title: Spinal myxopapillary ependymomas: a retrospective clinical and immunohistochemical study.
abstract: BACKGROUND
  Myxopapillary ependymoma (MPE) is a rare subtype of ependymoma that develops almost exclusively within the spinal cord. Despite its benign biological nature, MPE has a propensity to recur locally or distantly. Although variables influencing the prognosis, such as age, the extent of surgery and radiotherapy, have been widely discussed, no definitive standard has been established. Compared to other spinal tumors, many fewer histological markers have been elucidated to assist the determination of the prognosis.
  
  
  METHODS
  Twenty-seven patients who underwent resection of MPE were enrolled. We determined their demographic features, imaging characteristics, clinical presentations and outcomes, surgical procedures and histological properties by chart review, telephone contact, reviewing of surgical notes, pre-/postoperative imaging and immunohistological staining.
  
  
  RESULTS
  GTR (gross total resection) was achieved in 18 patients (66.7 %) and STR (subtotal resection) in 9 (33.3 %). Although GTR rendered a better disease control rate, the difference was not significant. Pediatric patients suffered from a greater risk of recurrence as well as a shorter period to disease relapse. In the majority of cases, we observed the overexpression of platelet-derived growth factor receptor α (PDGFRα), matrix metalloproteinase-2 (MMP2) and matrix metalloproteinase-14 (MMP14). Epidermal growth factor receptor (EGFR) was observed in the tumors of 7 of 23 nonrecurrent patients, but not in any recurrent tumors.
  
  
  CONCLUSIONS
  The results of the present study indicate that the extent of resection and age are major factors related to tumor recurrence. Therefore, gross total resection is recommended whenever possible unless following neurological dysfunction is predictable. Moreover, pediatric patients need considerable attention after surgery, particularly in the early stages. PDGFRα, MMP2 and MMP14 may be new diagnostic and therapeutic targets and EGFR a potential predictor of improved prognosis for MPE.
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China.
authors.forename: Xi
authors.initials: X
authors.lastname: Chen
authors.affiliation: Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai, China.
authors.forename: Chao
authors.initials: C
authors.lastname: Li
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China.
authors.forename: Xiaoming
authors.initials: X
authors.lastname: Che
authors.affiliation: Department of Neuropathology, Huashan Hospital, Fudan University, Shanghai, China.
authors.forename: Hong
authors.initials: H
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai, China. dr_zhengyanliu@163.com.
authors.forename: Zhengyan
authors.initials: Z
authors.lastname: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004806:Ependymoma / Q000097:blood / Q000601:surgery*; D066246:ErbB Receptors / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology*; D012189:Retrospective Studies; D013120:Spinal Cord Neoplasms / Q000097:blood / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C512478:EGFR protein, human; D066246:ErbB Receptors
keywords: Epidermal growth factor receptor; Gross total resection; Myxopapillary ependymoma