Myxopapillary ependymoma

FACTS

  • Origin: Ependymal cells of the filum terminale; WHO Grade 2 tumor.
  • Typical age: Young adults (20s–40s); slight male predominance.
  • Behavior: Slow-growing but can seed via CSF if capsule ruptured.
  • Prognosis: Excellent with gross total resection; recurrence higher if capsule disrupted.
  • Key distinction: Intradural extramedullary lesion (unlike intramedullary ependymomas higher up)

HPI

universal neuro exam
  • emphasis on back / leg pain and weakness
  • does pain worsen with Valsalva maneuvers (suggests intradural pressure).
  • Saddle anesthesia, urinary retention, or incontinence → red flags for cauda equina compression.
  • Duration often months to years due to slow growth.

PHYSICAL EXAM

universal neuro exam
  • rectal exam
  • Gait: Ataxic or spastic if chronic

IMAGING

MRI L-spine w/wo:
  • Intradural, extramedullary mass at conus medullaris or filum terminale.
  • Signal: T1 iso- to hypointense; T2 hyperintense; strong homogeneous enhancement with gadolinium.
  • Shape: Sausage-shaped or ovoid mass along filum.
  • Additional signs: Hemosiderin cap (chronic hemorrhage); cord displacement superiorly.
A/P
  • MRI of entire neuraxis to check for drop metastases (especially if capsule ruptured).
  • Microsurgical resection with goal of gross total resection (intact capsule).
  • Radiosurgery alone is ineffective, adjuvant XRT may be indicated if capsule violated, subtotal resection, or CSF dissemination
  • counsel:
    • “good” prognosis: sx < 1 year, confined to filum w/o adherence to cauda roots, GTR