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01.12.2017 | Letter to the Editor | Ausgabe 1/2017 Open Access

Orphanet Journal of Rare Diseases 1/2017

Receptor of ghrelin is expressed in cutaneous neurofibromas of individuals with neurofibromatosis 1

Zeitschrift:
Orphanet Journal of Rare Diseases > Ausgabe 1/2017
Autoren:
Rafaela E. Rozza-de-Menezes, Nicolle C. Gaglionone, Raquel M. Andrade-Losso, Orlando H. K. Siqueira, Lilian M. Almeida, Kamila da S. Peruzini, Marco A. C. Guimarães-Filho, Carolina I. Brum, Mauro Geller, Karin S. Cunha
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Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1186/​s13023-017-0734-x) contains supplementary material, which is available to authorized users.

Abstract

Background

Multiple cutaneous neurofibromas are a hallmark of neurofibromatosis 1 (NF1). They begin to appear during puberty and increase in number and volume during pregnancy, suggesting a hormonal influence. Ghrelin is a hormone that acts via growth hormone secretagogue receptor (GHS-R), which is overexpressed in many neoplasms and is involved in tumorigenesis. We aimed to investigate GHS-R expression in NF1 cutaneous neurofibromas and its relationship with tumors volume, and patient’s age and gender.

Results

Sample comprised 108 cutaneous neurofibromas (55 large and 53 small tumors) from 55 NF1 individuals. GHS-R expression was investigated by immunohistochemistry in tissue micro and macroarrays and quantified using a digital computer-assisted method. All neurofibromas expressed GHS-R, with a percentage of positive cells ranging from 4.9% to 76.1%. Large neurofibromas expressed more GHS-R than the small ones. The percentage of GHS-R-positive cells and intensity of GHS-R expression were positively correlated with neurofibromas volume. GHS-R expression was more common in female gender.

Conclusions

GHS-R is expressed in cutaneous neurofibromas. Larger neurofibromas have a higher percentage of positive cells and higher GHS-R intensity. Based on our results we speculate that ghrelin may have an action on the tumorigenesis of cutaneous neurofibromas. Future studies are required to understand the role of ghrelin in the pathogenesis of NF1-associated cutaneous neurofibroma.
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