Journal article
Pediatric Myxopapillary Ependymomas: A Clinicopathologic Evaluation
Journal of pediatric hematology/oncology, Vol.43(8), pp.e1194-E1200
11/01/2021
DOI: 10.1097/MPH.0000000000002041
PMID: 33395181
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.
Details
- Title: Subtitle
- Pediatric Myxopapillary Ependymomas: A Clinicopathologic Evaluation
- Creators
- Kathryn L Eschbacher - Departments of Laboratory Medicine and Pathology.Amulya Nageswara Rao - Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, Mayo Clinic, Rochester, MNPatricia T Greipp - Departments of Laboratory Medicine and Pathology.Troy J Gliem - Departments of Laboratory Medicine and Pathology.David J Daniels - Neurologic Surgery.Deepti Warad - Mayo Clinic in FloridaLaurence J Eckel - Diagnostic Radiology.Aditya Raghunathan - Departments of Laboratory Medicine and Pathology.
- Resource Type
- Journal article
- Publication Details
- Journal of pediatric hematology/oncology, Vol.43(8), pp.e1194-E1200
- DOI
- 10.1097/MPH.0000000000002041
- PMID
- 33395181
- ISSN
- 1077-4114
- eISSN
- 1536-3678
- Language
- English
- Date published
- 11/01/2021
- Academic Unit
- Pathology
- Record Identifier
- 9984276455602771
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