A review of ALK-rearranged renal cell carcinomas with a focus on clinical and pathobiological aspects.
Pol J Pathol
; 69(2): 109-113, 2018.
Article
em En
| MEDLINE
| ID: mdl-30351856
ABSTRACT
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.
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Base de dados:
MEDLINE
Assunto principal:
Carcinoma de Células Renais
/
Quinase do Linfoma Anaplásico
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Neoplasias Renais
Limite:
Humans
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article