scholarly journals Adult Nephroblastoma with Predominant Epithelial Component: A Differential Diagnostic Candidate of Papillary Renal Cell Carcinoma and Metanephric Adenoma—Report of Three Cases

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Shiho Watanabe ◽  
Hiroshi Naganuma ◽  
Michio Shimizu ◽  
Satoshi Ota ◽  
Shin-ichi Murata ◽  
...  

Although nephroblastoma is the commonest renal tumor of childhood, it is rare in adults. In cases of predominantly epithelial type occurring in adulthood, it might be difficult to distinguish it from papillary renal cell carcinoma and metanephric adenoma. Here, we report three cases of adult epithelial nephroblastoma in 24-, 76-, and 21-year-old females. Histologically, the tumors were composed of papillotubular architectures of small and uniform tumor cells with high nucleocytoplasmic ratio without blastemal element. Immunohistochemically, the tumor cells were positive for WT-1 and CD57 but negative for AMACR, which was helpful to exclude the possibility of papillary renal cell carcinoma. Metanephric adenoma is a benign tumor, which can be distinguished by the observation of the cellular atypism and growth pattern. However, nephroblastoma with predominant epithelial element mimics the malignant counterpart of metanephric adenoma, that is, “metanephric adenocarcinoma.”

2012 ◽  
Vol 15 (1) ◽  
pp. 65-70 ◽  
Author(s):  
Maria Laura Galluzzo ◽  
Maria T. Garcia de Davila ◽  
Gordan M. Vujanić

A coexistence of different renal tumors has rarely been reported. The most commonly described association is of Wilms tumor and renal cell carcinoma. Metanephric adenofibroma has also been associated with Wilms tumor or papillary renal cell carcinoma. Another reported association is metanephric adenoma and papillary renal cell carcinoma with sarcomatoid dedifferentiation. Herein we describe a complex renal tumor containing areas of metanephric adenofibroma, Wilms tumor, and undifferentiated renal cell carcinoma in a previously healthy 18-year-old boy. The tumor showed histologic and immunohistochemical features of these 3 different tumors, offering additional support to the view that these 3 tumors are related.


2016 ◽  
Vol 55 (4) ◽  
pp. 350-354 ◽  
Author(s):  
Theo J.M. Hulsebos ◽  
Susan Kenter ◽  
Frank Baas ◽  
Eline A. Nannenberg ◽  
Fonnet E. Bleeker ◽  
...  

2016 ◽  
Vol 34 (2_suppl) ◽  
pp. 506-506 ◽  
Author(s):  
Ilya Tsimafeyeu ◽  
Alexandra Naumova ◽  
Evgenia Stepanova ◽  
Alfia Khasanova ◽  
Ilya Varlamov ◽  
...  

506 Background: In our previous study we showed that fibroblast growth factor receptor 2 (FGFR2) mutations are rare across papillary types of renal cell carcinoma (pRCC). The aim of the present study is to test FGFR2 expression for association with survival outcome in the largest patient cohort to date. Methods: Formalin-fixed, paraffin-embedded specimens of removed primary tumors from 214 untreated metastatic pRCC patients were evaluated by immunohistochemistry with FGFR2 antibody (Santa Cruz Biotechnology). Expression was quantified by consensus of two independent observers using a four-value intensity score (0, 1+, 2+, and 3+) and the percentage (0-100%) of the extent of reactivity. Expression was scored according to the percentage of positive cells present among all tumor cells in the section. The cytoplasmic and nuclear expression score was obtained by multiplying the intensity and reactivity extension values (range, 0-300). FGFR2 expression was tested for associations with progression-free survival (PFS), overall survival (OS) and best objective response. Results: Expression of FGFR2 was observed in 23% (49/214) of primary pRCC, mostly in cytoplasm of tumor cells. 2 of 214 (1%) patients had nuclear FGFR2 expression. Intensity was 3+ in all cases. Expression of FGFR2 was significant lower in the normal tissue of kidney (1%, P=0.001). FGFR2 expression was strongly associated with a number of metastatic sites (2 and more metastatic sites vs. 0-1), type 2 of pRCC, lower nucleolar grade (P<0.001). FGFR2-positive patients had significantly shorter OS and PFS in first-line therapy (P<0.05; Table). On multivariate analysis, FGFR2 expression, MSKCC risk group, and type of pRCC were found to be independent predictors of survival. Conclusions: In this study, we described immunohistochemical expression of FGFR2 in a large series of pRCC specimens. FGFR2 expression was found to be prognostic factor for survival in patients with metastatic pRCC. Clinical trial information: rosoncoweb2011. [Table: see text]


