How to treat neurofibroma correctly?
summary
Neurofibroma is an autosomal genetic disease. There are many causes. There are different views on the source of it. So it has many different names. One of them is neurilemmoma or schwannoma. Another type is: neurofibroma or perineural fibroblastoma, which refers to the connective tissue of tumor cells deepened from the neuroendoderm. There is no effective treatment, patients can only suffer psychological torture at the same time, waiting for surgery again and again, until death. This is actually "boiling frogs in warm water.". Every case of such a patient, the doctor will be very helpless to shake his head, and then told: go back and wait when it doesn't work again.
How to treat neurofibroma correctly?
Neurofibromatosis type 2 is an autosomal hereditary disease. The incidence rate of births is tens of thousands. The cause of the disease is the inactivation of NF2 gene on autosomal 22q, which encodes the NF2 gene product "Merlin". The vast majority of young people with NF2 have progressive hearing loss due to bilateral vestibular schwannomas.
In contrast to neurofibroma protein, Merlin can exert its effect not only in the cortex but also in the nucleus, directly affecting multiple signal transduction pathways related to contact inhibition and tumor inhibition. Neurofibromatosis type 2 can have a variety of tumors involving the central nervous system and peripheral nervous system, namely schwannoma, meningioma and ependymoma.
A phase 2 clinical study of NF2 patients in adults and children with progressive vestibular schwannoma suggests that lapatinib can prevent tumor volume increase (24%) and hearing loss (31%) to some extent. However, the improvement of hearing function is slight and not lasting. However, everolimus did not produce any beneficial effect.
matters needing attention
Based on the recent biological research on Merlin deficient tumors, the preliminary clinical research of many molecular targeted agents has started again, including schwannoma and meningioma gene engineering mice. Currently, EGFR / erbB2 (lapatinib) has been transformed from preclinical study to clinical trial
