Temporal lobe malformations, focal epilepsy, and FGFR3 mutations: a non-causal association?

Temporal lobe abnormalities and focal epilepsy have been documented in FGFR3-related clinical condition, including hypochondroplasia and Muenke syndrome. FGFR3 is expressed in the brain during development and could play a role in nervous system development and hippocampal formation. These observations suggest a non-casual association between temporal malformation, epilepsy, and FGFR3 mutations. Herein, we report clinical, electroclinical, and neuroimaging findings of three additional cases of focal epilepsy and temporal lobe malformations occurring in children with FGFR3 gene mutations.

Endoscopic aqueductoplasty and stenting in the treatment of isolated fourth ventricle in children: 20-year institutional experience.

BACKGROUND: Endoscopic aqueductoplasty with aqueductal stenting is an effective surgical procedure for the treatment of isolated fourth ventricle (IFV). Due to the rarity of the underlying pathology, it can be considered a rare procedure that can be performed with different surgical techniques and approaches. OBJECTIVES: To assess long-term functioning of permanent aqueductal stents implanted in children affected by hydrocephalus and IFV and to describe some variations of the same procedure. METHODS: We reviewed retrospectively all the patients presenting at our institution in the years 1999-2019 for symptoms of isolated fourth ventricle who underwent a surgical procedure of endoscopic aqueductoplasty and/or aqueductal stent. Surgical reports, radiological images, and surgical videos were retrospectively analyzed. RESULTS: Thirty-three patients with symptomatic isolated fourth ventricle (IFV) underwent fifty (50) neuroendoscopic procedures in the period observed. The median age of the patients at the time of first surgery was 7 months, with 22 premature babies. In twenty-nine patients (87.8%), a precoronal approach was performed, while four patients received a suboccipital burr hole. Ten patients were never reoperated since. Twenty-three patients underwent further surgeries: a new aqueductoplasty with aqueductal stent was performed in 13 cases. The remaining 10 patients required a combination of other procedures for management of hydrocephalus. Long-term follow-up showed a permanent stent functioning rate of 87% at 2 years and 73% at 4 years, remaining stable afterwards at very long term (20 years). CONCLUSION: Endoscopic aqueductoplasty and stenting is a reliable procedure in the long-term management of isolated fourth ventricle.

Dural venous sinus anatomy in children with external hydrocephalus: analysis of a series of 97 patients.

PURPOSE: To evaluate the anatomical variations of dural venous sinuses in children with external hydrocephalus, proposing a radiological grading of progressive anatomic restriction to venous outflow based on brain phase-contrast magnetic resonance venography (PC-MRV); to evaluate the correlation between positional plagiocephaly and dural sinuses patency; and to compare these findings with a control group to ascertain the role of anatomical restriction to venous outflow in the pathophysiology of external hydrocephalus. METHODS: Brain MRI and PC MRV were performed in 97 children (76 males, 21 females) diagnosed with external hydrocephalus at an average age of 8.22 months. Reduction of patency of the dural sinuses was graded as 1 (stenosis), 2 (complete stop) and 3 (complete agenesis) for each transverse/sigmoid sinus and sagittal sinus. Anatomical restriction was graded for each patient from 0 (symmetric anatomy of patent dural sinuses) through 6 (bilateral agenesis of both transverse sinuses). Ventricular and subarachnoid spaces were measured above the intercommissural plane using segmentation software. Positional plagiocephaly (PP) and/or asymmetric tentorial insertion (ATI) was correlated with the presence and grading of venous sinus obstruction. These results were compared with a retrospective control group of 75 patients (35 males, 40 females). RESULTS: Both the rate (84.53% vs 25.33%) and the grading (mean 2.59 vs mean 0.45) of anomalies of dural sinuses were significantly higher in case group than in control group. In the case group, sinus anomalies were asymmetric in 59 cases (right-left ratio 1/1) and symmetric in 22. A significant association was detected between the grading of venous drainage alterations and diagnosis of disease and between the severity of vascular anomalies and the widening of subarachnoid space (SAS). Postural plagiocephaly (39.1% vs 21.3%) and asymmetric tentorial insertion (35.4% vs 17.3%) were significantly more frequent in the case group than in the control group. When sinus anomalies occurred in plagiocephalic children, the obstruction grading was significantly higher on the flattened side (p ≤ 0.001). CONCLUSION: Decreased patency of the dural sinuses and consequent increased venous outflow resistance may play a role in the pathophysiology of external hydrocephalus in the first 3 years of life. In plagiocephalic children, calvarial flattening may impact on the homolateral dural sinus patency, with a possible effect on the anatomy of dural sinuses and venous drainage in the first months of life.