2020 ◽  
Vol 17 (3) ◽  
Author(s):  
Gezahen Negusse Ayane ◽  
Khutsafalo Kadimo

Massive hemoperitoneum due to spontaneous rupture of renal cell carcinoma is a rare presentation during emergency laparotomy. A 60-year-old female patient presented with severe abdominal pain, nausea, vomiting and abdominal distension. A massive hemoperitoneum secondary to ruptured left kidney tumor was found during laparotomy. Histology confirmed a papillary renal cell carcinoma. Ruptured renal cell carcinoma can be a cause of bleeding into the retroperitoneum space. Emergency sonography and laparotomy are advocated for its management. Keywords: Renal cell carcinoma, Rupture, Complex mass, Nephrectomy, Papillary


2008 ◽  
Vol 2008 ◽  
pp. 1-4 ◽  
Author(s):  
F. Algaba

The renal adenomas can be confused by imaging diagnosis with malignant renal tumors, but there are also real biological dilemmas to determine their behavior. The consensus decisions are the following. (1) The adenoma of clear cells is not accepted, instead it is considered that all the clear-cell tumors are carcinomas, with greater or lesser aggressiveness. (2) Among the papillary neoplasms the WHO 2004 renal cell tumors classification are considered as papillary adenomas tumors with a maximum diameter of 5 mm and may represent a continuum biological process to papillary renal cell carcinoma. The papillary adenomas associated with End-kidney and/or acquired cystic disease may have a different pathogenesis. (3) To consider a tumor as an oncocytoma the size is not important, only the cytological features, microscopic, ultrastructural, and immunohistochemically can help, but some chromosomal observations introduce some questions about its relation with the chromophobe renal cell carcinoma. (4) Finally, the metanephric adenoma, a tumor with some morphological similarity with the nephroblastoma must be considered in the renal adenomas diagnosis.


2013 ◽  
Vol 90 (3) ◽  
pp. 369-372 ◽  
Author(s):  
Akinori Masuda ◽  
Takao Kamai ◽  
Tomoya Mizuno ◽  
Tsunehito Kambara ◽  
Hideyuki Abe ◽  
...  

2018 ◽  
Vol 81 (10) ◽  
pp. 878-883 ◽  
Author(s):  
Wei-Jen Chen ◽  
Chin-Chen Pan ◽  
Shu-Huei Shen ◽  
Hsiao-Jen Chung ◽  
Chih-Chieh Lin ◽  
...  

Author(s):  
Kylee A Rosette ◽  
Stephen M Lander ◽  
Calvin VanOpstall ◽  
Brendan David Looyenga

Papillary renal cell carcinoma (pRCC) represents the second most common kidney cancer and can be distinguished from other types based upon its unique histologic architecture and specific pattern of genomic alterations. Sporadic Type1 pRCC is almost universally driven by focal or chromosomal amplification of the receptor tyrosine kinase MET, though the specific mode of its activation is unclear. While the MET receptors found in human tumor specimens appear highly active, those found on the surface of in vitro cultured tumor cells are only weakly activated in the absence of exogenous hepatocyte growth factor (HGF) ligand. Furthermore, pRCC cells cultured in standard two-dimensional conditions with serum fail to respond functionally to MET knockdown or the selective MET inhibitor capmatinib despite clear evidence of kinase inhibition at the molecular level. To better model pRCC in vitro, we developed a three-dimensional (3D) coculture system in which renal tumor cells are layered on top of primary fibroblasts in a fashion that mimics the papillary architecture of human tumors. In this 3D spheroid model, the tumor cells survive and proliferate in the absence of serum due to trophic support of HGF-producing fibroblasts. Unlike tumor cells grown in monoculture, the proliferation of cocultured tumor cells is sensitive to capmatinib and parallels inhibition of MET kinase activity. These findings demonstrate the importance of stromal fibroblasts in pRCC and indicate that accurate in vitro representation of this disease requires the presence of both tumor and fibroblast cells in a structured coculture model.


Author(s):  
Niccolo Lenci ◽  
Pierconti Francesco ◽  
Eros Scarciglia ◽  
Vincenzo Fiorentino ◽  
Mattia Schino ◽  
...  

Abstract Metanephric adenoma (MA) is an uncommon benign renal tumor whose histomorphological aspect resembles that of Wilms’ tumor and papillary renal cell carcinoma. From a diagnostic and therapeutic perspective, recognition of this entity is important as it has a more favorable clinical outcome compared with Wilms’ tumor and papillary renal cell carcinoma. MA should not be treated with nephrectomy if the tumor size is small, opting for a conservative treatment. However, the preoperative diagnosis of this disease is extremely challenging. The present study describes a case of this rare disease, showing an ambiguous radiological imaging and that only after a percutaneous biopsy, was defined as a MA and treated with partial nephrectomy. Moreover, the histological diagnosis of this case was partially complicated by the equivocal immunohistochemical analysis showing negativity for BRAF VE1 staining. Only the mutational analysis demonstrated the presence of the BRAF V600K mutation (for the first time described in a case of metanephric adenoma), highlighting the necessity of sequencing in case of MA with negativity for BRAF VE1 clone.


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