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Multiple extracranial meningiomas in a child

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Abstract

Meningioma is an uncommon tumour in childhood. We report the case of a child presenting with proptosis, which proved to be due to an optic nerve sheath meningioma. Subsequently, he complained of back pain and stiffness, and upon investigation this was also revealed to be due to a meningioma.

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Purpose To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM). Methods Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed. Results The mean age at presentation was 11 years (range: 6–17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71–297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up. Conclusions This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.
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Extradural Middle Fossa Approach to a Clear Cell Meningioma in a Child - Volume 36 Issue 2 - James King, Michael Cusimano, Cynthia Hawkins, Peter Dirks
Article
Meningioma is a common tumor of the central nervous system in adults, accounting for 10%-20% of all primary intracranial tumors. It is rare in children, with an incidence of 2.19% (0.85%-2.3%). Although there are numerous case reports in the literature, no firm conclusions can be drawn. For this report we accumulated, reviewed, and analyzed reports in the literature from 1960-93. A total of 318 patients with meningioma were managed in King Faisal Specialist Hospital and Research Centre from 1981-93. Nine of these patients (2.8%) were children aged 16 years or less. These cases were analyzed retrospectively with regard to age, sex, clinical presentation, radiologic finding, pathologic findings, management and outcome. Childhood meningioma represented 2.8% of all meningioma cases and 2.2% of all central nervous system tumors seen in children. There were six males and three females. The average age at presentation was 10.1 years (range 1-16 years). There were four cases of meningioma in the orbit an one each in the temporal region, foramen magnum, tentorial region, subfrontal base, sellar region and ethmoidal air sinus. There were two cases of multiple meningioma. Meningothelial meningioma was the type most frequently seen. Meningioma is rare in children and males are affected more than females. Tumor locations that are rare in adults are more common in children. The meningothelial type is most frequently seen. Prognosis is poor compared with that in adults, as the tumors tend to grow more rapidly and to a larger size, undergo malignant changes, and have a greater rate of recurrence. Whenever feasible, every effort should be made to achieve total resection of the tumor at the first attempt. If radiation therapy is used in children it should be limited to those who have recurrent tumors so as to minimize organic and psychologic complications.
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The objective of this work was to gain more insight into the controversial characteristics of meningiomas occurring during childhood and adolescence. Management of meningiomas is an important field in pediatric neurosurgery. Every pediatric neurosurgeon has tried to resolve the problems relating to the clinical characteristics, biological behavior and outcome of this interesting and almost benign pathology, which rarely occurs in the first two decades of life. The records on central nervous system (CNS) tumors held by the two major neurosurgery centers of Tehran Medical University and Arad General Hospital were prospectively collected during last 15 years. Complete medical records are available for all 24 cases, and long-term follow-up was achieved 19 patients. All the cases were diagnosed and treated after the introduction of computed tomographic (CT) scanning. Angiography and magnetic resonance imaging (MRI) were performed as complementary studies in some cases. The sample consisted of 13 girls and 11 boys. The age range at the time of diagnosis varied between 2 and 17 years, with a mean of 9.47 and standard deviation of 3.43. Fifteen patients were below 10 years of age (62.5%), and 9 of them were between 10 and 17 years old (37.5%). The most common presenting symptoms, in declining order of frequency, were headache, epilepsy and focal neurological deficits. Similar cases associated with neurofibromatosis either at the time of presentation with meningioma or during the follow-up period were excluded (5 cases). The size of the presenting tumor was more than 5 cm in diameter in 17 cases. The locations of the lesions, taken as the site of the presumed widest dural base in each case were: spinal, orbital, ethmoidal and sphenoethmoidal in 1 case each, petroclival in 2, and tentorial or supratentorial in 18 patients. The only predisposing factor in this series of childhood meningiomas was whole-axis irradiation for previous malignancy in the case presenting with cervical intradural meningioma. There have been no surgical deaths, and gross total excision of the lesions was achieved in 21 cases. Tumor recurrence was observed during the follow-up period in 6 cases (25%). The follow-up period varied between 2 and 165 months, with a median interval of 130.2 months. This series of pediatric CNS meningiomas comprises almost 1.08% of all meningiomas operated on by the authors during the last 15 years and it also accounts for about 1.1% of all pediatric CNS tumors encountered. This series of patients has certain characteristics regarding sex distribution, unusual size, peculiar localizations, special histological features and benign clinical behavior distinguishing it from other series reported in the literature.
