Spinal cord tumors in children: A review of 21 cases treated at the same institution.

INTRODUCTION: Spinal cord tumors in children (SCTC) are rare with a frequent diagnostic delay. Its management is multidisciplinary and challenging due to functional implications. The position of surgery is now better established but the role and timing of chemotherapy (CT) and radiotherapy (RT) still remains under debate. Adverse effects of treatments are important to be taken into account, in the follow-up of these children. The aim of this paper was to present a series of 21 cases of SCTC treated at the same institution, to briefly present clinical features, treatments and outcome, with a special focus on spinal deformities in children with this condition. MATERIAL AND METHODS: Twenty-one consecutive SCTC were referred to our institution from 1990 to 2014. Data regarding age, sex, diagnostic delay, clinical examination, MRI, surgery, pathology, other treatment (CT and RT), orthopedic issues and follow-up of these children were retrospectively recorded. RESULTS: Mean age was 8years (standard deviation: 5.2years) (range: 4 months-17years). Mean diagnosis delay was 5.5 months (standard deviation: 6.5 months) (range: 0 days-18 months). All children (10 girls, 11 boys) were operated on (10 partial removals, 7 subtotal and 4 gross total removals) as first-line treatment. Pathological results showed 12 juvenile pilocytic astrocytomas, 1 grade III astrocytoma, 1 grade IV astrocytoma, 3 oligodendrogliomas, 2 ependymomas, 1 glioblastoma and 1 rhabdoid tumor. Fourteen children (66.7%) received additional treatment: 12 CT and 7 RT. Ten children had postoperative spinal deformities. Mean follow-up (FU) was 71 months (5 months-180 months), with a median FU at 60 months, where 8 tumor progressions and 4 deaths were observed. Overall, survival (at 5years) was 81% and progression free survival (at 5years) was 67%. CONCLUSION: Surgery is the goal standard for SCTC and the only appropriate treatment in cases of a low-grade lesion with stable disease on MR follow-up. Additional treatment must be reserved for high-grade lesions or tumor progression not attainable by a second look surgery. Spinal deformities are a frequent complication. Overall, survival and event free survival primarily depends on the pathology. Studies involving more centers are obligatory with the aim of collecting more cases and drawing more definitive conclusions regarding the management of these tumors.

[Nasal dermal sinus in children: a review based on a series of 6 cases]. / Fistules du dorsum nasal de l'enfant: mise au point à propos d'une série de 6 cas.

INTRODUCTION: Nasal dermal sinus in children (NDSC) is a rare malformation (1/20,000 to 1/40,000). Apart from local infection, they present as median nasal lump or pit on the dorsum and their diagnosis and treatment are often delayed. Consequences of untreated NDSC are: local infection, meningitis, and empyema, due to their frequent intracranial extension. PATIENTS AND METHODS: Six cases of NDSC were retrospectively reviewed (5 boys and one girl), all treated between 2006 and 2012 in our institution (Nancy University Hospital). All patients underwent a brain and facial CT-scan and MR imaging to check any bony lesions, skull base extension by foramen cæcum, course of the sinus and the possible associated brain malformations. Evolution, treatment and follow-up (FU) were conducted by the same multidisciplinary team (neurosurgeon, ENT surgeon, and plastic surgeon). Children were operated on by a conjoined approach (cranial and facial) for removal of the sinus and its intracranial extension. RESULTS: Mean age at diagnosis was 12 months (birth-36 months). Initial presentation consisted of three local infections, one dorsum nasal lump, one CSF leakage, and one asymptomatic child. Five children presented with a skull base extension. There were no associated brain malformations. We observed only one surgical complication (bleeding from the anterior part of the superior sagittal sinus during dissection) leading to blood transfusion. Pathology results confirmed three dermoid cysts, one epidermoid cyst, one cyst with granulation tissue, and negative in one case. Average FU was 30.8 months (4-84 months). Two recurrences (same child) occurred, leading to two re-operations. There were no recurrences or complications at the end of FU. CONCLUSION: NDSC are rare malformations, mostly diagnosed before the age of three years, due to an infectious complication. The aim of the treatment is complete removal to avoid recurrence, and a multidisciplinary strategy is required.

