Hedgehog-Patched pathway aberrations in a malignant triton tumor case study

Bura, Miljenko; Musani, Vesna; Čretnik, Maja; Botica, Iva; Levanat, Sonja (2008) Hedgehog-Patched pathway aberrations in a malignant triton tumor case study. Oncology Reports, 20 (2). pp. 347-352. ISSN 1021-335X

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Abstract

Transition from malignant schwannoma to malignant triton tumor is analyzed in a case report on a patient with recurring cancers and suspected familial predisposition. It is hypothesized that rhabdomyoblastic differentiation, which distinguishes triton from schwannoma, might be attributable to Hedgehog-Patched pathway malfunctioning. Loss of one Patched gene allele was found in the tissue of advanced triton, but the retained allele had no exon or promoter mutations. Protein levels at early cancer stages indicated possible Patched response to the pathway activation in the first occurrence of triton tumor. Later, in the recurring triton, Patched expression was several times lower than in the control tissue, suggesting that haploinsufficiency was aided by silencing of the remaining allele, although its promoter was not hypermethylated. These findings may justify further investigation of the Hedgehog- Patched pathway role in triton malignancies, especially because of the recent research on the therapeutical potential of the pathway.

Item Type:

Article

Uncontrolled Keywords:

malignant triton tumor; parotid gland; Schwanomma; Hedgehog pathway

Subjects:

BIOMEDICINE AND HEALTHCARE > Basic Medical Sciences

Divisions:

Division of Molecular Medicine

Projects:

Project title	Project leader	Project code	Project type
Signal transduction in tumors: Hh-Gli interactions and therapeutic potential (Prijenos signala u tumorima: Hh-Gli put, interakcije i potencijalne terapije)-	Sonja Levanat	098-0982464-2461	MZOS
Molecular base of thyroid cancer-criteria for treatment differentiated carcinoma (Molekularna osnova raka štitnjače-kriteriji u terapiji diferenciranih karcinoma)-	Miljenko Bura	108-0000000-0051	MZOS

Depositing User:

Maja Sabol

Date Deposited:

27 Nov 2015 12:20

URI:

http://fulir.irb.hr/id/eprint/2317

DOI:

10.3892/or_00000013