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Anaplastic myxopapillary ependymoma: A case report and review of literature
Hiroshi Kanno1, Yukiko Kanetsuna2, Masamichi Shinonaga3
1Department of Neurosurgery, International University of Health and Welfare Atami Hospital, Atami 413-0012, Shizuoka, Japan. hiroshikannomd@nifty.com.
Background:
Myxopapillary ependymoma (MPE) is a pathological grade I tumor that arises in the filum terminale. MPE with anaplastic features is extremely rare, and only 5 cases have shown malignancy at the time of recurrence.
Case Summary:
The patient (a 46-year-old woman) had undergone a MPE operation 30 years ago. After subtotal resection of the tumor located in L4-S1, it had a solid component that extended to the adjacent subcutaneous region. Histologically, the tumor consisted of a typical MPE with anaplastic features. The anaplastic areas of the tumor showed hypercellularity, a rapid mitotic rate, vascular proliferation, and connective tissue proliferation. Pleomorphic cells and atypical mitotic figures were occasionally observed. The MIB-1 index in this area was 12.3%. The immunohistochemical study showed immunoreactivity for vimentin, glial fibrillary acidic protein and S100. The morphological pattern and immunohistochemical profile were consistent with anaplastic MPE. The patient tolerated surgery well without new neurological deficits. She underwent local irradiation for the residual tumor and rehabilitation.
Conclusion:
Although extremely rare, anaplastic MPE occurs in both pediatric and adult patients, similar to other ependymomas. At a minimum, close monitoring is recommended, given concerns about aggressive biological potential. In the future, further study is needed to determine the WHO classification criteria and genetic indicators of tumor progression. The possibility of malignant transformation of MPE should be taken into account, and patients with MPE should be treated with care and follow-up.
Insights
Anaplastic myxopapillary ependymoma (MPE) is a rare, aggressive tumor. Close monitoring is crucial due to its potential for malignant transformation and recurrence, necessitating careful patient follow-up.
Area of Science:
- Neuro-oncology
- Pathology
- Spinal Cord Tumors
Background:
- Myxopapillary ependymoma (MPE) is a WHO grade I tumor typically arising in the filum terminale.
- MPE with anaplastic features is exceptionally rare, with limited documented cases exhibiting malignancy upon recurrence.
Observation:
- A 46-year-old woman with a history of MPE surgery 30 years prior presented with a recurrent tumor exhibiting anaplastic characteristics.
- Histological analysis revealed hypercellularity, high mitotic rate, vascular/connective tissue proliferation, pleomorphic cells, and atypical mitotic figures in anaplastic areas.
- Immunohistochemistry confirmed glial fibrillary acidic protein, vimentin, and S100 expression, consistent with anaplastic MPE.
Findings:
- The recurrent tumor demonstrated features of anaplastic MPE, including a high MIB-1 index (12.3%) in malignant areas.
- The patient underwent subtotal resection, local irradiation, and rehabilitation, tolerating the procedures well without new neurological deficits.
Implications:
- Anaplastic MPE can occur in both pediatric and adult patients, highlighting its aggressive biological potential.
- Close monitoring and careful follow-up are essential for patients with MPE due to the risk of malignant transformation.
- Further research is needed to establish WHO classification criteria and identify genetic markers for anaplastic MPE progression.
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