Artikel
Intraventricular ependymal tumors. Surgical management in adults
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Veröffentlicht: | 9. Juni 2017 |
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Gliederung
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Objective: Intraventricular ependymal tumors in adults represent a rare and challenging neurosurgical entity. The role of radiotherapy is subject to controversial discussion and chemotherapy has shown to be ineffective, therefore complete surgical resection remains the treatment of choice, but scientific data regarding the optimal treatment is sparse. We report our experience with 46 patients treated at our neurosurgical centre in a retrospective analysis.
Methods: 46 consecutive, patients (age<18y) with intraventricular ependymoma/subependymoma were treated at our institution between 1987 and 2016 and were included. Patient demographics, clinical presentation, imaging data, histology and surgical treatment with related morbidity was analyzed from patients charts, surgical reports. Clinical outcome was assessed using the modified Rankin Scale (mRS) and Spitzer Index for quality of life (SQLI).
Results: 35 male and 11 female patients with a mean age of 47 years (range 18 to 78 years) were included. 47 open surgeries and one biopsy were performed using suboccipital (n=31), frontal transcortical (n=12) and interhemispheric transcallosal (n=4) approaches. The most common tumor location was within the fourth ventricel (n=31) followed by the lateral ventricels (n=16). Gross total resection (GTR) was achieved in 39 operations (83%) and subtotal resection in 8 operations (17%). The overall rate of perioperative mortality was 4% (n=2). Postoperative morbidity in terms of neurological deficit was permanent in 13% (n=6) or transient in 8% (n=4). Neurological deficits presented as hemiparesis (n=1), cranial nerve disturbances (n=3), ataxia (n=1) and vertigo (n=1). Mean mRS was 1.75 at admittance, 1.71 at discharge and 1.54 at last Follow-up. Mean SQLI at last follow up was 9.5. Mean Follow-up was 52 months, mean Progression free survival was 42 months. Tumors were classified as subependymoma WHO grade I (n=25), ependymoma WHO grade II (n=20) and anaplastic ependymoma WHO grade III (n=3). The mean proliferation Index was 4.3. While no recurrence was seen in subependymoma WHO°I we observed 3 patients with recurrent tumors (WHO°II) and one patient with multiple recurrences (WHO°III). Two patients underwent radiotherapy after subtotal resection and one after tumor recurrence.
Conclusion: In our series we demonstrate that long-term tumor control with good clinical outcome and high quality of life can be achieved by surgical resection of intraventricular, ependymal tumors, making it the method of choice for this entity. Recurrence rates are especially low in subependymoma WHO°I, even after subtotal resection. Adjuvant therapy remains an option only for higher grade or recurrent tumors.