title: Clinicopathologic Features of Translocation Renal Cell Carcinoma.
abstract: Translocation renal cell carcinomas (TFE3 RCC) are associated with variable genetic rearrangements of the TFE3 gene on chromosome Xp11.2. Translocation tumors represent 1% to 5% of all cases of RCC, with the greatest frequency among children and young adults. We sought to characterize the clinicopathologic features of translocation RCC at a Middle Eastern institution.
  
  
  
  The clinical and pathologic data from a single institution were retrospectively reviewed. A total of 14 patients with translocation RCC had been diagnosed from 2005 to 2014. The outcome measures included patient characteristics, clinical manifestations, pathologic features, treatment outcomes, cancer-specific survival, and progression-free survival.
  
  
  
  The mean age at diagnosis was 35 years. Of the 14 patients, 5 were female. Translocation RCC was an incidental diagnosis for all but 2 of the 14 patients. The mean tumor size was 9 cm; 1 patient had bilateral tumors, and 3 presented with positive lymph nodes. Three patients underwent partial nephrectomy. Three patients had developed metastasis at 4 months, 5 months, and 3 years after diagnosis. One patent had died 4 months after surgery and one had died 21 months after surgery (both of metastases). The disease-free survival rate was 71% at a mean follow-up of 31 months.
  
  
  
  Translocation RCC is a rare and potentially aggressive subtype of kidney cancer. An overall survival of > 3 years has been noted, unless metastasis is present at diagnosis.
authors.affiliation: Division of Urology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Riyad T
authors.initials: RT
authors.lastname: Ellati
authors.affiliation: Division of Urology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Abukhiran
authors.affiliation: Division of Urology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Kholoud
authors.initials: K
authors.lastname: Alqasem
authors.affiliation: Division of Urology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Judy
authors.initials: J
authors.lastname: Jasser
authors.affiliation: Department of Pathology, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Juakub
authors.initials: J
authors.lastname: Khzouz
authors.affiliation: Division of Urology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Tamer
authors.initials: T
authors.lastname: Bisharat
authors.affiliation: Division of Cardiology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Al-Saidi
authors.affiliation: Division of Urology, Department of General Surgery, King Hussein Cancer Center, Amman, Jordan. Electronic address: AA.11119@khcc.jo.
authors.forename: Ali
authors.initials: A
authors.lastname: Al-Daghmin
mesh_terms: D000293:Adolescent; D000328:Adult; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics*; D002292:Carcinoma, Renal Cell / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D041321:Chromosomes, Human, X / Q000235:genetics; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D033162:Incidental Findings; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D008877:Middle East; D009362:Neoplasm Metastasis; D012189:Retrospective Studies; D016019:Survival Analysis; D014178:Translocation, Genetic; D047368:Tumor Burden; D055815:Young Adult
chemical_list: D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; C069203:TFE3 protein, human
keywords: RCC; TFE3; Translocation RCC; Xp11.2 translocation