Malignant Peripheral Nerve Sheath Tumors State of the Science: Leveraging Clinical and Biological Insights into Effective Therapies

Table 1

Summary of progress in preclinical, clinical, and therapeutic MPNST research and clinical management since the 2002 international consensus conference.

Characteristic	2002	2016
Natural history of PN growth	(i) Unknown; (ii) May be erratic	(i) Well characterized; (ii) Identification of distinct nodular lesions (DNL) with different growth pattern
Imaging	(i) Role of FDG-PET unclear; (ii) FLT PET should be considered	(i) FDG-PET has clear role; (ii) FLT-PET under evaluation
Pathology	(i) ANF do not fit in category; (ii) Locally aggressive; (iii) Do not metastasize	(i) Identification of ANF as MPNST precursor
Risk for transformation	(i) Nodular PN, large central PN, NF neuropathy	(i) Distinct nodular, FDG-avid lesions
Pathogenesis	(i) NF1 microdeletion; (ii) p27, p53, p16	(i) CDKN2A/B; (ii) SUZ12, EED
Mouse models	(i) Briefly mentioned	(i) Preclinical trials consortium using GEMM
Chemotherapy targeted therapy	(i) Very few, if any, MPNST-specific data	(i) Prospective trial of chemotherapy completed; (ii) MPNST-specific targeted trials ongoing; (iii) SARC and NF clinical trials consortium
Access to tissue	(i) Importance of tissue banking	(i) CTF NF biobank
Data collection	(i) International database recommended	(i) No international database established

ANF: atypical neurofibroma; DNL: distinct nodular lesion; FDG-PET: fluorodeoxyglucose positron emission tomography; FLT-PET: fluorothymidine positron emission tomography; GEMM: genetically engineered mouse model; PN: plexiform neurofibroma; SARC: Sarcoma Alliance for Research through Collaboration (research and advocacy group); CTF: Children’s Tumor Foundation.