Clinical aspects and long-term prognosis of infratentorial intracranial tumours in infancy and childhood

An unselected series of 185 children with intracranial tumours in the posterior fossa, aged from birth up to and including 14 years, was followed from 1 to 40 years after diagnosis. Follow-up was 100%. There were 102 boys and 83 girls, i.e. a male/female ratio of 1.22. the same sex ratio was found in all tumour localisations and in most of the histological groups, with the exception of ependymoma, where it was 2.0. All children with tumours in the fourth ventricle or in the cerebellar hemispheres had signs of increased intracranial pressure on admission to hospital, and the duration of these symptoms was less than 6 months in 77% of the children. 113 patients survived for more than 1 month after surgery or diagnosis, and 46 (41%) for at least 15 years. All 34 children with tumours in the brain stem died within the first 2 years of observation, apart from three who survived 4, 5, and 10 years. All but one of the children with medulloblastoma, and all but two with ependymoma died within 1–17 years. Five children with astrocytoma suffered tumour recurrence more than 10 years after primary surgery. One with astrocytoma in the fourth ventricle and 36 with cerebellar astrocytoma survived for at least 15 years. In the survivors the tumour was located in the fourth ventricle in three, in the cerebello-pontine angle in one, and in either the vermis or the cerebellar hemispheres in 42. 41 of these 46 survivors lead a normal life.     

儿童小脑毛细胞型星形细胞瘤的诊治(附15例报告)

目的总结儿童小脑毛细胞型星形细胞瘤的临床资料，探讨其诊治特点。方法分析儿童小脑毛细胞型星形细胞瘤15例的影像学、病理学特点及术中所见和预后情况。结果肿瘤全切除13例，次全切或大部切除2例。术后发热2例，均于术后3d内消失；头痛2例，随病情好转而逐渐消失。余病例未出现新的症状和体征。术后随访未见到复发。结论儿童小脑毛细胞型星形细胞瘤有其独特的临床特点，在条件允许的情况下应尽可能切除肿瘤。     

Sub-classification of low-grade cerebellar astrocytoma: is it clinically meaningful?

OBJECTIVES: The objectives were to identify prognostic factors for the survival of children with cerebellar astrocytoma, and to evaluate the reproducibility and prognostic value of histological sub-classification and grading. METHODS: Children aged 0-14 years treated in Denmark for a cerebellar astrocytoma in the period 1960-1984 were included and followed until January 2001 or until their death. The histological specimens from each patient were reviewed for revised grading and classification according to three different classification schemes: the WHO, the Kernohan and the Daumas-Duport grading systems. RESULTS: The overall survival rate was 81% after a follow-up time of 15-40 years. The significant positive prognostic factors for survival were "surgically gross-total removal" of the tumour at surgery and location of the tumour in the cerebellum proper as opposed to location in the fourth ventricle. No difference in survival time was demonstrated when we compared pilocytic astrocytoma and fibrillary astrocytoma. Moreover, we found that the Kernohan and the WHO classification systems had no predictive value and that the Daumas-Duport system is unsuitable as a prognostic tool for low-grade posterior fossa astrocytomas. CONCLUSION: Discordant observations due to interobserver variability make histological sub-classification of low-grade cerebellar astrocytomas in children insufficient for predicting prognosis and biological behaviour. Similar survival rates in a population of paediatric low-grade cerebellar astrocytomas of grades I and II indicate that tumour grade has no prognostic significance within this group of patients. "Surgically gross-total removal", especially if the tumour is located in the fourth ventricle is of the highest importance for long-term survival. Histological sub-classification of the tumours has no predictive value.     

Cognitive and affective disturbances in children after surgical treatment of cerebellar tumors

BACKGROUND AND PURPOSE: The cerebellum has been associated with motor control, but more recent studies have extended its contribution to other functions, such as modulation of emotions, behavioral organization or language. The cerebellar cognitive affective syndrome was described primarily in adults. In children, cerebellar lesions are relatively frequent and some are due to neoplasm. MATERIAL AND METHODS: Cognitive and emotional functions were studied in 66 children who underwent surgery for cerebellar pilocytic astrocytoma at the Department of Neurosurgery of the Children Memorial Health Institute. 36 children were psychologically examined and parents of 66 children filled in a questionnaire, answering questions about children's linguistic functioning, emotion regulation and ability to initiate, organize and efficiently realize various activities. RESULTS: The disturbances in the initiation and realization of activities were reported most often (77%). Problems in emotional regulation were noticed in 65% of children and were characterized by disinhibition, impulsivity and irritability. Language difficulties were observed in 42%. No significant differences between groups of children with different localization of tumor (vermis or cerebellar hemisphere) were observed. CONCLUSIONS: The present study confirms the psychological deficits following cerebellar lesion in children, similar to the cerebellar cognitive affective syndrome reported in adult patients.     