Suprasellar arachnoid cyst after subdural haemorrhage in an infant. A case based update.

INTRODUCTION: Brain arachnoid cysts (AC) are congenital or acquired malformations. Their prevalence in children ranges between 0.2 and 2.3% of the studied populations. Few reported studies exist where AC appears after a subdural haemorrhage. METHODS: We present one case of a symptomatic suprasellar AC after post-traumatic subdural haemorrhage in an infant. RESULTS: After endoscopic ventriculocystostomy, the child quickly improved and the cyst reduced in size. The child was monitored for 22 months and his neurocognitive development remained normal. CONCLUSION: Our case led us to the hypothesis that the inflammatory process due to subdural haemorrhage may locally result in arachnoiditis, and thus to the creation of a neomembrane, and eventually to cyst formation. This is also the case with the development of post-traumatic spinal AC.

[Orbital tumors in children: clinical examination, imaging, specific progression]. / Les tumeurs d'orbite de l'enfant : examen clinique, paraclinique, diagnostic et particularités évolutives.

"Orbital tumors (OT) are neoplasms of the bony orbit and contents except for the eyeball." Given this definition, we exclude the retinoblastomas, which are the most frequent tumors of this anatomical area in the pediatric population. Although these tumors are rare, there is a great variety of etiologies. Among them, the most frequent OTs in childhood are rhabdomyosarcomas and metastatic tumors: metastatic neuroblastomas and leukemias (chloromas). Moreover, adult and pediatric varieties of OT are made up of two clusters of distinctive histological entities. Examination must be careful: measurement, direction of proptosis, impairment of ocular motility, compressive optic neuropathy, strabismus, etc. Age at onset as well as unilateral or bilateral proptosis are important features. Benign tumors such as dermoid cysts or hemangiomas grow slowly, whereas rapid growth suggests a malignant tumor. Fundi and visual acuity should be checked. The general examination focuses especially on cutaneous abnormalities such as café au lait spots and subcutaneous nodules in neurofibromatosis type 1 (NF1), a hemangioma that often involves the cutaneous portion of the eyelid. When a metastatic tumor is suspected, abdominal palpation and ultrasonography must be performed. Ultrasonography leaves the posterior third of the orbit inaccessible to exploration. The depth to which it can penetrate the orbit is limited (20mm). Most of the time, standard x-rays, CT, and MR imaging allow for an adequate assessment and usually provide a diagnosis. In children, CT and MRI sometimes require anesthesia to be properly performed.

[Management of benign intracranial hypertension: analysis of the Nancy series]. / Prise en charge de l'hypertension intracrânienne bénigne: analyse de la série nancéenne.

INTRODUCTION: Benign intracranial hypertension (BIH) is a rare condition, especially in childhood. The aim of this study was to analyze retrospectively pediatric cases that were diagnosed and managed in the same institution during the 2002-2006 period. PATIENTS AND METHODS: Eight children (four girls and four boys) with a diagnosis of BIH were reviewed. The mean age at diagnosis was 10.5 years. Follow-up lasted a mean two years. The clinical features were those of intracranial hypertension. One child had previously had optic nerve sheath fenestration and another one occipitocervical decompression because of an associated Chiari I malformation. RESULTS: One child had a bilateral transverse sinus stenosis on angio-MRI. Seven children had a cerebrospinal fluid (CSF) pressure monitoring. Seven children were treated with acetazolamide. Three children are free of symptoms with the association of acetazolamide and depletive lumbar puncture (LP). For three others, a lumboperitoneal shunt had to be inserted. One child is in complete remission after depletive LP only. The clinical symptoms of BIH disappeared for all eight children, including normalization of the visual loss present in three children. CONCLUSION: BIH is a condition that threatens visual prognosis. Diagnosis is assessed by clinical, radiological, and raised CSF pressure criteria. First-line treatment is medical (acetazolamide at first intention) and surgery is recommended for refractory cases. The relationship between BIH and obesity is less clear than for adulthood. Depletion of CSF by LP is an important therapeutic factor.

