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Comparing Metastatic Clear Cell Renal Cell Carcinoma Model Established in Mouse Kidney and on Chicken Chorioallantoic Membrane
Published on: February 8, 2020
Xp11 translocation renal cell carcinoma.
1Departments of Pathology, Johns Hopkins Hospital, 401 North Broadway, Weinberg 2242, Baltimore, Maryland 21231, USA.
Pathology
|May 5, 2010
Summary
Xp11 translocation renal cell carcinoma (RCC) is a rare kidney cancer caused by gene fusions involving the TFE3 gene. This cancer, found in children and adults, has distinct features and requires long-term follow-up.
Area of Science:
- Oncology
- Genetics
- Pathology
Background:
- Xp11 translocation renal cell carcinoma (RCC) is defined by translocations at Xp11.2, leading to TFE3 gene fusions.
- This specific type of RCC is a significant cause of kidney cancer in children and can also affect adults, sometimes presenting aggressively.
Purpose of the Study:
- To review the characteristics, diagnosis, and clinical outcomes of Xp11 translocation RCC.
- To highlight the importance of TFE3 immunohistochemistry in diagnosing this rare neoplasm.
Main Methods:
- Review of histological and immunohistochemical features of Xp11 translocation RCC.
- Analysis of clinical presentation, risk factors, and patient outcomes.
Main Results:
- Histologically, Xp11 translocation RCC often shows clear cells, papillary architecture, and psammoma bodies.
- Immunohistochemistry is crucial, with TFE3 protein and cathepsin-K being sensitive and specific markers.
- Outcomes vary, with children having a better short-term prognosis than adults who often present with metastatic disease.
Conclusions:
- Xp11 translocation RCC is a distinct entity requiring specific diagnostic markers like TFE3.
- Long-term follow-up is essential due to the potential for late metastasis.
- A newly identified melanotic neoplasm may represent a TFE3-related cancer subset.
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