Malignant Peripheral Nerve Sheath Tumor of the Liver
Satoru Kakizaki1, Norio Horiguchi, Toshiyuki Otsuka
1Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Japan.
Abstract:
Malignant peripheral nerve sheath tumor (MPNST) of the liver is rare. Most cases of MPNST are accompanied by neurofibromatosis 1 (NF-1, von Recklinghausen's disease). We herein report an autopsy case of MPNST without NF-1 and review the pertinent literature. The tumor occupied the entire lobe of the liver, and was 18 cm in maximum diameter. The tumor revealed necrosis and cystic changes with hemorrhage and it had also metastasized to the peritoneum. Microscopically, the tumor was composed of pleomorphic spindle cells with hyperchromatic nuclei and mitogenic figures. The spindle cells stained positive for both S-100 and vimentin antibodies.
Insights
Malignant peripheral nerve sheath tumors (MPNST) of the liver are rare, especially without neurofibromatosis 1 (NF-1). This report details an autopsy case of a large liver MPNST in a patient without NF-1.
Area of Science:
- Oncology
- Pathology
Background:
- Malignant peripheral nerve sheath tumors (MPNST) are rare hepatic neoplasms.
- Most MPNST cases are associated with neurofibromatosis 1 (NF-1).
Observation:
- This report details an autopsy of a large MPNST (18 cm) occupying an entire liver lobe.
- The tumor exhibited necrosis, cystic changes, and hemorrhage, with peritoneal metastasis.
Findings:
- Microscopic examination revealed pleomorphic spindle cells with hyperchromatic nuclei and mitoses.
- Tumor cells stained positive for S-100 and vimentin, characteristic of nerve sheath origin.
Implications:
- This case highlights the possibility of MPNST occurring in the liver without NF-1.
- Understanding the characteristics of rare MPNST is crucial for diagnosis and management.
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