Endoscopic ultrasonic aspiration of brain abscess.

BACKGROUND: Intracerebral abscess is a rare, but very serious complication of neonatal sepsis. Aggressive medical and surgical strategy is warranted in patients with large abscesses. CASE REPORT: A neonate with bilateral large frontal abscesses, caused by Serratia marcescens, was operated using a neuroendoscope coupled with an ultrasonic aspiration device. To our knowledge, this is the first report of such utilization of this new tool. The left abscess was surgically drained through endoscopic aspiration using ultrasonic aspirator. Clinical condition rapidly improved, and postoperative MRI of the brain revealed an almost complete resolution of the left abscess. A second endoscopic procedure was performed 1 week later, and the right abscess was endoscopically drained with the same technique. The remaining clinical course was uneventful. DISCUSSION AND CONCLUSION: Ultrasonic aspiration is effective in obtaining complete removal of the purulent material and of the dense fibrin layer adherent to the capsule. However, further studies are warranted to determine its real effectiveness, compared with the classic technique. In our opinion, it should be considered an option in more difficult cases, such as abscesses caused by aggressive germs in young or immunocompromised patients, or in case of recurrent lesions, in order to avoid craniotomy and open surgery.

Contemporary occurrence of hydrocephalus and Chiari I malformation in sagittal craniosynostosis. Case report and review of the literature.

Chiari malformation type I (CM-I) and hydrocephalus are often associated with complex craniosynostosis. On the contrary, their simultaneous occurrence in monosutural synostosis is extremely rare. The pathophysiological hypothesis is that they may alter posterior fossa growth and lead to cerebellar tonsil herniation also without skull base primary involvement. Hydrocephalus is multifactorial and may be secondary to fourth ventricle outlet obstruction. The management of these cases is quite complex and not well defined. Cranial vault remodeling should be the only treatment when CM-I is asymptomatic and not related to syringomyelia. Suboccipital decompression should be reserved only in complicated CM-I, usually as a second surgical step following the correction of the supratentorial deformity. In our opinion, the associated hydrocephalus should be treated first in order to normalize intracranial hypertension before opening the cranial sutures. We report the case of a 26-month-old child that presented with sagittal craniosynostosis, hydrocephalus, and CM-I. He was managed by performing endoscopic third ventriculostomy (ETV) first and cranial vault remodeling thereafter. Clinico-radiological outcome was very satisfying. Concerning literature is reviewed; physiopathology and surgical management are discussed.

Cortical gluing and Ringer lactate solution inflation to avoid cortical mantle collapse and subdural fluid collections in pediatric neurosurgery: safety and feasibility.

INTRODUCTION: Subdural fluid collections following intraventricular and/or paraventricular procedures in pediatric neurosurgery are common and can be hard to treat. We describe our technique to close cortical defects by the aid of a fibrin adhesive and subsequent Ringer inflation with the aim to avoid cortical mantle collapse and to prevent the development of subdural fluid collections. MATERIALS AND METHODS: We report the preliminary results of a prospective study on a consecutive series of 29 children who underwent 37 transcortical or transcallosal surgical procedures since 2008 in our department. RESULTS: In 17 procedures, we performed a transcortical approach on lesions, and in other 19 operations, we operated by a transcallosal. In 5/17 transcortical approaches (29%) and in 3/20 transcallosal approaches (15%), we observed a 5-mm-thick subdural fluid collection of the 5 patients with subdural fluid collections in the transcortical group, 3 patients (17%) underwent surgery for symptomatic or progressive subdural fluid collections. Of the 3 patients in the transcallosal group, a subduro-peritoneal shunt was necessary only for 1 patient (5%). At the very end of the treatment (including chemotherapy and radiotherapy), it was possible to remove the subduro-peritoneal shunt in all these patients because of disappearance of the subdural fluid collections. CONCLUSION: In pediatric patients after transcortical or transcallosal procedures, the use of a fibrin adhesive to seal surgical opening and subsequent inflation of the residual cavity with Ringer lactate solution to avoid cortical mantle collapse seems safe and appears to prevent the development of subdural fluid collections.

Changes in venous drainage after posterior cranial vault distraction and foramen magnum decompression in syndromic craniosynostosis.

