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Sporadic Malignant Perineurioma: A Rare Diagnosis Among Malignant Peripheral Nerve Sheath Tumors
Megan C Everson1, Courtney Pendleton2, Megan M Jack1
1Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Background:
Malignant perineurioma is a rare subset of malignant peripheral nerve sheath tumors (MPNSTs) with ultrastructural and immunohistochemical features of perineurial differentiation, distinguishing it from other MPNSTs, which typically demonstrate Schwannian features. The clinical course and prognosis of this rare tumor is not well defined.
Methods:
The electronic medical records were searched for patients with a diagnosis of MPNST. Patients with a pathologic diagnosis of malignant perineurioma or MPNST with perineurial features were identified and further evaluated.
Results:
Five patients with malignant perineurioma, or MPNST with perineurial features, were identified. Four patients (2 male and 2 female) were included with tumors associated with a common digital nerve, small muscular branch to the deltoid, sciatic nerve, and accessory nerve. One patient with the pathology diagnosis meeting inclusion criteria was excluded, as no clinical information was available for this patient.
Conclusions:
Patients in our series presented at varied stages of disease. Clinical courses after diagnosis of malignant perineurioma, where follow-up was available, were largely uncomplicated with regard to recurrence and metastatic disease. Careful follow-up is indicated, and further work is needed to characterize the clinical course of these rare tumors.
Insights
Malignant perineurioma, a rare nerve tumor, shows perineurial differentiation. Early findings suggest a generally uncomplicated clinical course regarding recurrence and metastasis, but further study is needed.
Area of Science:
- Oncology
- Pathology
- Neurology
Background:
- Malignant perineurioma is a rare tumor subset of malignant peripheral nerve sheath tumors (MPNSTs).
- Distinguished by perineurial differentiation, unlike Schwannian-featured MPNSTs.
- The clinical course and prognosis of malignant perineurioma remain undefined.
Observation:
- This study identified five patients with malignant perineurioma or MPNST with perineurial features.
- Four patients with available clinical data were included, with tumors arising from various nerves.
- The patients presented at different disease stages.
Findings:
- Follow-up data for the included patients indicated largely uncomplicated clinical courses.
- Recurrence and metastatic disease were not significant issues in this small cohort.
- Malignant perineurioma exhibits distinct pathological features.
Implications:
- Careful, long-term follow-up is essential for patients diagnosed with malignant perineurioma.
- Further research is required to fully characterize the clinical behavior and prognosis of this rare tumor.
- Understanding perineurial differentiation is key to distinguishing malignant perineurioma from other MPNSTs.
