Characteristics of Renal Cell Carcinoma Harboring TPM3-ALK Fusion
Yonsei Medical Journal
;
: 262-266, 2020.
Artículo
en Inglés
| WPRIM
| ID: wpr-811468
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.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Fosfotransferasas
/
Vacuolas
/
Organización Mundial de la Salud
/
Carcinoma de Células Renales
/
Reordenamiento Génico
/
Citoplasma
/
Eosinófilos
/
Riñón
/
Linfoma
/
Nefrectomía
Tipo de estudio:
Estudio pronóstico
Límite:
Adolescente
/
Humanos
Idioma:
Inglés
Revista:
Yonsei Medical Journal
Año:
2020
Tipo del documento:
Artículo
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