title: Clinicopathological features of myxopapillary ependymoma.
abstract: Myxopapillary ependymoma (MPE) is a rare and distinct variant of ependymoma with a tendency for local recurrence and metastasis. Its clinicopathological spectrum is heterogenous, underscoring the need to understand and characterize MPE for better diagnosis and treatment. The purpose of this study was to explore the tumor biology and assess the management of patients with MPE. Tumors from a cohort of 19 patients were analyzed by light microscopy, electron microscopy, immunohistochemistry and fluorescence in situ hybridization (FISH). Clinical characteristics, therapeutic options and clinical follow-up data were also analyzed. Back pain was the most common presenting symptom. The main pathological morphology observed was papillae embedded in a myxoid background, but other rare morphologies were also present. Immunostaining revealed epidermal growth factor receptor (EGFR) expression in four MPE, while FISH for EGFR was negative. No correlation between tumor recurrence and EGFR overexpression was found. Ultrastructural examination revealed adherens junctions and intracytoplasmic lumina with microvilli. Patients with gross-total resection (GTR) had no tumor recurrence (p=0.021). Also, patients with subtotal resection (STR) followed by radiotherapy showed a higher local control rate than patients with STR alone (p=0.043). The diagnosis of MPE should be made considering the histology, immunohistochemistry, imaging studies and anatomical site. GTR of the tumor or STR followed by radiotherapy are more likely to avoid tumor recurrence than STR alone. Based on our findings, there is no correlation between tumor recurrence and EGFR expression.
authors: Department of Pathology, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing 210002, China. Electronic address: drwh77@hotmail.com.
authors: Hai
authors: H
authors: Wang
authors: Department of Pathology, Affiliated Hospital of Nantong University, Nantong, China.
authors: Shu
authors: S
authors: Zhang
authors: Department of Molecular & Cellular Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors: Sumaiyah K
authors: SK
authors: Rehman
authors: Department of Neurosurgery, Jinling Hospital, Nanjing University School of Medicine, Nanjing 210002, China.
authors: Zhiyuan
authors: Z
authors: Zhang
authors: Department of Pathology, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing 210002, China.
authors: Wanchun
authors: W
authors: Li
authors: Department of Molecular & Cellular Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors: Mohammad Shahidul
authors: MS
authors: Makki
authors: Department of Pathology, Jinling Hospital, Nanjing University School of Medicine, 305 East Zhongshan Road, Nanjing 210002, China.
authors: Xiaojun
authors: X
authors: Zhou
mesh_terms: D022005:Adherens Junctions / Q000648:ultrastructure; D000293:Adolescent; D000328:Adult; D000368:Aged; D001416:Back Pain / Q000209:etiology; D015331:Cohort Studies; D004806:Ependymoma / Q000150:complications / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D066246:ErbB Receptors / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008279:Magnetic Resonance Imaging; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D013120:Spinal Cord Neoplasms / Q000150:complications / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C512478:EGFR protein, human; D066246:ErbB Receptors
keywords: EGFR; Immunohistochemistry; Myxopapillary ependymoma; Pathology; Radiotherapy; Ultrastructure