잠시만 기다려 주세요. 로딩중입니다.

Characteristics of Renal Cell Carcinoma Harboring TPM3-ALK Fusion

Yonsei Medical Journal 2020년 61권 3호 p.262 ~ 266
우창곡 ( Woo Chang-Gok ) - Chungbuk National University Hospital Department of Pathology

 ( Yun Seok-Jung ) - Chungbuk National University Hospital Department of Urology
손승명 ( Son Seung-Myoung ) - Chungbuk National University Hospital Department of Pathology
 ( Lim Young-Hyun ) - Chungbuk National University College of Medicine Department of Pathology
이옥준 ( Lee Ok-Jun ) - Chungbuk National University College of Medicine Department of Pathology

Abstract


The World Health Organization 2016 edition assigned anaplastic lymphoma kinase (ALK) rearrangement-associated renal cell carcinoma (ALK-RCC) as an emerging renal tumor entity. Identifying ALK-RCC is important because ALK inhibitors have been shown to be effective in treatment. Here, we report the case of a 14-year-old young man with ALK-RCC. Computed tomography revealed a well-demarcated 5.3-cm enhancing mass at the upper pole of the left kidney. There was no further history or symptoms of the sickle-cell trait. The patient underwent left radical nephrectomy. Pathologically, the mass was diagnosed as an unclassified RCC. Targeted next-generation sequencing identified a TPM3-ALK fusion gene. The present report and literature review demonstrate that TPM3-ALK RCC may be associated with distinct clinicopathological features. Microscopically, the tumors showed diffuse growth and tubulocystic changes with inflammatory cell infiltration. Tumor cells were dis-cohesive and epithelioid with abundant eosinophilic cytoplasm and cytoplasmic vacuoles. If morphological features and TFE3 expression are present in adolescent and young patients, molecular tests for ALK translocation should be performed. This awareness is critically important, because ALK rearrangement confers sensitivity to ALK inhibitors.

키워드

Anaplastic lymphoma kinase; TPM3 protein; gene rearrangement; renal cell carcinoma; ALK inhibitors
원문 및 링크아웃 정보
  
등재저널 정보