ALK-rearranged renal cell carcinomas in children

Mariana Morais Cajaiba*, Lawrence J Jennings, Stephen M. Rohan, Antonio R. Perez-Atayde, Adrian Marino-Enriquez, Jonathan A. Fletcher, James I. Geller, Katrin Carlson, Julia A. Bridge, Elizabeth J Perlman

*Corresponding author for this work

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

Knowledge of the clinicopathological and molecular spectrum of pediatric renal cell carcinomas (RCC) remains limited, and approximately 16%-24% of these neoplasms cannot be classified into specific subtypes. In this review of 168 pediatric RCC prospectively registered on Children's Oncology Group AREN03B2 protocol, six RCC (3.5%) that demonstrated a unique epithelioid morphology and a peculiar immunophenotypic profile that includes expression of ALK, TFE3, and retention of INI1 was identified. Further investigation revealed ALK rearrangements in all cases, manifested molecularly by fusion transcripts of either VCL-ALK (3 patients all with sickle cell trait which had been previously reported) or TPM3-ALK (3 patients, none with sickle cell trait). Based on the shared unique morphologic, immunophenotypic, and genetic features, it was proposed that these neoplasms belonged to a distinct subgroup of RCC frequently occurring in pediatric patients, which they have termed as ALK-rearranged RCC. Importantly, additional therapeutic options may be available for these patients.

Original languageEnglish (US)
Pages (from-to)442-451
Number of pages10
JournalGenes Chromosomes and Cancer
Volume55
Issue number5
DOIs
StatePublished - May 1 2016

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Renal Cell Carcinoma
Sickle Cell Trait
Pediatrics
Neoplasms
Therapeutics

ASJC Scopus subject areas

  • Genetics
  • Cancer Research

Cite this

Cajaiba, M. M., Jennings, L. J., Rohan, S. M., Perez-Atayde, A. R., Marino-Enriquez, A., Fletcher, J. A., ... Perlman, E. J. (2016). ALK-rearranged renal cell carcinomas in children. Genes Chromosomes and Cancer, 55(5), 442-451. https://doi.org/10.1002/gcc.22346
Cajaiba, Mariana Morais ; Jennings, Lawrence J ; Rohan, Stephen M. ; Perez-Atayde, Antonio R. ; Marino-Enriquez, Adrian ; Fletcher, Jonathan A. ; Geller, James I. ; Carlson, Katrin ; Bridge, Julia A. ; Perlman, Elizabeth J. / ALK-rearranged renal cell carcinomas in children. In: Genes Chromosomes and Cancer. 2016 ; Vol. 55, No. 5. pp. 442-451.
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Cajaiba, MM, Jennings, LJ, Rohan, SM, Perez-Atayde, AR, Marino-Enriquez, A, Fletcher, JA, Geller, JI, Carlson, K, Bridge, JA & Perlman, EJ 2016, 'ALK-rearranged renal cell carcinomas in children', Genes Chromosomes and Cancer, vol. 55, no. 5, pp. 442-451. https://doi.org/10.1002/gcc.22346

ALK-rearranged renal cell carcinomas in children. / Cajaiba, Mariana Morais; Jennings, Lawrence J; Rohan, Stephen M.; Perez-Atayde, Antonio R.; Marino-Enriquez, Adrian; Fletcher, Jonathan A.; Geller, James I.; Carlson, Katrin; Bridge, Julia A.; Perlman, Elizabeth J.

In: Genes Chromosomes and Cancer, Vol. 55, No. 5, 01.05.2016, p. 442-451.

Research output: Contribution to journalArticle

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Cajaiba MM, Jennings LJ, Rohan SM, Perez-Atayde AR, Marino-Enriquez A, Fletcher JA et al. ALK-rearranged renal cell carcinomas in children. Genes Chromosomes and Cancer. 2016 May 1;55(5):442-451. https://doi.org/10.1002/gcc.22346