Cerebellar astrocytomas: a 24-year experience

INTRODUCTION: Cerebellar astrocytomas are the most benign tumors of the CNS. Seventy to eighty percent are found in children. METHODS AND RESULTS: We report on 38 children under 18 who had cerebellar astrocytoma in the posterior fossa and were treated by a multidisciplinary team in our Neurosurgical Department from January 1974 to December 1997. We included all patients in whom the histopathological diagnosis was astrocytoma, regardless of malignancy. The diagnostic methods used were pneumoventriculography, cranial X-rays, CT scan, and MRI. All patients were treated surgically. Neither radiotherapy nor chemotherapy was indicated in patients with pilocytic or fibrillary astrocytomas. A greater prevalence was observed in female (25/38; 66%) than in male (13/38; 34%) patients. Histopathological results revealed 27 (71%) pilocytic astrocytomas, 8 (21%) diffuse fibrillary astrocytomas, 1 (2%) anaplastic astrocytoma and 2 (6%) glioblastomas. These tumors were more frequently located in the right cerebellar hemisphere; increased intracranial pressure syndrome was the most frequent form of clinical presentation. Total tumor resection was obtained in 29 (83%) cases and subtotal resection in 9 (17%). In 6 (16%) cases, ventriculoperitoneal shunts were placed to control persistent hydrocephalus after tumor excision. CONCLUSION: The most frequent complication was increased ataxia. The mortality rate was 8.5%.     

Follow-up and quality of survival of 67 consecutive children with CNS tumors

We report the finding at follow-up in 67 consecutive children with central nervous system tumors treated over a 5-year-period at a single institution. The diagnoses were supratentorial astrocytoma (n = 12), cerebellar astrocytoma (n = 10), ependymoma (n = 9), medulloblastoma (n = 9), brain stem glioma (n = 6), optic pathway glioma (n = 5), and others (n = 16). The survival rates were 83% for supratentorial astrocytomas at a median of 46.5 months, 90% for cerebellar astrocytomas and 55% for ependymomas at 40 months, respectively, 55% for medulloblastomas at 22 months, 33% for brain stem gliomas at 23 months, and 80% for optic pathway gliomas at 49 months. With regard to neurological sequelae, 13 patients were treated for epilepsy, 13 patients had mild to moderate neurological deficits, and 4 patients were severely disabled. Seventeen of 37 tested patients performed below average on formal neuropsychometric testing, one-fourth attended special education courses, and at least one-fourth suffered from behavioral and adjustment problems.     

Paediatric brain tumours treated at a single,tertiary paediatric neurosurgical referral centre from 1999 to 2010 in Australia

Paediatric brain tumours are the most common solid tumour of childhood and the most common cancer cause of death among children. A retrospective review of 313 histopathologically proven brain tumours over an 11-year period has been performed at the Children’s Hospital Westmead, New South Wales, Australia, to determine proportions and locations of different tumours, age distribution, survival rates and usage of various treatment modalities. Pilocytic astrocytoma was the most common paediatric brain tumour (29%) followed by medulloblastoma (12%) and ependymoma (6%). Most tumours were histologically benign (59%), and 42% of tumours were located in the posterior fossa. The average age at diagnosis was 7.9 years. About 50% of children were treated with surgery alone, whereas the other 50% had surgery or biopsy plus adjuvant treatment. The overall one-year survival rate was 89% and the five-year survival rate was 80%. The five-year survival rates for pilocytic astrocytoma was 91%; medulloblastoma, 75%; ependymoma, 82%; and high grade glioma, 15%. Thus, a large proportion of paediatric brain tumours were histologically benign and were treated with surgery alone, but a subset of benign tumours required adjuvant treatment and were associated with mortality (25%). The overall survival rates were high and are improving, although for some tumours, such as high grade glioma, the outlook remains poor.     

