Clinicopathological features of Xp11.2 translocation renal cell carcinoma
Korean Journal of Urology
;
: 212-217, 2015.
Artigo
em Inglês
| WPRIM
| ID: wpr-60932
ABSTRACT
PURPOSE:
Xp11.2 translocation renal cell carcinoma (RCC) is characterized by various translocations of the TFE3 transcription factor gene. These rare cancers occur predominantly in children and young adults. Here, we review the clinicopathological features of Xp11.2 translocation RCC. MATERIALS ANDMETHODS:
We identified 21 patients with Xp11.2 translocation RCC. We retrospectively analyzed patient characteristics, clinical manifestations, and specific pathological features to assess definitive diagnosis, surgical and systemic treatments, and clinical outcomes.RESULTS:
The mean age at diagnosis was 43.4+/-20.0 years (range, 8-80 years; 8 males and 13 females). Eleven patients were incidentally diagnosed, nine patients presented with local symptoms, and one patient presented with systemic symptoms. The mean tumor size was 6.2+/-3.8 cm (range, 1.9-14 cm). At the time of diagnosis, 11, 1, and 5 patients showed stage I, II, and III, respectively. Four patients showed distant metastasis. At analysis, 15 patients were disease-free after a median follow-up period of 30.0 months. Four patients received target therapy but not effectively.CONCLUSIONS:
Xp11 translocation RCC tends to develop in young patients with lymph node metastasis. Targeted therapy did not effectively treat our patients. Surgery is the only effective therapy for Xp11 translocation RCC, and further studies are needed to assess systemic therapy and long-term prognosis.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Prognóstico
/
Translocação Genética
/
Carcinoma de Células Renais
/
Biomarcadores
/
Estudos Retrospectivos
/
Cromossomos Humanos X
/
Fatores de Transcrição de Zíper de Leucina e Hélice-Alça-Hélix Básicos
/
Neoplasias Renais
/
Metástase Linfática
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Limite:
Adolescente
/
Adulto
/
Idoso
/
Aged80
/
Criança
/
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Korean Journal of Urology
Ano de publicação:
2015
Tipo de documento:
Artigo
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