Biomaterial Database

Posterior fossa lateral ependymoma in childhood. 1996

M G Nagib, and M T O'Fallon

Neurosurgical Associates, Ltd., Minneapolis, MN, USA.

Over a period extending from 1984 to 1993, 16 children ranging in ages from 2 months to 12 years with posterior fossa ependymoma were treated. Four of these tumors were classified as lateral ependymomas because of their configuration and suspected site of origin. These 4 patients' ages ranged from 2 months to 5 years. Signs and symptoms of increased intracranial pressure and cranial nerve dysfunction were the hallmark of their presentation. Their imaging evaluation included a preoperative and postoperative CT san and/or MRI. A gross total surgical resection' was completed in the 4 children. At least a 1-year follow-up was available for all the patients. Intraoperative brain stem evoked potentials and facial and glossopharyngeal nerve monitoring were used in all the surgeries. Three children required a ventriculoperitoneal shunt postoperatively. Chemotherapy was used postoperatively in 3 children. The 4th child did not receive chemotherapy due to parental refusal and succumbed to a recurrence 1 years postoperatively. Radiation therapy was given to 2 children. Tumor recurrences developed in all children at 12 months, 18 months, 3 years and 4 years postoperatively in proximity to the original tumor site. Histologic sections in 3 patients, including those at the time of recurrence, were consistent with a low mitotic index. Only 1 child's histologic diagnosis was consistent with a high-grade ependymoma. The lower cranial nerve dysfunction transiently worsened in all the patients and was permanent in 2. The child's age, tumor histology and extent of resection play an important role in the prognosis of posterior fossa ependymoma. The lateral posterior fossa ependymoma has a particularly poor prognosis due to its location and its significant postoperative morbidity. The authors propose a combined midline and lateral suboccipital approach in order to expose the fourth ventricular floor, upper cervical spine, lateral recess, cerebellomedullary fissure, cerebellopontine and the cerebellomedullary cistern in an attempt at a "total' resection with a focus on the site of origin. The usage of neurophysiological monitoring appears to be useful in limiting and predicting the extent of postoperative complications. However, prolonged morbidity is likely and is commonly related to lower cranial nerve deficits. The author proposes a proactive approach in order to limit the sequelae of these complications.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D009460	Neurologic Examination	Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system.	Examination, Neurologic,Neurological Examination,Examination, Neurological,Examinations, Neurologic,Examinations, Neurological,Neurologic Examinations,Neurological Examinations
D002528	Cerebellar Neoplasms	Primary or metastatic neoplasms of the CEREBELLUM. Tumors in this location frequently present with ATAXIA or signs of INTRACRANIAL HYPERTENSION due to obstruction of the fourth ventricle. Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA. The cerebellum is a relatively common site for tumor metastases from the lung, breast, and other distant organs. (From Okazaki & Scheithauer, Atlas of Neuropathology, 1988, p86 and p141)	Benign Cerebellar Neoplasms,Cerebellar Cancer,Malignant Cerebellar Neoplasms,Cerebellar Neoplasms, Benign,Cerebellar Neoplasms, Malignant,Cerebellar Neoplasms, Primary,Cerebellar Tumors,Neoplasms, Cerebellar,Neoplasms, Cerebellar, Benign,Neoplasms, Cerebellar, Malignant,Neoplasms, Cerebellar, Primary,Primary Neoplasms, Cerebellum,Benign Cerebellar Neoplasm,Cancer, Cerebellar,Cerebellar Cancers,Cerebellar Neoplasm,Cerebellar Neoplasm, Benign,Cerebellar Neoplasm, Malignant,Cerebellar Neoplasm, Primary,Cerebellar Tumor,Cerebellum Primary Neoplasm,Cerebellum Primary Neoplasms,Malignant Cerebellar Neoplasm,Neoplasm, Benign Cerebellar,Neoplasm, Cerebellar,Neoplasm, Cerebellum Primary,Neoplasm, Malignant Cerebellar,Primary Cerebellar Neoplasm,Primary Cerebellar Neoplasms,Primary Neoplasm, Cerebellum,Tumor, Cerebellar