Late malignant recurrence of childhood cerebellar astrocytoma

Juvenile pilocytic astrocytoma of the cerebellum has a benign course and a good prognosis. We report a case of juvenile cerebellar astrocytoma in a 6-year-old girl that underwent surgical resection of the tumor and had two recurrences, 13 and 35 years after first removal. After surgery the patient did not receive any radiation therapy. The last relapse showed histological features of an anaplastic astrocytoma. Six months later the patient died with a diffuse leptomeningeal dissemination. Late malignant transformation of a benign cerebellar astrocytoma is very rare and it is thought to be favored by postsurgical irradiation. The possible pathogenetic mechanisms of this evolution are discussed. This case and the few others reported in the literature emphasize the risk of an unpredictable outcome with the low-grade cerebellar astrocytomas of childhood.     

Intracranial Metastases of Cervical Intramedullary Low-Grade Astrocytoma without Malignant Transformation in Adult

The first case of intracranial metastases of a cervical intramedullary low-grade astrocytoma without malignant transformation in adult is presented in this report. Seven years ago, a 45 year-old male patient underwent biopsy to confirm pathologic characteristics and received craniocervical radiation and chemotherapy for a grade II astrocytoma in the cervical spinal cord. Two years later, posterior fusion was necessary for progressive kyphosis in the cervical spine. He was well for approximately 7 years after the primary surgery. Two months ago, he presented with partial weakness and incoordination with gait difficulty. MRI Scan demonstrated multiple small lesions in the cerebellar vermis and left hemisphere. After suboccipital craniectomy and posterior cervical exposure, the small masses in the cerebellar vermis and hemispheres were excised to a large extent by guidance of an intraoperative navigation system. The tumor at the cervical and brain lesions was classified as an astrocytoma (WHO grade II). When a patient with low-grade astrocytoma in the spinal cord has new cranial symptoms after surgery, radiaton, and chemotherapy, the possibility of its metastasis should be suspected because it can spread to the intracranial cavity even without malignant transformation as shown in this case.     

Perception and estimation of time in long-term survivors of childhood posterior fossa tumors.

We examined short duration perception (400 ms), long duration estimation (30 and 60 min), and spatiotemporal estimation in long-term survivors of childhood cerebellar tumors with a mean time since diagnosis of 14.2 years. Groups of individuals with tumors treated with surgery only (astrocytoma, N = 20) were compared to those with tumors treated with surgery, focal radiation, and craniospinal radiation (medulloblastoma, N = 20), and to age-matched controls (N = 40). Childhood lesions of the cerebellum produced enduring deficits in short-duration perception, but spared the ability to functionally estimate long durations, regardless of the pathology or treatment of the tumor. Evidence did not support any functional recovery over time of the cerebellar system that underlies short-duration perception. Younger age at treatment was not a protective factor. Although no group differences were present in the functional measures of long-duration estimation, tumor-related prospective memory deficits interfered with the ability to produce long-duration prospective estimates. The utilization of sensory and somatomotor information to refine real-world spatiotemporal estimates was compromised in the medulloblastoma group only.     

Impact of surgery and adjuvant therapy on balance function in children and adolescents with cerebellar tumors

OBJECTIVES: This study examined the effects of posterior fossa tumor surgery and concomitant irradiation and/or chemotherapy on the long-term recovery of balance function in children and adolescent patients. SUBJECTS AND METHODS: 22 patients, treated during childhood for a benign (n = 14) or malignant cerebellar tumor (n = 8), were examined in chronic state (mean latency between surgery and testing: 7.7 years, range 3 - 17 years). Postural impairments were assessed with static and dynamic posturography. All cerebellar lesions were documented by standardized and normalized MRI data. Healthy age- and gender-matched subjects served as a control group. RESULTS: Comparing the balance function of (i) children with or without affected cerebellar nuclei and (ii) children with and without adjuvant chemotherapy and/or radiotherapy revealed that damage to the cerebellar nuclei had more impact on neurological impairment than concomitant tumor therapy. Balance abnormalities were most pronounced when a lesion affected the fastigial nucleus. Chemotherapy with its neurological side effect was associated with enhanced postural sway in only two children with malignant tumors. CONCLUSIONS: The study results indicate that the sparing of the deep cerebellar nuclei had the greatest impact on the recovery of balance function in pediatric patients treated for both a benign or malignant cerebellar tumor.     

