Premium
Transcriptional activation of Hedgehog pathway components in aggressive haemangioma
Author(s) -
WendlingKeim Danielle S.,
Wanie Lynn,
Schweinitz Dietrich,
Grantzow Rainer,
Kappler Roland
Publication year - 2017
Publication title -
experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.108
H-Index - 96
eISSN - 1600-0625
pISSN - 0906-6705
DOI - 10.1111/exd.13346
Subject(s) - sonic hedgehog , hemangioma , hedgehog , hedgehog signaling pathway , downregulation and upregulation , cancer research , fibroblast growth factor , medicine , biology , pathology , gene , receptor , genetics
Infantile hemangioma is a vascular neoplasm and is one of the most common tumors diagnosed in young children. Although most hemangiomas are harmless and involute spontaneously, some show severe progression, leading to serious complications, such as high‐output cardiac failure, ulcerations, compression of the trachea or deprivation amblyopia, depending on their size and localization. However, the pathogenesis and cause of hemangioma are largely unknown to date. The goal of this study was to identify markers that could predict hemangiomas with aggressive growth and severe progression that would benefit from early intervention. By using a PCR ‐based screening approach, we first confirmed that previously known markers of hemangioma, namely FGF 2 and GLUT 1, are highly expressed in hemangioma. Nevertheless, these genes did not show any differential expression between severely progressing tumors and mild tumors. However, transcriptional upregulation of several Hedgehog signalling components, comprising the ligand Sonic Hedgehog ( SHH ), the transcription factor GLI 2 and its target gene FOXA 2 were detected in extremely aggressive hemangioma specimens during the proliferation phase. Notably, GLI 2 was even overexpressed in involuting hemangiomas if they showed an aggressive growth pattern. In conclusion, our data suggest that overexpression of the Hedgehog components SHH , GLI 2 and FOXA 2 might be used as markers of an aggressive hemangioma that would benefit from too early intervention, while FGF 2 and GLUT 1 are more general markers of hemangiomas.