Myxopapillary ependymomas (MPE) are benign spinal intradural tumors (WHO grade I) that characteristically occur in the conus medullaris/filum terminale region. They are slow growing tumors with a good prognosis and long-term survival. 1 We report a rare case of double MPE occurring at both extremities of the filum terminale. In the light of the present case and an extensive review of the literature, we summarize the imaging characteristics and intra-operative findings that should be present when considering the diagnosis of double MPE. Knowledge of this entity should remind recall physicians to assess in detail the entire region of the conus medullaris/filum terminale to prevent missing the diagnosis of a second MPE. This is important since the surgical treatment should address both lesions.
CASE REPORTA 28-year-old man previously in good health presented with a severe lower back pain that had increased over the last two months. The questionnaire was negative for lower extremity paresthesia or paresia, for voiding difficulty and for sexual dysfunction. Complete neurological examination was normal including perianal sensation and rectal tone. Lumbosacral magnetic resonance imaging (MRI) revealed two isolated intradural lesions. The most rostral lesion located at L3-L4 measured 2.5 cm in its longest axis. The second, extending from L5-S2, measured 6.6 cm in its longest axis. Both lesions were heterogeneous in T1 and T2 and strongly enhanced after gadolinium injection (Figure 1, A-F The lesions were approached through a L2 laminotomy and L3 to S2 laminectomy. After opening the dura, both lesions appeared well encapsulated and isolated from one another (Figure 1, G&H). Both lesions' capsule was intact, with no signs of previous rupture or diffuse growth on the nerve roots of the cauda equina. The filum terminale was macroscopically inspected and seemed of normal size and shape between both lesions. For the most rostral lesion, an en bloc excision was performed. The cauda equina lesion was partially removed given the dense adherences with the lumbosacral roots. Intra-operative preliminary report stated both lesions were identical MPE.The definitive pathological assessment for both lesions showed tumor cells arranged in a papillary pattern around vascular areas, forming pseudorosettes, as well as large regions of mucoid degeneration. The immunohistochemistry for glial fibrillary acidic protein was strongly positive in both lesions. Cytokeratine (AE1/AE3) was mainly negative. MIB-1 was less