[Germ-cell tumors of the central nervous system in childhood: retrospective study of 13 patients]. / Les tumeurs germinales du système nerveux central de l'enfant : étude rétrospective de 13 patients.

BACKGROUND AND PURPOSE: Germ cell tumors (GCT) of the central nervous system are rare (2% of all brain tumors in children). Although originating from germ cells, GCT cover a spectrum of different tumors with different management and prognosis, depending on whether they secrete tumor markers or not. The aim of this study is to present a series of children with GCT and comment on overall management practices. METHODS: We retrospectively reviewed 13 children under the age of 18 years (nine boys and four girls), treated in the same institution between 1986 and 2006 for one or more primitive GCT of the central nervous system. RESULTS: Median age at diagnosis is 12.9 years (7-17 years). Tumor markers (alpha foetoprotein [alphaFP], human chorionic gonadotrophin [betaHCG]) were assessed 11 times in blood as well as cerebrospinal fluid (CSF). Tumors were located as follows: pineal region (10 cases), hypothalamus (eight cases), basal ganglia (one case) and corpus callosum (one case). Six were bifocal (pineal region and hypothalamus). Clinical signs were mostly dominated by diabetes insipidus and intracranial hypertension. Seven children required surgery for hydrocephalus. Tumor markers were positive in three cases and these children subsequently received chemotherapy followed by radiotherapy, except one child. Eventually, the three patients with positive markers required surgery because of a residual lesion. The eight other patients had a stereotactic biopsy for diagnosis. At the end of follow-up, treatment morbidity appears to be low and neither death nor recurrence was observed. Mean follow-up is 8.85 years (2-20 years). CONCLUSIONS: The prognosis of cerebral GCTs in children is excellent because of their pronounced chemo- and radiosensitivity. Surgery is crucial for diagnosis in the event of negative markers, or if there is evidence of residual tumor with normalization of tumor markers at the end of chemotherapy. Tumor markers must be monitored to check the diagnosis and for follow-up. The place of tumor biopsy during endoscopic third ventriculostomy (performed if hydrocephalus is present) is still debated.

Intraventricular glioblastoma: a paediatric case report.

The authors report the case of a nine-year-old boy admitted with raised intracranial pressure in relation with a tumour of the right lateral ventricle. Complete removal of the lesion was achieved and pathological diagnosis was glioblastoma multiform. Adjuvant radio and chemotherapy was given, but the child died from a recurrence of the disease one year after diagnosis. In addition, last MRI demonstrated, distant from the initial tumor site, an extra-cerebral lesion that is supposed to be a glioma metastasis rather than a radio-induced tumour.

Germinoma located in the basal ganglia in an 8-year-old girl.

CASE REPORT: The authors describe a unique case of an 8-year-old girl with a germinoma located in the left basal ganglia. Medical history begins 5 months before with a central diabetes insipidus, loss of weight (5 kg at admission), vomiting and asthenia. Computed tomography (CT) and cranial magnetic resonance imaging (MRI) are performed and demonstrate a left basal ganglia tumour (nucleus lentiformis). Diabetes insipidus is considered as non-visible germinoma localization on the pituitary stalk rather than as a possible consequence of peri-tumoural oedema surrounding the hypothalamus. Spinal MRI is normal. Neurological as well as general examination is normal. DISCUSSION: The first hypothesis is low-grade glioma, but pathological examination following a stereotactic biopsy of the lesion reveals a cerebral germinoma. A few days before the biopsy, the girl experienced a mild left facial palsy, and CT scans at the time of biopsy reveals an intra-tumoural haemorrhage. Alpha fetoprotein and human chorionic gonadotrophin were negative as blood and cerebrospinal fluid markers, whereas placental alkaline phosphatase was positive on immunohistochemical profile of the tumour samples. Dedicated chemotherapy, followed by focal irradiation (40 Gy, 30 sessions, 45 days; SIOP CNS GCT 93 protocol), is performed with a complete response. The outcome is good (Glasgow Outcome Scale=I), without any cognitive impairment and the persistence of a mild facial palsy and a slight right arm dystonia on last neurological examination. There is still no evidence of tumour recurrence.

