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Updated: May 22, 2026

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Intracranial MPNST with multivacuolated cells and hyaline globules: a case with ultrastructural findings
Young Eun Kim1, Yeon-Lim Suh, Seung Eun Lee
1Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Abstract:
A case of intracranial malignant peripheral nerve sheath tumor (MPNST) with uncommon features due to recurrence is reported. The primary tumor showed typical histopathological features of MPNST with wavy nuclei and S-100 positivity. The patient's latest recurrent tumor resembled undifferentiated sarcoma with lipoblast-like multivacuolated cells and hyaline globules (HGs). Ultrastructurally, the vacuolated spaces contained granular materials derived from cystic dilation of the rough endoplasmic reticulum. The HG consisted of round osmophilic inclusions with or without a limiting membrane. The HGs and lipoblast-like multivacuolated cells may have been caused by the degeneration of tumor cells in myxoid stroma and abundant vasculature.
Insights
This study reports a rare case of intracranial malignant peripheral nerve sheath tumor (MPNST) that recurred with unusual features. The recurrent tumor showed characteristics of undifferentiated sarcoma, differing from the primary MPNST.
Area of Science:
- Neuro-oncology
- Surgical Pathology
- Electron Microscopy
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive neoplasms.
- Intracranial MPNSTs are exceptionally uncommon, and their recurrent behavior can present unique challenges.
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