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Pediatric Myxopapillary Ependymomas: A Clinicopathologic Evaluation
Kathryn L Eschbacher1, Amulya Nageswara Rao2, Patricia T Greipp1
1Departments of Laboratory Medicine and Pathology.
Abstract:
Myxopapillary ependymomas (MPEs) have an indolent clinical course, corresponding to World Health Organization Grade I. A total of 13 pediatric MPEs have been reported in the literature with "anaplastic features," including elevated proliferative activity (≥5 mitoses/10 high-power fields), necrosis, and microvascular proliferation. No consensus exists regarding the prognostic significance of such features. A retrospective clinicopathologic review of pediatric MPEs diagnosed between 1996 and 2018 at Mayo Clinic was performed. Totally, 8 pediatric MPEs (6 male; age: 7.52 to 16.88 y) were identified. Totally, 3 had disseminated disease at presentation. All patients underwent surgical resection (7 gross total; 1 subtotal). Totally, 5 cases harbored ≥5 mitoses/10 high-power fields (range: 5 to 9), 3 of which showed necrosis (2 with disseminated disease). Postsurgery, 2 patients received radiation; one with disseminated disease and another with increased mitotic activity/necrosis; neither has recurred (follow-up: 1.18 and 3.19 y). In all, 2 patients with disseminated disease, elevated mitotic activity, and necrosis had new metastatic disease/progression of nonresected metastatic foci (2.6 and 26.8 mo), received radiation therapy, and remain progression free (3.01 and 9.34 y). All patients are alive (median follow-up 1.31 y, range: 0.66 to 11.75). Among pediatric MPEs, the concurrent presence of elevated mitotic activity and necrosis may be associated with an aggressive clinical course, warranting closer surveillance and consideration of adjuvant therapies.
Insights
Pediatric myxopapillary ependymomas (MPEs) with high mitotic activity and necrosis may indicate aggressive disease. Close monitoring and adjuvant therapies are recommended for these high-risk MPE cases.
Area of Science:
- Pediatric neuro-oncology
- Neuropathology
- Clinical oncology
Background:
- Myxopapillary ependymomas (MPEs) are typically World Health Organization Grade I tumors with an indolent course.
- Anaplastic features, including high mitotic activity, necrosis, and microvascular proliferation, have been reported in a small subset of pediatric MPEs.
- The prognostic significance of these anaplastic features in pediatric MPEs remains unclear.
Purpose of the Study:
- To investigate the clinicopathologic features and outcomes of pediatric myxopapillary ependymomas.
- To evaluate the prognostic significance of anaplastic features, specifically elevated mitotic activity and necrosis, in pediatric MPEs.
Main Methods:
- Retrospective clinicopathologic review of pediatric MPEs diagnosed between 1996 and 2018 at Mayo Clinic.
- Analysis of patient demographics, tumor characteristics, treatment modalities, and clinical outcomes.
- Correlation of anaplastic features with disease progression and survival.
Main Results:
- Eight pediatric MPEs were identified, with 3 presenting with disseminated disease.
- Five cases showed elevated mitotic activity (≥5 mitoses/10 high-power fields), and 3 of these also exhibited necrosis.
- Patients with concurrent elevated mitotic activity and necrosis, particularly those with disseminated disease, showed aggressive behavior and progression, but remained progression-free after radiation therapy.
Conclusions:
- The concurrent presence of elevated mitotic activity and necrosis in pediatric MPEs may be associated with an aggressive clinical course.
- These findings suggest the need for closer surveillance in pediatric MPEs with these features.
- Consideration of adjuvant therapies may be warranted for pediatric MPEs exhibiting both elevated mitotic activity and necrosis.