[Subdural empyema in children: therapeutic strategy. Five cases]. / Empyèmes sous-duraux de l'enfant: stratégie thérapeutique. A propos de cinq cas.

We report a series of five subdural empyema (SDE) in children and young adults treated in the same neurosurgical department. These five cases were reviewed retrospectively. There were four boys and one girl, aged from three months to 18 years at time of diagnosis (median age: 7 years). SDE following intracranial surgery were excluded from the study. All patients were treated surgically (burr hole evacuation or craniotomy, repeated in some cases), followed by intravenous antibiotic therapy (mean time: 52 days) adapted to the micro-organism. Only the two patients treated by large craniotomy at first had a single surgical procedure. Involved micro-organisms are as follow: Streptococcus intermedius (n=2), Streptococcus pneumoniae (n=1), Escherichia coli (n=1), absence of any identified micro-organism (n=1). The five patients are alive (median follow-up: 22 month) without any sequelae. We advocate an aggressive surgical treatment of SDE in children with a large bone flap to allow the surgeon to remove pus and membranes as much as possible, even in the interhemispheric fissure, followed by intravenous appropriate antibiotherapy and eradication of the source of infection. Even this "aggressive" treatment may sometimes not avoid re-operation. A careful follow-up is mandatory, because of the high risk of recurrence.

Neurosurgical concepts and approaches for orbital tumours.

Orbital tumours are lesions that appear within the orbital craniofacial borders. To this end, treatment of these tumours is assured by teams of different specialists. Furthermore, these pathologies are different in adults and in children. We have endeavoured, in this chapter, to highlight the specifically neurosurgical features of orbital tumours or, to be more precise, tumours affecting the posterior two thirds of the orbit and tumours originating in or intruding into the optic canal. The list of aetiologies is long. After recapitulating the main types of tumour (as well as those of most concern), we have also studied the different stages of surgery, namely approaches and reconstructions which we have illustrated at each stage by a tumour that, in our view, seemed emblematic of the problem in question: the lateral eyebrow approach for schwannoma and cavernous angioma, the transorbital subfrontal approach for optic nerve glioma, the pterional and orbital approaches for spheno-orbital meningioma, problems with reconstruction and with plexiform neurofibroma affecting the orbit and fibrous dysplasia of bone.

[Childhood diencephalic pilocytic astrocytoma. A review of seven observations]. / Les astrocytomes pilocytiques du diencephale de l'enfant. à propos de sept observations.

BACKGROUND AND PURPOSE: Pilocytic astrocytoma (PA) is a WHO grade I tumor of the central nervous system mostly arising in children and young adults. Management of diencephalic PA is a difficult challenge. Surgical treatment has to cope with both the difficulties of deep location and eloquent area tumors. MATERIALS AND METHODS: We retrospectively reviewed seven pediatric cases (female: 4, male: 3) of diencephalic PA. Opto-chiasmatic tumors were excluded from the series. Mean age at diagnosis was 108 months (9 years) (range: 4 month-18 years), median age was 111 months. Median follow-up for the series was 125 months. Tumor locations were as followed: right thalamus: 2, both thalami: 1, hypothalamus: 3, and right basal ganglia: 1. At the onset, the first symptom was mostly raised intracranial pressure. The delay in diagnosis ranged from 48 hours up to 6 years. TREATMENT: a shunting procedure was performed in 3 patients, a direct surgical approach in 5 patients (gross total removal: 2; partial removal: 3) and one patient had only a biopsy. Three children were re-operated. Three patients were treated by radiationtherapy (RT) after surgery. Chemotherapy was delivered for 4 children. RESULTS: The overall survival rate was 71.4 months (almost 6 years) (range: 3-184 months). Median survival rate was 42 months (3.5 years). Three children died, two by tumor progression and one death related to late side-effects of RT. Four patients have a good quality of life with GOS I (n = 3) or II (n = 1). We observed tumor regression in two patients at 1 and 17 years after the beginning of treatment. Correct diagnosis was only made for two cases at the initial pathological examination. CONCLUSION: The course of diencephalic PA is still unpredictable. The tumor can be controlled by a partial surgical removal, and a residual tumor can sometimes decrease in size after surgery. Gross total removal of these tumors, although difficult, may be performed. With cranial navigation systems, the risk is low. Pathological diagnosis is sometimes difficult to assess.

