Related Experiment Video
Updated: Aug 6, 2026
Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Diagnosing metastatic angiosarcoma arising from MPNST using H3K27me3 staining in NF1: illustrative case
Michael K A Hagan1, Robert J Spinner2, Jorge Torres-Mora3
1Mayo Clinic Alix School of Medicine, Mayo Clinic College of Medicine and Science, Rochester.
Background:
Malignant peripheral nerve sheath tumors (MPNSTs) are malignant tumors that rarely demonstrate heterologous differentiation from angiosarcomatous components. These lesions can be hard to accurately diagnose.
Observations:
A 25-year-old man with neurofibromatosis type 1 and a history of a left lumbar subcutaneous MPNST developed a lung metastasis of only the angiosarcoma component of the MPNST. Prior to H3K27me3 staining of the lung mass, there was no association of the angiosarcoma with the MPNST. Reinterpretation of the lumbar subcutaneous MPNST led to the identification of a previously unrecognized angiosarcomatous portion in that tumor as well.
Lessons:
This case highlights the importance of using H327me3 staining for accurate diagnosis of biopsies only capturing the heterologous components of MPNST. https://thejns.org/doi/10.3171/CASE251012.
