pediatric cerebellopontine angle lesions

OBJECT: The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors. METHODS: The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort...

Neurenteric cysts are rare and benign lesions that consist of ectopic alimentary tissue residing in the central nervous system. They tend to occur most frequently in an intraspinal rather than intracranial location. Intracranial neurenteric cysts are a rare occurrence in the pediatric population. These lesions typically present as unilateral cystic structures in the lower cerebellopontine angle and craniocervical junction. To the authors' knowledge, there have been no reported cases of bilateral localization of intracranial neurenteric cysts...

OBJECT: Neurenteric cysts (NCs) are endothelium-lined structures of presumed endodermal origin. There have been few pediatric series of intracranial NCs reported previously. The authors present their experience in the management of these lesions. METHODS: A retrospective chart review of all cases of NCs identified between 1977 and 2007 was carried out. Demographics, details of clinical presentation, surgical therapy, and outcome data were extracted. RESULTS: Eleven cases were identified, involving 6 girls and 5 boys...

Cerebellopontine angle (CPA) tumors are uncommon in children, and the pathological spectrum is different from that of adults. In this study, we reviewed the pathological diagnosis of pediatric patients with a CPA tumor to determine the pattern in this age group. In a cohort of 267 patients with posterior fossa tumor, tumor locations were determined with preoperative magnetic resonance imaging (MRI). The pathological diagnosis, imaging characteristic, and treatment outcomes of patients with CPA tumors was reviewed and analyzed...

OBJECTIVE: Epidermoid cysts of the cerebellopontine angle (CPA) can be a surgical challenge for the pediatric neurosurgeon. Ideally, total removal must be achieved; however, occasional adhesions of these tumors to vital neurovascular structures and extension far beyond the midline may preclude their total removal. The aims of this article are to present an alternative surgical approach to these lesions and to provide the rationale for this technique. MATERIAL AND METHODS: A 16-year-old boy was admitted to our pediatric neurosurgery department with a 1-year history of nonspecific headaches...

Hemifacial spasm is a disorder of the seventh cranial nerve, which is characterized by irregular, involuntary and recurrent tonic and clonic contractions of the ipsilateral facial expression muscles. This disorder affects mainly adults, and there are few cases reported in childhood. The main etiologies are vascular problems, although tumors are an important cause of hemifacial spasm via a direct or an indirect mass effect. We report a 6-year-old girl who presented with right hemifacial spasm. Magnetic resonance imaging showed a lesion in the cerebellopontine angle, extending from the midbrain to the pons with a slight mass effect on the fourth ventricle...

OBJECT: Epidermoid tumors are benign lesions representing 1% of all intracranial tumors. There have been few pediatric series of intracranial epidermoid tumors reported previously. The authors present their experience in the management of these lesions. METHODS: The neurosurgical database at the Hospital for Sick Children was searched for children with surgically managed intracranial epidermoid tumors. The patients' charts were reviewed for demographic data, details of clinical presentation, surgical therapy, and follow-up...

A chloroma or granulocytic sarcoma is an extramedullary leukemia. It can be encountered at any anatomical location, but until now only three cases have been reported in the cerebellopontine angle. We present an 8-year old patient with an extraaxial chloroma of the cerebellopontine angle to highlight this very rare and malignant pathology in the differential diagnosis of cerebellopontine angle tumors. The presented case, being the fourth chloroma in the cerebellopontine angle, occurred in the absence of relapse which is very unusual for these lesions...

PURPOSE: Craniopharyngiomas with a large posterior fossa extension beyond the level of the foramen magnum are very rare and usually removed in two stages. The objective of this paper is to report that such rare cases of giant cystic predominantly retrochiasmatic retroclival craniopharyngiomas can be completely excised by an anterior transpetrous approach in a single stage. MATERIALS AND METHODS: The first case was a 6-year-old boy who presented with a 1-year history of recurrent episodes of seizures, headache, and vomiting...

