ABSTRACT
OBJECTIVE: Strategies for managing supratentorial primitive neuroectodermal tumors(PNETs) in children include surgical resection, craniospinal irradiation and chemotherapy. This study is performed in order to compare the efficacy of various methods of treatment and to describe its optimal management. METHODS: We have reviewed all medical records and pathology slides of six children(four males and two females) with supratentorial PNET from November, 1987 to May, 2003. The extent of resection was confirmed by computed tomography and magnetic resonance studies. RESULTS: The patients were aged 1 to 13 years and treated postoperatively with/without adjuvant therapy. Tumor location included was four cortical, one gangliobasal, and one pineal region. The presenting symptoms and signs consisted of increased intracranial pressure and focal neurological deficits such as seizure and hemiparesis. The treatment consisted of surgical resection alone in one patient, postoperative radiotherapy in one patient, postoperative chemotherapy in one, and postoperative radiotherapy with chemotherapy in three. Five patients lived more than 12 months after diagnosis and one patient among them has been living more than 5 years after diagnosis. CONCLUSION: We can improve the survival and prognosis of supratentorial PNET patients by radical gross total resection of tumor followed by craniospinal irradiation and aggressive chemotherapy. First of all, gross total resection of tumor is the most important among many factors.
Subject(s)
Child , Humans , Male , Craniospinal Irradiation , Diagnosis , Drug Therapy , Intracranial Pressure , Medical Records , Neural Plate , Neuroectodermal Tumors, Primitive , Paresis , Pathology , Prognosis , Radiotherapy , SeizuresABSTRACT
OBJECTIVE: Traditionally, the main indications for surgery in vascular-related lesion were based upon reduction or control of seizures, reversal of symptoms of deficits related to mass effect, and prevention of hemorrhage or recurrent hemorrhage. However, the results of surgical treatment for seizure control are disappointing in some reports. Here we describe surgical strategies and our experience in treating patients with intractable seizures associated with vascular-related lesions according to sophisticated presurgical and intraoperative evaluation. METHODS: Twelve(4.5%) patients were selected for this study out of total 264 patients with resective epilepsy surgery at our epilepsy center during four years since 1992. All were treated with anticonvulsant agents but became refractory. These patients operated on under local or general anesthesia for resection surgery, underwent presurgical and intraoperative evaluation for identification of adjacent, beyond or remote epileptogenic area and the eloquent area. RESULTS: Of these 12 patients, vascular malformations(AVM, cavernous angioma) were 7, overt hemorrhage due to vascular lesion were 2 and intractable ongoing seizure after vascular surgery were 3. Other vascular lesion including occlusive disease, moyamoya disease or previous hemorrhage were excluded in this study. The location of the lesion was mainly temporal and peri-Rolandic areas, and dual pathology was verified in 2 cases of 6 temporal lesion. The surgical outcome(class I;7, II;3, III;1, IV;1) was excellent by Engel's classification. CONCLUSION: Control of seizures related to vascular lesions remains strong indication for surgical resection. For this reason, careful presurgical evaluations are essential to evaluate the remote epileptogenic area, especially in temporal lesion. Intraoperative acute recording(ECoG) and functional mapping by electrical stimulation or SSEP are important for maximal resection of epileptogenic area with minimal sequellae.