PRCC-TFE3 Renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma

Ali Mirza Onder, Uygar Teomete, Pedram Argani, Stuart Toledano, Gaston E Zilleruelo, Maria Rodriguez

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

The genetics of renal tumors in children is widely recognized. However, most of the studies published to date emphasize the association between Wilms tumor and the WT-1 gene. Recently, a unique translocation between the X chromosome and chromosome 1 or t(X;1) has been described in several reports of renal cell carcinomas (RCCs) diagnosed in children and adolescents that results in PRCC-TFE3 gene fusion. We report here a 9-year old African-American boy with a history of a right congenital mesoblastic nephroma treated with nephrectomy and followed by annual checkups. After 9 years, he was diagnosed with a mass at the hilum of the left kidney during the work-up of new-onset hypertension. A limited biopsy revealed densely hyalinized connective tissue that was initially interpreted to be a hyalinized contralateral mesoblastic nephroma. The child received chemotherapy, but the mass continued to grow. He underwent a left nephrectomy, and the pathology was diagnostic for a clear cell RCC. Chromosomal analysis disclosed a t(X;1)(p11.2;q21) translocation, which is known to result in a PRCC-TFE3 gene fusion. The tumor showed nuclear labeling for TFE3 protein by immunohistochemistry, supporting the above diagnosis. He has been on hemodialysis, is tumor free, and has not been receiving chemotherapy for 24 months. This is the first report of a RCC as a second malignant neoplasm in a child treated for a congenital mesoblastic nephroma.

Original languageEnglish
Pages (from-to)1471-1475
Number of pages5
JournalPediatric Nephrology
Volume21
Issue number10
DOIs
StatePublished - Oct 1 2006

Fingerprint

Mesoblastic Nephroma
Renal Cell Carcinoma
Gene Fusion
Nephrectomy
Kidney
Drug Therapy
Neoplasms
Second Primary Neoplasms
Wilms Tumor
Chromosomes, Human, Pair 1
X Chromosome
African Americans
Connective Tissue
Renal Dialysis
Immunohistochemistry
Pathology
Hypertension
Biopsy
Genes
Proteins

Keywords

  • Carcinoma
  • Chromosome translocation
  • Kidney
  • Mesoblastic nephroma
  • Pediatric
  • PRCC-TFE3 gene fusion

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

Cite this

Onder, A. M., Teomete, U., Argani, P., Toledano, S., Zilleruelo, G. E., & Rodriguez, M. (2006). PRCC-TFE3 Renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma. Pediatric Nephrology, 21(10), 1471-1475. https://doi.org/10.1007/s00467-006-0141-3

PRCC-TFE3 Renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma. / Onder, Ali Mirza; Teomete, Uygar; Argani, Pedram; Toledano, Stuart; Zilleruelo, Gaston E; Rodriguez, Maria.

In: Pediatric Nephrology, Vol. 21, No. 10, 01.10.2006, p. 1471-1475.

Research output: Contribution to journalArticle

Onder, AM, Teomete, U, Argani, P, Toledano, S, Zilleruelo, GE & Rodriguez, M 2006, 'PRCC-TFE3 Renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma', Pediatric Nephrology, vol. 21, no. 10, pp. 1471-1475. https://doi.org/10.1007/s00467-006-0141-3
Onder AM, Teomete U, Argani P, Toledano S, Zilleruelo GE, Rodriguez M. PRCC-TFE3 Renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma. Pediatric Nephrology. 2006 Oct 1;21(10):1471-1475. https://doi.org/10.1007/s00467-006-0141-3
Onder, Ali Mirza ; Teomete, Uygar ; Argani, Pedram ; Toledano, Stuart ; Zilleruelo, Gaston E ; Rodriguez, Maria. / PRCC-TFE3 Renal cell carcinoma in a boy with a history of contralateral mesoblastic nephroma. In: Pediatric Nephrology. 2006 ; Vol. 21, No. 10. pp. 1471-1475.
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