Related Experiment Video For H3K27me3
Updated: May 10, 2026
Defining Gene Functions in Tumorigenesis by Ex vivo Ablation of Floxed Alleles in Malignant Peripheral Nerve Sheath Tumor Cells
Published on: August 25, 2021
Malignant peripheral nerve sheath tumor presenting in the cauda equina: diagnostic and biological pearls.
Mason Bartman1, Preston D'Souza2, Kseniia Malkova3
1John Sealy School of Medicine, The University of Texas Medical Branch, Galveston, Texas.
Background:
Malignant peripheral nerve sheath tumors (MPNSTs) are rare and highly malignant tumors of neural crest origin. No matter their anatomical location, they are difficult to treat and carry a poor prognosis.
Observations:
This report highlights the complexities of the diagnosis and management of a patient with MPNST of the cauda equina. The tumor retained the expression of H3K27me3, which is expected in spinal MPNSTs but uncommon in most sporadic and neurofibromatosis type 1-driven MPNSTs.
Lessons:
The described diagnostic nuance should be taken into consideration in patients with suspected MPNST of the cauda equina, as these tumors require swift neurosurgical intervention in order to maintain neurological function. Additional treatment modalities include neoadjuvant and adjuvant chemotherapy and radiotherapy. Although improvements in local control and progression-free survival have been reported, the role of radiation therapy and chemotherapy has been underinvestigated due to the rarity of MPNSTs presenting in this region. https://thejns.org/doi/10.3171/CASE24723.
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