Geller Mauro, Mezitis Spyros G E, Nunes Fabio Pereira, Ribeiro Marcia G, Araújo Alexandra Prufer de Q C, Bronstein Marcello D, Siqueira-Batista Rodrigo, Gomes Andréia Patrícia, Oliveira Lisa, Cunha Karin Soares Gonçalves
Teresópolis Medical School (UNIFESO)-Teresópolis, Rio de Janeiro, Brazil.
Clin Med Pathol. 2008;1:93-7. doi: 10.4137/cpath.s1002. Epub 2008 Sep 15.
Neurofibromatosis type 1 (NF1) or von Recklinghausen disease is a genetic disorder affecting the growth of cells in nervous system. One of the most remarkable characteristics of this disease is the development of benign tumors of the nervous system (neurofibromas).The purpose of this study was to test tissue samples taken from neurofibromas and plexiform neurofibromas of NF1 patients for the presence of estrogen and progesterone receptors. We used previously collected samples from patients registered in the database of the Centro Nacional de Neurofibromatose (CNNF-Brazil). Samples from twenty-five patients in the database presenting plexiform neurofibromas (N1 group) and 25 samples from the same database from patients presenting neurofibromas (N2 group) were tested.We observed positive staining for progesterone receptors in 13 of the neurofibroma samples and 19 of the plexiform neurofibroma samples. Among the neurofibroma samples, we observed one sample with positive estrogen receptor staining, but none of the plexiform neurofibroma samples showed positive staining. We suggest further studies to investigate in greater depth possible hormonal influences on the development and growth of neurofibromas and plexiform neurofibromas in NF1.
1型神经纤维瘤病(NF1)或冯雷克林霍增氏病是一种影响神经系统细胞生长的遗传性疾病。这种疾病最显著的特征之一是神经系统良性肿瘤(神经纤维瘤)的形成。本研究的目的是检测取自NF1患者神经纤维瘤和丛状神经纤维瘤的组织样本中雌激素和孕激素受体的存在情况。我们使用了先前从巴西国家神经纤维瘤病中心(CNNF - 巴西)数据库中登记的患者收集的样本。对数据库中25例患有丛状神经纤维瘤的患者样本(N1组)和来自同一数据库的25例患有神经纤维瘤的患者样本(N2组)进行了检测。我们观察到13例神经纤维瘤样本和19例丛状神经纤维瘤样本中孕激素受体呈阳性染色。在神经纤维瘤样本中,我们观察到1例雌激素受体染色呈阳性,但丛状神经纤维瘤样本均未显示阳性染色。我们建议进一步开展研究,以更深入地探究激素对NF1患者神经纤维瘤和丛状神经纤维瘤的发生和生长可能产生的影响。