OBJECTIVE: The authors' objective was to measure the effect of posterior cranial vault distraction (PCVD) plus foramen magnum decompression (FMD) on dural sinus volume and venous flow in patients with syndromic craniosynostosis. METHODS: The volumes of the sagittal, straight, transverse, and sigmoid sinuses of 5 consecutive patients with syndromic craniosynostosis who underwent PCVD+FMD were calculated in cubic centimeters with T2-weighted volumetric MRI sequences before surgery, immediately after surgery, and after the end of the distraction process. Tridimensional reconstructions of phase-contrast magnetic resonance angiography (PC-MRA) images were obtained with multiplanar reconstruction (MPR). RESULTS: The average total volume of all dural sinuses increased immediately after surgery (from 10.06 cm3 to 12.64 cm3) and continued to increase throughout the 30-day distraction period (from 12.64 cm3 to 14.71 cm3) (p = 0.04), except that the right sigmoid sinus remained stable after the initial increase. The most important increases were observed for the left transverse sinus (+113.2%), right transverse sinus (+104.3%), left sigmoid sinus (+91.3%), and sagittal sinus (+41.8%). Less important modifications were evident for the right sigmoid sinus (+33.7%) and straight sinus (+23.4%). Significant improvements in venous flow were noted on the tridimensional reconstructions of the PC-MRA images. Venous obstruction grading score improved in 4 patients (average [range] 2.4 [ 2-5]) (p = 0.023) and remained stable in 1 patient. All patients had chronic tonsillar herniation (CTH) (mean [range] 16.6 [8-26] mm), and 3 had syringomyelia. CTH showed improvement on the last follow-up MRI evaluation in 4 patients (mean [range] 10.5 [0-25] mm) and worsened from 15 mm to 19 mm in 1 patient. Syringomyelia improved in 2 patients and remained unchanged in 1. CONCLUSIONS: This study has provided the first radiological evidence of the impact of craniofacial surgery on dural sinus anatomy and venous drainage. The venous anomalies described in patients with syndromic craniosynostosis are not static, and PCVD+FMD triggers a dynamic process that can lead to significant modifications of intracranial venous drainage. The traction exerted by the distracted bone flap onto the occipitoparietal dura mater adherent to the inner calvaria may account for the enlargement of the dural sinus throughout the distraction period. The impact of these modifications on venous pressure, intracranial pressure, CTH, and hydrocephalus remains to be determined.

Optic pathway ganglioglioma with intraventricular cyst.

Gangliogliomas originating in the optic pathway are rare, with less than 20 cases reported in the literature. Diffuse, bilateral involvement of the entire optico-chiasmatic pathway is exceptional. We report a case of suprasellar ganglioglioma that involved bilaterally the entire pregeniculate optic pathway. The patient presented with visual deficit, nystagmus, papilledema and acute biventricular hydrocephalus secondary to intraventricular cyst that required urgent surgery. Endoscopic fenestration of the tumoral cyst allowed control of hydrocephalus and decompression of the visual pathway. Through microsurgical procedure by pterional approach, partial removal of the tumor and histological diagnosis were accomplished 1 week later. The patient was managed with chemotherapy and radiation therapy. He presents stable residual disease at 4-year follow-up. Embryological origins, histological features, neuroradiological appearance, management and prognosis of optic pathway gangliogliomas are reviewed.

Hydrocephalus and spinal cord tumors: a review.

INTRODUCTION: Hydrocephalus secondary to intraspinal tumors is a well-known but rare condition since about 1% of patients with spinal cord tumors have various degrees of hydrocephalus at initial presentation. DISCUSSION: The mechanism of development of intracranial hypertension and hydrocephalus in patients with spinal cord tumor is not exactly known. The problematic aspects of this condition, with regard to clinical presentation and pathophysiology, are discussed and the relevant literature is reviewed. This uncommon association should always be kept in mind in the differential diagnosis of hydrocephalus of unknown etiology for three main reasons: the possibility of neurological deterioration if the patient is shunted prior tumor removal, the possibility to treat the hydrocephalus without shunting by simply removing the tumor, and the possible role of hydrocephalus as an early sign of intracranial metastasis in patients previously operated upon for removal of intramedullary gliomas. Due to the very slow evolution of the disease, a careful and close clinical and neuroradiological follow-up are essential for many years afterward. The presence of intracranial hypertension in a patient previously operated for a spinal tumor should be considered and investigated as an early sign of neoplastic intracranial seeding.

Hydrocephalus in aqueductal stenosis.

