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Clinicopathological features of Xp11.2 translocation renal cell carcinoma.
- Source :
-
Korean journal of urology [Korean J Urol] 2015 Mar; Vol. 56 (3), pp. 212-7. Date of Electronic Publication: 2015 Feb 26. - Publication Year :
- 2015
-
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.<br />Materials and Methods: 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.<br />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.<br />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.
- Subjects :
- Adolescent
Adult
Aged
Aged, 80 and over
Biomarkers
Carcinoma, Renal Cell diagnosis
Child
Female
Humans
Kidney Neoplasms diagnosis
Lymphatic Metastasis
Male
Middle Aged
Prognosis
Retrospective Studies
Translocation, Genetic
Young Adult
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors genetics
Carcinoma, Renal Cell genetics
Chromosomes, Human, X chemistry
Kidney Neoplasms genetics
Subjects
Details
- Language :
- English
- ISSN :
- 2005-6745
- Volume :
- 56
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Korean journal of urology
- Publication Type :
- Academic Journal
- Accession number :
- 25763125
- Full Text :
- https://doi.org/10.4111/kju.2015.56.3.212