New results in endocrine neoplasia research
January 19, 2016 Source: Bio Valley
Window._bd_share_config={ "common":{ "bdSnsKey":{ },"bdText":"","bdMini":"2","bdMiniList":false,"bdPic":"","bdStyle":" 0","bdSize":"16"},"share":{ }};with(document)0[(getElementsByTagName('head')[0]||body).appendChild(createElement('script')) .src='http://bdimg.share.baidu.com/static/api/js/share.js?v=89860593.js?cdnversion='+~(-new Date()/36e5)];Leading this research is Guang Ning, an academician of the New Engineering Institute of Shanghai Jiao Tong University School of Medicine. Academician Ningguang has long been committed to the clinical and scientific research of endocrine and metabolic diseases, and has achieved innovative results in the field of diagnosis and treatment of endocrine tumors and diabetes. More than 200 papers have been published in SCI journals such as Science, JAMA, Nat Genet, Nat Cell Biol, and J Am Coll Cardiol.
Pheochromocytoma (PCC) is derived from adrenal medullary chromaffin cells and secretes catecholamines: adrenaline, norepinephrine and dopamine. Very few tumors are silent; paraganglioma (PGL) originates from adrenal hoarding. Chromium cells, located in the thoracic, abdominal and pelvic paraspinal sympathetic chain; the two are collectively referred to as PPGL.
PPGL accounts for about 7% of sympathetic nervous system tumors, with an annual incidence of 0.3/1 000 000. More than 20 to 50 years old, more women than men. Most PPGLs are benign, and about 12% of PPGL is malignant. There are currently no good tissue or molecular markers to identify benign and malignant PPGLs. In the previous cross-sectional study, Ningguang's group observed that ERBB-2 overexpression was associated with malignant PPGLs. The new study aims to assess the predictive value of ERBB-2 overexpression for PPGLs metastasis in large populations.
The researchers studied a total of 262 patients with PPGLs diagnosed in their institution in 2002-2012. They used immunohistochemistry (IHC) to analyze the expression of ERBB-2 protein in primary PPGL tumors and to detect ERBB-2 amplification by fluorescence in situ hybridization (FISH). The ERBB-2 exon 20 mutation was detected by direct Sanger sequencing. The occurrence of malignant PPGLs was recorded during follow-up. Kaplan-Meier analysis and Cox proportional hazard model were used to assess the association between ERBB-2 overexpression and PPGLs metastasis.
The results showed that 26 (9.9%) patients had ERBB-2 overexpression in primary PPGL tumors, which was significantly associated with ERBB-2 amplification. No ERBB-2 mutation was found. After an average of 4.5 years of follow-up, a total of 23 malignant PPGLs were recorded, including 8 (30.8%) patients in the ERBB-2 overexpression group and 15 (6.4%) patients in the ERBB-2 negative group. The incidence of metastasis between the ERBB-2 overexpression group and the ERBB-2 negative group was 5.3 cases/100 person-years and 1.4 cases/100 persons, respectively. Kaplan-Meier analysis showed that ERBB-2 overexpression was associated with a shortened metastasis-free survival. After adjusting the size and location of the primary tumor, Cox regression revealed that ERBB-2 overexpression was independently associated with the risk of malignant PPGLs.
New research confirms that patients with overexpression of ERBB-2 in tumors have a higher risk of developing malignant PPGLs.
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