Individualized treatment of craniopharyngioma in children: ways and means.

BACKGROUND: Medium- and long-term prognosis of craniopharyngioma is overwhelmed by the risks of hypothalamic and visual impairment. This problem has been underestimated for a long time because the major concern for the neurosurgeon was the risk of recurrences, their best prevention being thought to be complete tumour resection. Today, we know that radical surgery not only is not an absolute guarantee against recurrences but also can cause hypothalamic and visual complications. METHODS: The authors suggest that instead of complete removal, the first choice treatment should be, when possible, a less aggressive, multistaged and personalized treatment. In this perspective they focus on other therapeutic methods: endocavity treatment of cysts with rhenium-186, triconformational radiotherapy, radiosurgery, and widespread use of the trans-sphenoidal approach. CONCLUSIONS: These simple methods should reduce the risks of visual aggravation and metabolic syndrome.

Thalamic ependymal cyst presenting with tremor.

CASE REPORT: We report the case of a 6-year-old boy presenting with intention tremor. CT and MR scanning showed a large thalamic cyst. TREATMENT: Stereotactic tapping of this cyst was carried out as the first step in surgical management. Following drainage of the cyst the tremor disappeared, but recurred 18 months later. Repeat MRI showed that the thalamic cyst had refilled. As the initial tapping of the cyst had resulted in effective but only transient clinical improvement a cysto-peritoneal shunting procedure was performed. OUTCOME: The clinical symptoms again disappeared and the child has now remained tremor free for 3 years.

[Methylation status of RARbeta gene promoter in low and high grade cerebral glioma. Comparison with normal tissue. Immuno-histochemical study of nuclear RARbeta expression in low and high grade cerebral glioma cells. Comparison with normal cells. 48 tumors]. / Statut de méthylation du promoteur du gène RARB dans les tumeurs cérébrales gliales de bas et de haut grade. Comparaison avec le tissu sain. Etude immuno-histochimique du marquage au RARbeta des noyaux des cellules tumorales dans les gliomes de bas et de haut grade. Comparaison avec les cellules saines, à propos de 48 tumeurs.

Retinoic acid receptor beta (RARbeta) is a nuclear receptor often deregulated in tumors. An immunohistochemical study was conducted to examine the level of expression of this receptor in the nucleus of glial cell tumors (low and high grade glioma) as well as a study of the methylation status of the gene promoter coding this receptor on the same tumor samples. A comparison with normal tissue was done each time. 48 tumors were eligible for the study (15 glioblastomas, 20 grade III oligodendrogliomas and 13 grade II oligodendrogliomas). A constant decrease of RARbeta expression was found by comparison with normal tissue whatever the histological grade of the tumor, suggesting a deregulation of RARbeta gene expression. Methylation of RARbeta promoter gene was a rare event (12.5% of all cases), except for grade III oligodendrogliomas (20%), and is thus not a major event of this gene deregulation. Other reasons of this deregulation of RARbeta should be studied, such as loss of 3p24 heterozygoty, mRNA studies and RARbeta interactions with other retinoid receptors.

[Pleomorphic xanthoastrocytoma. A review of five observations]. / Le xanthoastrocytome pléomorphe. A propos de cinq observations.

