A review of ALK-rearranged renal cell carcinomas with a focus on clinical and pathobiological aspects
| Autor: | Emiko Sugawara, Yoshiaki Yuba, Kengo Takeuchi, Kenji Yorita, Hironori Kusano, Naoto Kuroda |
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| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty Focus (geometry) medicine.drug_class Cell TFE3 urologic and male genital diseases Pathology and Forensic Medicine Renal medullary carcinoma 03 medical and health sciences 0302 clinical medicine Renal cell carcinoma hemic and lymphatic diseases medicine Renal medulla Anaplastic lymphoma kinase Humans Anaplastic Lymphoma Kinase Carcinoma Renal Cell In Situ Hybridization Fluorescence business.industry General Medicine medicine.disease female genital diseases and pregnancy complications Kidney Neoplasms ALK inhibitor 030104 developmental biology medicine.anatomical_structure 030220 oncology & carcinogenesis business |
| Zdroj: | Polish journal of pathology : official journal of the Polish Society of Pathologists. 69(2) |
| ISSN: | 1233-9687 |
| Popis: | ALK-rearranged renal cell carcinoma (ALK-RCC) has been recently proposed and incorporated into the recent World Health Organisation Classification of renal tumours as a provisional entity. In this article, we review ALK-RCC with a focus on clinical and pathobiological aspects. Seventeen cases have been described to date. ALK-RCC accounts for less than 1% of all renal tumours. The age of patients ranges from 6 to 61 years with a mean age of 29.6 years. Grossly, the tumour forms were ill-demarcated or well demarcated solid mass in the renal medulla. Histologically, RCC with VCL-ALK translocation resembles renal medullary carcinoma and mucinous cribriform pattern, signet-ring cell pattern and solid rhabdoid pattern are often observed in RCC with non-VCL-ALK fusion. Immunohistochemically, ALK protein diffusely expresses and TFE3 is often expressed. ALK gene can fuse to VCL, TPM3, EML4, HOOK1 or STRN gene. A break-apart fluorescence in situ hybridisation study is clinically available for the practice of definite diagnosis. ALK inhibitor therapy will provide great benefit for patients with advanced stage of ALK-RCC in the near future. |
| Databáze: | OpenAIRE |
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