D002551	Cerebral Ventricle Neoplasms	Neoplasms located in the brain ventricles, including the two lateral, the third, and the fourth ventricle. Ventricular tumors may be primary (e.g., CHOROID PLEXUS NEOPLASMS and GLIOMA, SUBEPENDYMAL), metastasize from distant organs, or occur as extensions of locally invasive tumors from adjacent brain structures.	Intraventricular Neoplasms,Ventricular Neoplasms, Brain,Ventricular Tumors, Brain,Brain Ventricular Neoplasms,Cerebral Ventricle Tumors,Cerebroventricular Neoplasms,Neoplasms, Cerebral Ventricle,Neoplasms, Cerebroventricular,Neoplasms, Intraventricular,Neoplasms, Ventricular, Brain,Brain Ventricular Neoplasm,Brain Ventricular Tumor,Brain Ventricular Tumors,Cerebral Ventricle Neoplasm,Cerebral Ventricle Tumor,Cerebroventricular Neoplasm,Intraventricular Neoplasm,Neoplasm, Brain Ventricular,Neoplasm, Cerebral Ventricle,Neoplasm, Cerebroventricular,Neoplasm, Intraventricular,Neoplasms, Brain Ventricular,Tumor, Brain Ventricular,Tumor, Cerebral Ventricle,Tumors, Brain Ventricular,Tumors, Cerebral Ventricle,Ventricle Tumor, Cerebral,Ventricle Tumors, Cerebral,Ventricular Neoplasm, Brain,Ventricular Tumor, Brain
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003131	Combined Modality Therapy	The treatment of a disease or condition by several different means simultaneously or sequentially. Chemoimmunotherapy, RADIOIMMUNOTHERAPY, chemoradiotherapy, cryochemotherapy, and SALVAGE THERAPY are seen most frequently, but their combinations with each other and surgery are also used.	Multimodal Treatment,Therapy, Combined Modality,Combined Modality Therapies,Modality Therapies, Combined,Modality Therapy, Combined,Multimodal Treatments,Therapies, Combined Modality,Treatment, Multimodal,Treatments, Multimodal
D003388	Cranial Fossa, Posterior	The infratentorial compartment that contains the CEREBELLUM and BRAIN STEM. It is formed by the posterior third of the superior surface of the body of the sphenoid (SPHENOID BONE), by the occipital, the petrous, and mastoid portions of the TEMPORAL BONE, and the posterior inferior angle of the PARIETAL BONE.	Clivus,Cranial Fossas, Posterior,Fossa, Posterior Cranial,Fossas, Posterior Cranial,Posterior Cranial Fossa,Posterior Cranial Fossas
D004806	Ependymoma	Glioma derived from EPENDYMOGLIAL CELLS that tend to present as malignant intracranial tumors in children and as benign intraspinal neoplasms in adults. It may arise from any level of the ventricular system or central canal of the spinal cord. Intracranial ependymomas most frequently originate in the FOURTH VENTRICLE and histologically are densely cellular tumors which may contain ependymal tubules and perivascular pseudorosettes. Spinal ependymomas are usually benign papillary or myxopapillary tumors. (From DeVita et al., Principles and Practice of Oncology, 5th ed, p2018; Escourolle et al., Manual of Basic Neuropathology, 2nd ed, pp28-9)	Ependymoma, Myxopapillary,Ependymoma, Papillary,Anaplastic Ependymoma,Cellular Ependymoma,Clear Cell Ependymoma,Papillary Ependymoma,Anaplastic Ependymomas,Ependymoma, Anaplastic,Ependymomas,Ependymomas, Anaplastic,Ependymomas, Myxopapillary,Ependymomas, Papillary,Myxopapillary Ependymoma,Myxopapillary Ependymomas,Papillary Ependymomas