BACKGROUND AND PURPOSE: We present a review of five patients who developed pleomorphic xanthoastrocytomas, presenting the clinical features, pathology findings and therapeutic approach of these rare tumours which almost always occur in children and young adults. METHODS: This paper is a retrospective study of five patients (three males and two females), treated from 1985 to 1996. RESULTS: Seizure was the most frequent symptom. The tumor was located in the temporal or temporo-parietal lobe in two patients, in the parietal lobe in one, the frontal in one and in hypothalamus and third ventricle in the final patient. To our knowledge, no case of pleomorphic xanthoastrocytoma located in the area of hypothalamus/third ventricle has been reported in the literature. All patients underwent surgery which was complete for two patients, subtotal for two others and partial for one. Four were given post-operative cerebral radiation therapy (two recurrences and malignant transformation and two erroneous diagnoses) (mean dose: 44.75 Gy), for two patients surgery was followed by chemotherapy. One patient was lost for follow-up. Two patients presented at least one tumor recurrence. Two died from malignant transformation. Two patients are alive without recurrence at 12 and 16 years from diagnosis with excellent quality-of-life (Glasgow Outcome Scale=I). CONCLUSION: Surgery is the gold standard treatment. Prognosis is very good (except for malignant transformation), so that a close long-term clinical and radiological follow-up is mandatory.

Chemotherapy for unresectable and recurrent intramedullary glial tumours in children. Brain Tumours Subcommittee of the French Society of Paediatric Oncology (SFOP).

Adjuvant treatment for intramedullary tumours is based on radiotherapy. The place of chemotherapy in this setting has yet to be determined. Between May 1992 and January 1998, eight children with unresectable or recurrent intramedullary glioma were treated with the BB SFOP protocol (a 16-month chemotherapy regimen with carboplatin, procarbazine, vincristine, cyclophosphamide, etoposide and cisplatin). Six children had progressive disease following incomplete surgery and two had a post-operative relapse. Three patients had leptomeningeal dissemination at the outset of chemotherapy. Seven of the eight children responded clinically and radiologically, while one remained stable. At the end of the BB SFOP protocol four children were in radiological complete remission. After a median follow-up of 3 years from the beginning of chemotherapy, all the children but one (who died from another cause) are alive. Five patients remain progression-free, without radiotherapy, 59, 55, 40, 35 and 16 months after the beginning of chemotherapy. The efficacy of this chemotherapy in patients with intramedullary glial tumours calls for further trials in this setting, especially in young children and patients with metastases.

[Dysembryoplastic neuroepithelial tumors. Analysis of 16 cases]. / Tumeurs neuro-épithéliales dysembryoplasiques. Analyse de 16 observations.

OBJECTIVES: We report on 16 cases of dysembryoplastic Neuroepithelial tumor (DNT) treated in the Nancy University Hospital from 1987 to 1997. PATIENTS AND METHODS: There were 9 males and 7 females. Mean age at onset of symptoms was 9.5 years (range: 3 months to 29 years) and the mean age at surgery was 16 years. Nine patients experienced partial complex seizures, 5 patients generalized seizures, 1 patient partial seizures with secondary generalization and one patient ataxia. The diagnosis of DNT was made under consideration of clinical, radiological and neuropathological features. All patients underwent surgery. RESULTS: Removal of the tumor was complete for 10 patients, subtotal or partial for 6 patients. Histological examination revealed that 7 cases were specific forms of DNT due to the presence of the specific glioneuronal element. For the 9 remained cases, the diagnosis of DNT could only be made with the consideration of clinical and radiological features. Mean post-surgical follow-up was 3.5 years (range: 1-8 years). Eight patients were seizures-free, 7 had a significative reduction in seizures frequency with minimal anti-convulsivant treatment. The patient with ataxia remained unchanged. For the patients with partial resection of the tumor, follow-up MRI and CT scan showed no significant growth of the remnant and the remaining patients have had no recurrence to date. CONCLUSION: The recognition of this surgically curable entity is mandatory. Knowledge of the good prognosis associated with the DNT is essential to avoid deleterious side effects of overtreatment by radiotherapy and/or chemotherapy.

[Intrasellar epidermoid cysts]. / Les kystes épidermoïdes intrasellaires.

Two patients presenting with an intrasellar epidermoid cyst and operated on through a transsphenoidal approach, are reported. Problems regarding pathological controversies about the differential diagnosis (particularly craniopharyngiomas) are addressed. In order to establish the adequate preoperative diagnosis, the interest of MRI is discussed.