Orig&al papers llllqS 9 Springer-Verlag 1993 Child's Nerv Syst (1993) 9:3-6 Ependymomas of the fHum terminale in childhood: report of four cases and review of the literature Franco Maria Gagliardi, Luigi Cervoni, Maurizio Domenicucci, Paolo Celli, Maurizio Salvati Department of Neurological Sciences - Neurosurgery, "La Sapienza" University, Viale dell'Universitfi, 30a, 1-00185 Rome, Italy Received: 14 July 1992 Abstract. Four cases of ependymoma of the filum ter- minale occurring in childhood are reported. The clinical, therapeutic and prognostic features seen at this age and in adults were compared. Key words: Filum terminale - Ependymoma Childhood - Radiotherapy Introduction The filum terminale is a frequent localization for spinal ependymomas, accounting for 40-50% of cases [3, 9, 23]. Only 8-14% of ependymomas at this site affect children (age < 16 years) [5, 7, 23]. The treatment offilum terminale ependymoma is surgical excision, but in about half of cases this is possible only in part [25]; in this event, partial removal is followed, in general, by radiotherapy [12, 25]. On the basis of our experience and of published reports [1, 2, 5, 6, 9, 20, 23], we studied the clinical and therapeutic features of filum terminale ependymomas in children with respect to their adult counterparts. Particular attention is paid to the question of recurrences and metastases in relation to the type of surgical removal and the efficiency of radiotherapy. Patients and methods In the Neurosurgical Section of the Department of Neurological Sciences of Rome's La Sapienza University, 90 cases of spinal ependymoma were operated between 1951 and 1989, accounting for 24% of the total number of spinal cord tumors operated on during this period. Twenty-eight of these (32%) were located in the filum terminale, and four of these (12%) were in children (Table 1). The average was 13.2 years (range 12-14 years), three patients were male and one female. The duration of the clinical history averaged 12 months (range 6-24 months). The most frequent symp- toms were: lower lumbar pain or sciatic neuralgia, in all cases; Correspondence to." L. Cervoni, Via Conteverde, 50, 1-00185 Rome, Italy paresthesia, in two: gait disturbance, in two: urinary dysfunction, in two. Neurological findings were: flaccid paraparesis, in two cases; polyradicular sensory deficits, in two. Diagnostic investigation comprised: (1) cerebrospinal fluid analysis that showed an increased protein content in all four cases (range 0.1 0.6 g/l); (2) myelogra- phy that demonstrated a total block at L3 in three cases and a subtotal block at L2 in the last; (3) spinal computed tomography (CT), which was performed in two patients, showing a hyperdense neoformation that enhanced noticeably after administration of con- trast medium. The tumor was removed totally in two cases and partially in the other two, despite the aid of an operating microscope. Partial exci- sion had to be performed in the two cases because the tumor in- volved many spinal roots and/or infiltrated the conus medullaris. In all four cases, histological analysis classified the tumor as myxopap- illary ependymoma. One of the two young patients in whom re- moval was partial underwent a course of radiotherapy (total dose: 2500 rads), while the other was reoperated for a recurrence of the tumor 36 months after first operation. All four patients are alive and well after an average of 6.5 years from operation (range 4-9.1 years). Discussion The incidence of ependymomas varies from 10% to 20% of spinal tumors [9, 23]. Their location in the filum termi- Table 1. Clinical features in our four cases of ependymoma Total number 4 Mean age (years) 13.2 Ratio M:F 3:1 Mean clinical hystory (months) 12 Symptoms Pain 2 Paresthesia 2 Gait disturbance 2 Urinary dysfunction 2 Treatment Total removal 2 Partial removal 2 Radiotherapy 1 Recurrence 1 Mean survival (years) 6.5