Background. Although myxopapillary ependymomas are generally benign wi
th a tendency for slow growth and local recurrence, they are capable o
f spread within the nervous system and of extraneural metastasis. Hist
ologic features have not been helpful in determining which patients ar
e at risk for recurrence or dissemination, making management decisions
difficult. Methods. A retrospective review was conducted of 14 cases
of myxopapillary ependymoma. The nucleolar organizing region (NOR) sta
ining method was used to determine if this technique was useful in the
management of these tumors. Results. Five patients had total resectio
ns of encapsulated lesions, four had total resections of adherent tumo
rs, and four had subtotal resections. Twelve received postoperative ra
diation therapy. With a mean follow-up of 80 months, 12 patients are w
ell and disease-free. Two patients have had recurrences after surgery
and irradiation, leading to death in one and disability in the other.
The mean number of NOR per cell in eight specimens ranged from 0.4-1.6
4. The patient who died with intracranial spread had the highest numbe
r of NOR per cell. Conclusions. Based on these data and a review of th
e literature, it is recommended that radiation be delayed until recurr
ence in tumors that have been totally resected. Local radiation therap
y may be indicated in subtotally resected tumors. NOR staining shows p
romise in predicting the likelihood of spread of tumor. Patients with
myxopapillary ependymomas should be followed indefinitely because of t
he potential for late recurrence, even after aggressive therapy.