INTRODUCTION: The sylvian aqueduct is the most common site of intraventricular blockage of the cerebro-spinal fluid. Clinical aspects, neuroradiological appearance, and treatment of hydrocephalus secondary to aqueductal stenosis are specific. METHODS: An extensive literature review concerning etiologic, pathogenetic, clinical, and neuroradiological aspects has been performed. Therapeutic options, prognosis, and intellectual outcome are also reviewed. CONCLUSION: The correct interpretation of the modern neuroradiologic techniques may help in selecting adequate treatment between the two main options (third ventriculostomy or shunting). In the last decades, endoscopic third ventriculostomy has become the first-line treatment of aqueductal stenosis; however, some issues, such as the cause of failures in well selected patients, long-term outcome in infant treated with ETV, and effect of persistent ventriculomegaly on neuropsychological developmental, remain unanswered.

Hydrocephalus in Dandy-Walker malformation.

INTRODUCTION: Even if the first description of Dandy-Walker dates back 1887, difficulty in the establishment of correct diagnosis, especially concerning differential diagnosis with other types of posterior fossa CSF collection, still persists. Further confusion is added by the inclusion, in some classification, of different malformations with different prognosis and therapeutic strategy under the same label of "Dandy-Walker". METHODS: An extensive literature review concerning embryologic, etiologic, pathogenetic, clinical and neuroradiological aspects has been performed. Therapeutic options, prognosis and intellectual outcome are also reviewed. CONCLUSION: The correct interpretation of the modern neuroradiologic techniques, including CSF flow MR imaging, may help in identifying a "real" Dandy-Walker malformation. Among therapeutical strategies, single shunting (ventriculo-peritoneal or cyst-peritoneal shunts) appears effective in the control of both ventricle and cyst size. Endoscopic third ventriculostomy may be considered an acceptable alternative, especially in older children, with the aim to reduce the shunt-related problems. Prognosis and intellectual outcome mostly depend on the presence of associated malformations, the degree of vermian malformation and the adequate control of hydrocephalus.

A novel MEIS2 mutation explains the complex phenotype in a boy with a typical NF1 microdeletion syndrome.

Concurrence of distinct genetic conditions in the same patient is not rare. Several cases involving neurofibromatosis type 1 (NF1) have recently been reported, indicating the need for more extensive molecular analysis when phenotypic features cannot be explained by a single gene mutation. Here, we describe the clinical presentation of a boy with a typical NF1 microdeletion syndrome complicated by cleft palate and other dysmorphic features, hypoplasia of corpus callosum, and partial bicoronal craniosynostosis caused by a novel 2bp deletion in exon 2 of Meis homeobox 2 gene (MEIS2) inherited from the mildly affected father. This is only the second case of an inherited MEIS2 intragenic mutation reported to date. MEIS2 is known to be associated with cleft palate, intellectual disability, heart defects, and dysmorphic features. Our clinical report suggests that this gene may also have a role in cranial morphogenesis in humans, as previously observed in animal models.

Brain Herniation into the Subdural Space: Rare Iatrogenic Complication of Treatment of a Giant Calcified Subdural Hematoma.

BACKGROUND: Chronic subdural hematoma associated with dural calcifications in previously pediatric shunted patients is a rare condition. The inner dural membrane opening can lead to progressive brain herniation into the subdural space due to brain reexpansion. CASE DESCRIPTION: A 15-year-old boy, previously shunted at birth for congenital hydrocephalus, presented with a giant chronic right hemispheric subdural hematoma. After 2 surgical procedures in which the subdural calcified neomembrane was opened and a subdural shunt was implanted, he developed a cortical brain herniation into the subdural space, resulting in brain ischemia and upper limb weakness and vomiting. The final surgical treatment consisted of an extensive wide peeling of visceral calcified membrane mutually to programmable valve placement along the subduroperitoneal shunt, in order to create a positive gradient between the subdural space and the ventricles. The patient experienced a prompt clinical improvement. CONCLUSIONS: This case illustrates a rare complication of the treatment of a chronic subdural hematoma caused by insufficient opening of the calcified inner mambrane of the hematoma and encouraged by gradient pressure between the ventricular and subdural compartments. To avoid this complication, it is preferable to not open a thick, calcified, unelastic inner membrane. However, in case of lack of clinical and radiologic improvement, it may become necessary to open it: a wide opening should be attempted, in order to prevent brain strangulation.

The value of the maxillo-malar osteotomy in the treatment of Crouzon syndrome with exorbitism.