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The authors add 9 cases of pediatric meningiomas to 112 previously published cases and review clinical findings. Characteristic clinical and radiologic features are discussed in an attempt to identify settings where the diagnosis of intracranial meningioma should be considered in a child. Preoperative evaluation and outcome are also reviewed.
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The authors have presented 81 children with intraspinal tumors, all less than 16 years of age. The clinical features indicated that gait disturbance, pain, and sphincter disturbance are the most prominent complaints. Reflex changes, paralysis, and sensory impairment are the most frequent physical findings. Cerebrospinal fluid protein was recorded in 47 patients and was abnormal in 34. Spine radiography was abnormal in 58% of the patients. Seventy of the patients had myelography, and it was diagnostic in 67. Surgery was performed on 79 patients, with a 2.5% operative mortality (30 days postsurgery). Of the 49 patients who are alive, 91.5% are able to walk. Mean follow-up period was 8.5 years. The authors document the benign course of the intramedullary astrocytomas and suggest an approach to their treatment. They encourage an aggressive approach to metastatic intraspinal tumors. The iatrogenic origin of some epidermoid tumors is noted. The importance of early diagnosis and treatment of the intraspinal tumors in children is emphasized.
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Thirteen cases of meningiomas in the pediatric age group seen at the University of California are reviewed. Four of these patients are alive; one had complete surgical resection of tumor and three had subtotal resection followed by irradiation, with total tumor doses of 4500 to 5500 rads. On the basis of these data, together with our total experience in the treatment of 188 patients with meningiomas, we conclude that patients who have complete surgical resection need no further treatment but patients who have subtotal removal may have recurrences delayed or prevented by the addition of postoperative irradiation to their therapeutic regimens.
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We report the case of a 4-year, 5-month-old boy, who had unusual multiple meningiomas occurring simultaneously in the intracranial, intraorbital, and spinal spaces. All lesions were surgically excised and histologically proved. This is the youngest patient with multiple meningiomas without history of neurofibromatosis reported in the literature.
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The clinical presentation and pathological characteristics of 18 histologically verified meningiomas in the paediatric age group are reviewed. There was a 1:1 sex ratio. Two children presented with seizures. The majority were supratentorial in location and large in size. In 4 patients, the meningiomas showed sarcomatous changes, while in 6 patients they were cystic.
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Review of clinical, roentgenographic, and histologic material of 75 purported meningiomas in patients younger than 20 years old revealed 48 cases that we considered clearly to represent meningiomas. The meningiomas were characterized by posterior fossa location (19 per cent), intraventricular location (17 per cent), roentgenographic evidence of tumor calcification (32 per cent), and frequent association with neurofibromatosis (23 per cent). These findings are contrasted with those of the adult population.
Article
The clinical features and pathological materials of 51 cases of histologically verified meningiomas in patients under 21 years of age were reviewed. The age range was 7 to 20 years, with the majority of cases being clustered in the second decade of life. There was virtually a 1:1 sex ratio, with 26 females and 25 males. Thirty-eight patients had intracranial meningiomas, three had intraorbital tumors, and 10 had intraspinal tumors. Twelve patients (24%) had neurofibromatosis. Twenty patients (39%) had tumor recurrence. The cases were subdivided into five histological categories: meningotheliomatous, fibrous, transitional, psammomatous, and papillary. In each case, the clinical course was correlated with histological subtype. The 15-year survival rate in patients with intracranial meningiomas of all types was 68%. Factors adversely affecting survival included infratentorial location, papillary histology, and evidence of brain invasion.
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This is a report of a surgically proven meningioma en plaque of the cervical region in a 10-year-old girl.
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A case of a child with multiple meningiomas in separated neuroaxial compartments is reported. The first tumor was in the frontal region and was removed surgically when first diagnosed 8 years prior to the appearance of the other tumor. The second tumor was located in the foramen magnum region, and reached considerable dimensions before becoming symptomatic and giving signs of brain stem compression as evidenced by progressive spastic tetraparesis and respiratory disturbances, without hydrocephalus. The diagnosis was confirmed by a low CT scan section, the tumor was successfully removed with the aid of the microscope and the patient made a good recovery.
Article
The authors add 9 cases of pediatric meningiomas to 112 previously published cases and review clinical findings. Characteristic clinical and radiologic features are discussed in an attempt to identify settings where the diagnosis of intracranial meningioma should be considered in a child. Preoperative evaluation and outcome are also reviewed.
The treatment of meningiomas in childhood
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