Please use this identifier to cite or link to this item: doi:10.22028/D291-27772
Title: The Importance of the Hedgehog Signaling Pathway in Tumorigenesis of Spinal and Cranial Chordoma
Author(s): Akhavan-Sigari, Reza
Schulz-Schaeffer, Walter
Angelika Harcej, Amanda
Rohde, Veit
Language: English
Title: Journal of Clinical Medicine
Volume: 8
Issue: 2
Publisher/Platform: MDPI
Year of Publication: 2019
Free key words: hedgehog signaling cascade
spinal and cranial chordomas
DDC notations: 610 Medicine and health
Publikation type: Journal Article
Abstract: Chordomas is rare malignant bone tumors thought to arise from remnants of embryonic notochord along the spine, frequently at the skull base and sacrum. Although chordoma is slow growing tumors, while are extremely recurrent, and aggressive, as well as the rate of prognosis remains poorly. Radical surgery and high-dose radiation are the most used treatments. Currently, there is no effective chemotherapeutic standard for chordomas. The Hedgehog (HH) pathway adjusts various processes included in expansion and differentiation of tissues and organs throughout the fetus’s life, furthermore cell growth and differentiation in the adult organism, of the cell in an adult organism, in which acute anesthesia is involved in multiple cancers. To study the role of signaling the hedgehog in the base of the skull and sacrum chordomas, the expression of SHH and GLI-1 levels were detected immuno histochemically, Additionally, PTCH-1 and GLI-1 expressions were distinguished by in- Situ- hybridization. Based on the findings presented herein, it is likely that the HH signal cascade was revealed even in cranial, where consecoently spinal chordoma and their recurrences play an important role. Our staining exhibited a canonical, ligand- dependent and autocrine Hedgehog signaling in skull base and sacrum chordomas including relapse. Due to the high levels of SHH and GLI-1 expression in all investigated chordoma samples, the study suggests a possible autocrine ligand-dependent activation of the canonical HH signaling cascade. A paracrine or non-canonical pathway cannot be excluded. Our results suggest that Hedgehog-inhibitors, like SHH-, GLI- and SMO- inhibitors, might serve as a potential and effective target for the treatment of chordomas.
DOI of the first publication: 10.3390/jcm8020248
Link to this record: urn:nbn:de:bsz:291--ds-277725
ISSN: 2077-0383
Date of registration: 5-Nov-2020
Faculty: M - Medizinische Fakultät
Department: M - Medizinische und Klinische Psychologie
Professorship: M - Prof. Dr. Walter Schulz-Schaeffer
Collections:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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