Premature fusion of the cranial sutures along with midface hypoplasia, shallow orbits, and ocular proptosis are the principal features of Crouzon syndrome. Treatment varies according to the variable expressivity of the disease and usually begins during a child's first year with fronto-orbital advancement with cranial decompression. Subsequent development of midfacial hypoplasia needs correction. Procedures for this purpose include the Le Fort III osteotomy or its segmental variants, monobloc frontofacial advancement, or bipartition osteotomy. Adult Crouzon often presents with marked midface hypoplasia and exorbitism. We report an adult-diagnosed Crouzon syndrome case with typical facial features and exorbitism corrected by orbital decompression and zygomaticomaxillary advancement.

Surgical treatment of thalamic tumors in children.

OBJECTIVE In the past, the outcome of surgical treatment for thalamic tumor was poor. These lesions were often considered inoperable. However, contemporary microsurgical techniques, together with improvements in neuroimaging that enable accurate presurgical planning, allow resection to be accomplished in a safer way. METHODS The medical records, imaging studies, and operative and pathology reports obtained for pediatric patients who were treated for thalamic tumors at the authors' department were reviewed. Neuronavigation and intraoperative monitoring of motor and somatosensory evoked potentials were used. Preoperative tractography, which helped to identify internal capsule fibers, was very important in selecting the surgical strategy. Postoperatively, an MRI study performed within 24 hours was used to assess the extent of tumor resection as partial (≤ 90%), subtotal (> 90%), or gross total (no residual tumor). RESULTS Since 2002, 27 children with thalamic tumors have been treated at the authors' department. There were 9 patients with unilateral thalamic tumors, 16 with thalamopeduncular tumors, and 2 with a bilateral tumor. These last 2 patients underwent endoscopic biopsy and implantation of a ventriculoperitoneal shunt. Thirty-nine tumor debulking procedures were performed in the remaining 25 patients. Different surgical approaches were chosen according to tumor location and displacement of the posterior limb of the internal capsule (as studied on axial T2-weighted MRI) and corticospinal tract (as studied on diffusion tensor imaging with tractography, after it became available). In 12 cases, multiple procedures were performed; in 7 cases, these were done as part of a planned multistage resection. In the remaining 5 cases, the second procedure was necessary because of late recurrence or regrowth of residual tumor. At the end of the surgical phase, of 25 patients, 15 (60%) achieved a gross-total resection, 4 (16%) achieved a subtotal resection, and 6 (24%) achieved a partial resection. Eighteen patients harbored low-grade tumors in our series. In this group, the mean follow-up was 45 months (range 4-132 months). At the end of follow-up, 1 patient was dead, 12 patients were alive with no evidence of disease, 4 patients were alive with stable disease, and 1 was lost to follow-up. All patients were independent in their daily lives. The outcome of high-grade tumors in 9 patients was very poor: 2 patients died immediately after surgery, 6 died of progressive disease, and 1 was alive with residual disease at the time of this report. CONCLUSIONS This institutional review seems to offer further evidence in favor of attempts at radical resection in pediatric patients harboring unilateral thalamic or thalamopeduncular tumors. In low-grade gliomas, radical resection in a single or staged procedure can be curative without complementary treatment. Recurrences or residual regrowth can be safely managed surgically. In high-grade tumors, the role of and opportunity for radical or partial resection remains a matter of debate.

Neuroendoscopic treatment of multiloculated hydrocephalus in children.

OBJECT: The authors performed a retrospective analysis of data obtained in a series of 30 patients suffering from multiloculated hydrocephalus and treated endoscopically. The goal of the study was to evaluate the effectiveness of neuroendoscopic treatment as an alternative to the placement of multiple shunts to relieve intracranial hypertension, to simplify the shunt system, and to reduce the high rate of shunt revision. METHODS: The endoscopic procedures included intraventricular septum fenestration, aqueductoplasty, Monro foraminoplasty, and third ventriculostomy. The patients were divided into two groups: Group A included 23 children in whom a shunt was already in place at the time of endoscopy, and Group B included seven children in whom a shunt had never been placed. The control of hydrocephalus by a single shunt placement or the absence of a shunt was achieved in 25 (83.3%) of 30 children. In Group A, five children no longer had shunts, 14 needed a single shunt, three required two shunts, and one required three shunts. The mean preendoscopy shunt revision rate in this group decreased from 2.07/year to 0.35/ year following the endoscopic procedure. Seven patients required endoscopic reoperations (endoscopic reoperation rate 0.31/year, total reoperation rate [shunt revisions plus endoscopic reoperation] 0.66/year). In Group B, three children did not require shunts, three needed a single shunt, and one required two shunts. Two patients required repeated endoscopic surgery (endoscopic reoperation rate 0.19/year), and two patients required shunt revisions (shunt revision rate 0.07/year) (total operation rate 0.26/year). CONCLUSIONS: Neuroendoscopic procedures are a valid alternative to shunt revision in the management of multiloculated hydrocephalus. Early diagnosis comprising close monitoring with high-resolution magnetic resonance imaging and early treatment are the keys of success.

