half of renal cell carcinoma diagnosis. The use of renal mass biopsy may offer additional pathological information but the clinician must be reminded of the technical and diagnostic limitations of renal mass biopsy. Patient-dependent factors, such as life expectancy and comorbidities, guide the management of small renal masses, which include active surveillance, partial nephrectomy, radical nephrectomy, and ablative techniques (cryoablation and radiofrequency ablation). Partial nephrectomy has demonstrated durable oncologic control for small renal masses while preserving renal function and, if feasible, is the current treatment of choice. In the other extreme of the renal cell carcinomas spectrum and in the presence of metastatic disease, the removal of the renal primary tumor is termed cytoreductive nephrectomy. Two randomized trials (SWOG 8949 and EORTC 30947) have demonstrated a survival benefit with cytoreductive nephrectomy before the initiation of immunotherapy. These two studies have also been the motivation to perform cytoreductive nephrectomy in the targeted therapy era. Currently, there are two ongoing randomized prospective trials accruing to investigate the timing and relevance of cytoreductive nephrectomy in the contemporary setting of targeted therapy.


2019 ◽  
Vol 7 ◽  
pp. 2050313X1986947 ◽  
Author(s):  
Ihab Eldessouki ◽  
Ola Gaber ◽  
Mahmoud A Shehata ◽  
Tariq Namad ◽  
Joseph Atallah ◽  
...  

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.


2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Heath Liddell ◽  
Anton Mare ◽  
Sean Heywood ◽  
Genevieve Bennett ◽  
Hin Fan Chan

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.


2016 ◽  
Vol 8 (02) ◽  
pp. 123-125 ◽  
Author(s):  
Biswajit Dey ◽  
Bhawana Badhe ◽  
Krishna Kumar Govindarajan ◽  
Ranjith Arumbakkam Ramesh

ABSTRACTXp11.2 translocation renal cell carcinomas (TRCCs) are a group of neoplasms with distinct clinical, histopathological appearance, immunohistochemical, and cytogenetic profile. We report a case of Xp11.2 translocation TRCC in an 11-year-old male diagnosed based on immunohistochemistry and fluorescence in situ hybridization.


2020 ◽  
Author(s):  
Jie Dong ◽  
Weifeng Xu ◽  
Zhigang Ji ◽  
Boju Pan

Abstract Background. Xp11.2 translocation renal cell carcinoma, a rare malignancy, is more common in children than in adults. It manifests with an aggressive course in adults and relatively indolent in children. Prognostic studies for adult patients are scare for the rarity of the disease; and the prognostic value of preoperative plasma fibrinogen awaits further illumination.Methods. This retrospective single-center study enrolled 24 consecutive newly diagnosed Xp11.2 translocation RCC adult patients. Clinical presentations, baseline laboratory results and follow-up data were collected. Possible risk factors for progression free survival and overall survival were first scanned with chi-square tests and t-tests to compare patients who suffered from progression or death and who did not. Multivariate Cox regression was further utilized to identify independent risk factors.Results. Twenty-four adult patients (median age 32, range 16-73), with a male-to-female ratio of 1:1, was included from 2010.4 to 2020.3. After a mean follow-up of 35.7months, seven patients died. With univariate analysis, higher C-reactive protein-to-albumin ratio (p=0.028), higher baseline fibrinogen (p=0.006), and presence of distant metastasis (p=0.007) were associated with progression of disease; higher preoperative fibrinogen (p=0.014) and distant metastasis (p=0.020) were associated with death. With multivariate Cox regression, only baseline fibrinogen level (p=0.001) was identified as an independent risk factor for progression free survival; meanwhile, fibrinogen level (p=0.048) and distant metastasis (p=0.043) were identified as independent risk factors for survival.Conclusions. Preoperative plasma fibrinogen, a routinely tested parameter before surgery, is a promising tool for risk stratification in adult patients with Xp11.2 translocation renal cell carcinoma.JIE et al: Preoperative plasma fibrinogen predicts outcome in Xp11.2 translocation RCC


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