Artikel
The putative oncogene CPI-17 is up-regulated in schwannoma
Suche in Medline nach
Autoren
Veröffentlicht: | 14. September 2016 |
---|
Gliederung
Text
Question: Protein kinase C potentiated inhibitor (CPI-17) was previously described to act as an oncoprotein in the merlin pathway. The myosin phosphatase and its substrate merlin are part of a tumour suppressor cascade that can be blocked in two ways, by mutation of the NF2 gene encoding the tumour suppressor protein merlin and by up-regulation of the specific myosin phosphatase inhibitor CPI-17. Inactivation of the merlin tumour suppressor pathway is thought to be a major principle of transformation by CPI-17, the relevance of this oncogenic pathway in the human peripheral nervous system (PNS) is not known.
Methods: CPI-17 expression was screened immunohistochemically in 28 samples of peripheral nerves with non-tumorous pathology and in 160 peripheral nervous system tumours (PNST), including perineurioma, ganglioneuroma, Schwannoma of different aetiology, neurofibromatosis 1-associated neurofibroma and malignant peripheral nerve sheath tumour (MPNST).
Results: CPI-17 was demonstrated in 39/43 schwannomas, whereas all other PNST were CPI-17-negative, except for the perineurioma and two MPNST with mesenchymal differentiation. NF2-associated schwannomas showed lower levels of CPI-17 than sporadic schwannomas. High levels of CPI-17 were associated with increased proliferation.
Conclusion: CPI-17 was identified as a relevant oncoprotein in schwannoma and as a promising diagnostic marker differentiating between schwannoma and other PNST or non-tumorous peripheral nerve lesions.