Initial experience with endoscopic ultrasonic aspirator in purely neuroendoscopic removal of intraventricular tumors.

OBJECTIVE Neuroendoscopic removal of intraventricular tumors is difficult and time consuming because of the lack of an effective decompression system that can be used through the working channel of the endoscope. The authors report on the utilization of an endoscopic ultrasonic aspirator in the resection of intraventricular tumors. METHODS Twelve pediatric patients (10 male, 2 female), ages 1-15 years old, underwent surgery via a purely endoscopic approach using a Gaab rigid endoscope and endoscopic ultrasonic aspirator. Two patients presented with intraventricular metastases from high-grade tumors (medulloblastoma, atypical teratoid rhabdoid tumor), 2 with subependymal giant cell astrocytomas (associated with tuberous sclerosis), 2 with low-grade intraparaventricular tumors, 4 with suprasellar tumors (2 craniopharyngiomas and 2 optic pathway gliomas), and 2 with pineal tumors (1 immature teratoma, 1 pineal anlage tumor). Hydrocephalus was present in 5 cases. In all patients, the endoscopic trajectory and ventricular access were guided by electromagnetic neuronavigation. Nine patients underwent surgery via a precoronal bur hole while supine. In 2 cases, surgery was performed through a frontal bur hole at the level of the hairline. One patient underwent surgery via a posterior parietal approach to the trigone while in a lateral position. The endoscopic technique consisted of visualization of the tumor, ventricular washing to dilate the ventricles and to control bleeding, obtaining a tumor specimen with biopsy forceps, and ultrasonic aspiration of the tumor. Bleeding was controlled with irrigation, monopolar coagulation, and a thulium laser. RESULTS In 7 cases, the resection was total or near total (more than 90% of lesion removed). In 5 cases, the resection was partial. Histological evaluation of the collected material (withdrawn using biopsy forceps and aspirated with an ultrasonic aspirator) was diagnostic in all cases. The duration of surgery ranged from 30 to 120 minutes. One case was complicated by subdural hygroma requiring a subduro-peritoneal shunt implant. CONCLUSIONS In this preliminary series, endoscopic ultrasonic aspiration proved to be a safe and reliable method for achieving extensive decompression or complete removal in the management of intra- and/or paraventricular lesions in pediatric patients.

Giant melanotic neuroectodermal tumor of infancy (melanotic progonoma) of the head and neck: report of a malignant case.

Melanotic neuroectodermal tumor of infancy is a rare congenital pigmented neoplasm of neural crest origin, locally aggressive, growing rapidly and developing during the 1st year of life. It most commonly arises from the maxilla, cranial vault, and mandible. Occasionally, it exhibits malignant behavior with local lymph nodes involvement. Cases misdiagnosed and left untreated for a long time can present challenges due to the tumor mass and infiltration. In these cases, adjuvant chemotherapy can be extremely helpful before radical excision. Authors of this report describe a 4-year-old boy from a developing country who was referred to their hospital with an ulcerated bulging lesion in the midline/right parietooccipital region, extending to the right laterocervical and parotid regions, resulting in significant craniofacial deformation. Magnetic resonance imaging of the brain revealed a highly enhancing tumor with intracranial and extracranial development extending mainly at the level of the right parietooccipital region, with lytic and hypertrophic alterations of the skull. The patient was managed with neoadjuvant and adjuvant chemotherapy and radically resective surgery on metastatic lymph nodes and the primary tumor of the skull. Scheduled radiotherapy was not performed, according to the parents' wishes. The patient returned to his native country where the lesion recurred, and he ultimately died approximately 10 months after the end of the treatment. The literature indicates that tumor removal alone has been the treatment of choice in most isolated cases, but in cases of highly advanced tumor with involvement of the skull and cervical lymph nodes, it is preferable to proceed with preoperative chemotherapy with the aim of reducing the tumor volume, allowing better technical conditions for complete surgical removal, and decreasing the risk of local recurrence or metastasis.