scholarly journals Does the Fuhrman or World Health Organization/International Society of Urological Pathology Grading System Apply to the Xp11.2 Translocation Renal Cell Carcinoma?

2018 ◽  
Vol 188 (4) ◽  
pp. 929-936 ◽  
Author(s):  
Ning Liu ◽  
Weidong Gan ◽  
Feng Qu ◽  
Zhen Wang ◽  
Wenyuan Zhuang ◽  
...  
Uro ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 2-13
Author(s):  
Maj Rabjerg ◽  
Oke Gerke ◽  
Birte Engvad ◽  
Niels Marcussen

This study was undertaken to compare Fuhrman grading with World Health Organization/International Society of Urological Pathology (WHO/ISUP) grading and stereologically measured nuclear area in patients with Clear Cell Renal Cell Carcinoma (ccRCC) or Papillary Renal Cell Carcinoma (PRCC) and to evaluate the independent predictive value of Fuhrman, WHO/ISUP and stereologically measured nuclear area combined with necrosis in a series of patients with ccRCC in relation to cancer-specific survival. In all, 124 cases of ccRCC and PRCC were included. All slides were blindly scored by two trained pathologists according to the Fuhrman and WHO/ISUP grading systems. Nuclear measurements were performed on digitally scanned slides in Visiopharm® and correlated to survival. Analysis of ccRCC and PRCC cases showed that application of WHO/ISUP grading resulted in a significant downgrading of cases from G2 to G1, when comparing with Fuhrman grading. Neither of these patients experienced progression. Cancer specific survival estimates in 101 ccRCC patients showed that WHO/ISUP grading was slightly superior in predicting cancer-specific survival. Novel models included WHO/ISUP grading and mean nuclear area (MNA) each of which combined with necrosis. Both demonstrated an increased ability to predict cancer-specific survival. The study demonstrates that WHO/ISUP grading provides superior prognostic information compared to Fuhrman grading and stereologically measured nuclear area. Necrosis in combination with either WHO/ISUP grading or MNA adds additional prognostic information.


2017 ◽  
Vol 71 (6) ◽  
pp. 918-925 ◽  
Author(s):  
Julien Dagher ◽  
Brett Delahunt ◽  
Nathalie Rioux-Leclercq ◽  
Lars Egevad ◽  
John R Srigley ◽  
...  

2016 ◽  
Vol 140 (10) ◽  
pp. 1026-1037 ◽  
Author(s):  
Aaron M. Udager ◽  
Rohit Mehra

Molecular and morphologic interrogation has driven a much-needed reexamination of renal cell carcinoma (RCC). Indeed, the recently released 2016 World Health Organization classification now recognizes 12 distinct RCC subtypes, as well as several other emerging/provisional RCC entities. From a clinical perspective, accurate RCC classification may have important implications for patients and their families, including prognostic risk stratification, targeted therapeutics selection, and identification for genetic testing. In this review, we provide a conceptual framework for approaching RCC diagnosis and classification by categorizing RCCs as tumors with clear cytoplasm, papillary architecture, and eosinophilic (oncocytic) cytoplasm. The currently recognized 2016 World Health Organization classification for RCC subtypes is briefly discussed, including new diagnostic entities (clear cell papillary RCC, hereditary leiomyomatosis and RCC-associated RCC, succinate dehydrogenase–deficient RCC, tubulocystic RCC, and acquired cystic disease–associated RCC) and areas of evolving RCC classification, such as transcription elongation factor B subunit 1 (TCEB1)–mutated RCC/RCC with angioleiomyoma-like stroma/RCC with leiomyomatous stroma, RCC associated with anaplastic lymphoma receptor tyrosine kinase (ALK) gene rearrangement, thyroidlike follicular RCC, and RCC in neuroblastoma survivors. For each RCC subtype, relevant clinical, molecular, gross, and microscopic findings are reviewed, and ancillary studies helpful for its differential diagnosis are presented, providing a practical approach to modern RCC classification.


Rare Tumors ◽  
2009 ◽  
Vol 1 (2) ◽  
pp. 164-166
Author(s):  
Jigarkumar Parikh ◽  
Teresa Coleman ◽  
Nidia Messias ◽  
James Brown

Xp11.2 translocation renal cell carcinomas (TRCCs) are a rare family of tumors newly recognized by the World Health Organization (WHO) in 2004. These tumors result in the fusion of partner genes to the TFE3 gene located on Xp11.2. They are most common in the pediatric population, but have been recently implicated in adult renal cell carcinoma (RCC) presenting at an early age. TFE3-mediated direct transcriptional upregulation of the Met tyrosine kinase receptor triggers dramatic activation of downstream signaling pathways including the protein kinase B (Akt)/phosphatidylinositol-3 kinase (PI3K) and mammalian target of rapamycin (mTOR) pathways. Temsirolimus is an inhibitor of mammalian target of rapamycin (mTOR) kinase, a component of intracellular signaling pathways involved in the growth and proliferation of malignant cells. Here we present a case of a 22-year old female who has been treated with temsirolimus for her Xp11.2/ TFE3 gene fusion RCC.


2018 ◽  
Vol 143 (5) ◽  
pp. 643-647 ◽  
Author(s):  
Tsung-Heng Tsai ◽  
Wen-Ying Lee

Succinate dehydrogenase (SDH)–deficient renal cell carcinoma is a recently recognized distinct subtype of renal cell carcinoma in the 2016 World Health Organization classification. It is associated with SDH gene germline mutations, which also cause paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma. The tumor most commonly presents in young adulthood. The tumors are arranged in solid nests or in tubules and frequently show cystic change. The tumors are composed of cuboidal to oval cells with round nuclei, dispersed chromatin, and inconspicuous nucleoli. The cytoplasm is eosinophilic or flocculent but not truly oncocytic. The most distinctive histologic feature is the presence of cytoplasmic vacuoles or inclusions. Loss of SDH subunit B immunostaining is needed for a definite diagnosis. The prognosis is good for low-grade tumors but worse for tumors with high-grade nuclei, sarcomatoid change, or coagulative necrosis. Long-term follow-up is indicated.


2019 ◽  
Vol 87 (3) ◽  
pp. 119-124
Author(s):  
Fabio Salvatori ◽  
Marco Macchini ◽  
Marco Misericordia ◽  
Enrico Paci ◽  
Andrea Giovagnoni ◽  
...  

Tubulocystic renal cell carcinoma is a rare subtype of renal tumor according to the 2016 World Health Organization, and less than 100 cases have been documented up to date in literature. The imaging features are not well known and to the best of our knowledge, there is not a radiology description of recurrence from tubulocystic renal cell carcinoma in the literature. We describe the case of a 70-year-old man with unusual cystic lesions in the left hypochondrium 11 years after a nephrectomy for tubulocystic renal cell carcinoma on the same side, and we report a review of the clinical characteristics of metastatic tubulocystic renal cell carcinoma.


Sign in / Sign up

Export Citation Format

Share Document