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J. CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions. American Journal of Surgical Pathology. 17 (10), 1039-1045 (1993).","journal-title":"American Journal of Surgical Pathology"},{"issue":"174","key":"ref32","doi-asserted-by":"crossref","DOI":"10.3791\/62740-v","type":"journal-article","article-title":"Defining gene functions in tumorigenesis by ex vivo ablation of floxed alleles in malignant peripheral nerve sheath tumor cells","author":"Longo","year":"2021","unstructured":"Longo, J. F., Brosius, S. N., Carroll, S. L. Defining gene functions in tumorigenesis by ex vivo ablation of floxed alleles in malignant peripheral nerve sheath tumor cells. Journal of Visualized Experiments. 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