OBJECTS: Intra-axial dermoid cysts are rare intracranial space occupying lesions, more so in the pediatric age group. Dermoid cysts account for about 0.2 to 1.8% of all intracranial tumors and are commonly located in the cisternal spaces, mainly in the cerebellopontine angle and parasellar cisterns. A purely intra-axial position as reported in this paper is quite exceptional. MATERIALS AND METHODS: We report a case of intra-axial dermoid cyst located in the cerebellum that was surgically treated...

BACKGROUND: Cerebellopontine angle (CPA) lesions are more commonly found in adults in which they account for 5-10% of all intracranial tumors. However, they are uncommon in children, with an incidence of only 1%. MATERIALS AND METHODS: This is a review of the management of CPA lesions in children admitted to the Hospital Nacional de Pediatría "Profesor Doctor Juan P. Garrahan" (Argentine National Pediatrics Hospital "Professor Juan P. Garrahan") between January 1988 and December 2003...

A 3-month-old boy presented to the pediatric neurosurgery service with central hypotonia. Magnetic resonance images of the brain revealed a homogeneously enhancing dumbbell-shaped mass located in the fourth ventricle and extending into the left cerebellopontine angle (CPA). A suboccipital craniotomy was performed and a gross-total resection of the mass was achieved. Pathological examination of the resected tissue confirmed the diagnosis of capillary hemangioma. To the authors' knowledge, this is the first reported case of a capillary hemangioma occurring in this location...

Endolymphatic sac tumors (EST) are rare intracranial tumors originating from the pars rugosa of the endolymphatic sac. Although typically described as histologically nonaggressive lesions, nevertheless they are termed adenocarcinomas and often become locally invasive. We report two patients with histologically proven EST with unique clinical features: the first pediatric case of an EST in an 11-year-old patient whose complaints started at the age of seven; and, a second patient, a 43-year-old man, the first report of metastatic EST which appeared in a remote location from the original site of surgery...

INTRODUCTION: This review is primarily based on peer-reviewed scientific publications and on the authors' experience in the field of intraoperative neurophysiology. The purpose is a critical analysis of the role of intraoperative neurophysiological monitoring (INM) during various neurosurgical procedures, emphasizing the aspects that mainly concern the pediatric population. Original papers related to the field of intraoperative neurophysiology were collected using medline. INM consists in monitoring (continuous "on-line" assessment of the functional integrity of neural pathways) and mapping (functional identification and preservation of anatomically ambiguous nervous tissue) techniques...

Acoustic schwannoma is a slow-growing tumor and usually occurs in adult patients. We report a rare pediatric case of acoustic schwannoma with high proliferative potential. A 10-year-old boy was diagnosed as having a right cerebellopontine angle tumor. The tumor was subtotally resected. Histological examination revealed a typical acoustic schwannoma with a few mitotic figures. Chromosomal analysis showed no abnormality on the long arm of chromosome 22 associated with neurofibromatosis type 2. The lesion re-grew rapidly as an acoustic schwannoma, necessitating subtotal resection on three occasions and CyberKnife radiosurgery...

Stereotactically guided external beam irradiation may be a useful form of treatment for small, well-circumscribed, but surgically inaccessible, primary brain tumors that are either benign or of low malignant potential. Between March 1988 and December 1991, 10 children and adolescents with previously untreated primary brain tumors were treated with stereotactic external beam irradiation (SEBI) using a linac-based dynamic technique. Eleven lesions were treated in the 10 patients. Treatment was given using a collimator diameter of 1...

Ear canal, middle ear, temporal bone, and CPA angle masses (except for cholesteatomas) are rare in the pediatric population. The physician needs to have a high degree of suspicion for such lesions if a child presents with ear pain unrelated to infection or otorrhea that fails to improve after treatment. A precise diagnosis needs to be made in these children and also in those with hearing loss, vertigo, and facial paralysis. The most useful imaging procedures for ear, temporal bone, and CPA masses are CT and MR imaging...