Arrested growth and spontaneous tumor regression of partially resected low-grade cerebellar astrocytomas in children

Purpose

The prognosis of children with low-grade cerebellar astrocytoma who have partial resection of tumor is largely unpredictable. The purpose of this study was to review the long-term outcome of such patients.

Methods

The medical charts, imaging findings, operative notes, histopathological reports, and survival times of 12 patients with cerebellar astrocytoma were reviewed.

Results

Five patients had total resection and seven had partial resection. Nine patients had grade I histology and three patients had grade II. Follow-up duration ranged from 3 to 25 years. Among the seven patients with residual tumor, five had tumor progression, one had arrested tumor growth, and one had spontaneous tumor regression. Five patients with partial resection received radiotherapy and three had malignant transformation of tumor during follow-up. Six patients, including five who had partial resection, underwent a second operation. One patient with partial resection died of pneumonia 23 years after surgery.

Conclusions

Patients with complete tumor resection had a better prognosis than patients with partial resection. For patients with partial resection, we recommend a “wait and see” policy with surveillance using MRI. The phenomenon of arrested tumor growth and spontaneous tumor regression in patients with cerebellar astrocytoma who have subtotal resection warrants further study.     

Differential prefrontal-like deficit in children after cerebellar astrocytoma and medulloblastoma tumor

Background  

This study was realized thanks to the collaboration of children and adolescents who had been resected from cerebellar tumors. The medulloblastoma group (CE+, n = 7) in addition to surgery received radiation and chemotherapy. The astrocytoma group (CE, n = 13) did not receive additional treatments. Each clinical group was compared in their executive functioning with a paired control group (n = 12). The performances of the clinical groups with respect to controls were compared considering the tumor's localization (vermis or hemisphere) and the affectation (or not) of the dentate nucleus. Executive variables were correlated with the age at surgery, the time between surgery-evaluation and the resected volume.     

Reversible cerebral perfusion alterations in children with transient mutism after posterior fossa surgery

Mutism is an infrequent and transitory complication observed following posterior fossa surgery. Patients become mute in the immediate postoperative period, with restoration of speech within a few weeks in the absence of additional neurological alterations. The anatomical structures thought to be involved are the connections between the cerebellar dentate nucleus, the ventrolateral nucleus of the contralateral thalamus and the supplementary motor area. In an attempt to understand the pathophysiology of this syndrome, and to depict the perfusion of different brain areas semiquantitatively, in two children who had become mute after posterior fossa surgery we performed a Tc99M-HM-PAO SPECT study during the period of mutism and again when normal speech had returned. In one patient, who had a left cerebellar astrocytoma, the SPECT study showed a marked reduction of cerebral perfusion in the right fronto-parietal region, and in the other, who had a medulloblastoma, a left fronto-temporo-parietal perfusion alteration was observed. When the patients regained normal speech, the follow-up SPECT studies revealed normalization of the cerebral perfusion. This study demonstrates the occurrence of a focal dysfunction of cerebral perfusion in children with cerebellar mutism after posterior fossa surgery. These observations are useful in extending our understanding of the pathophysiology of this postoperative clinical syndrome. Received: 12 September 1997 Revised: 17 November 1997     

小脑性缄默4例报告及文献复习

目的探讨后颅窝手术后小脑性缄默的临床特点及其形成的病理生理机制。方法回顾性总结分析我科4例和英文文献报告的137例共141例后颅窝手术后出现的小脑性缄默病例。结果儿童病例127例,占90.1%。手术病变位于小脑蚓部者125例(88.7%)。小脑性缄默均为暂时性,发生的平均潜伏期和持续时间分别为1.7d和56.2d。结论小脑性缄默多见于儿童小脑蚓部肿瘤手术后其发生机制可能与小脑齿状核丘脑腹外侧核运动区和辅助运动区之间的纤维联系的直接或间接损害有关。     

Surgical Treatment of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex Patients

BackgroundSubependymal giant cell astrocytoma is a brain tumor associated with tuberous sclerosis complex. There are two treatment options for subependymal giant cell astrocytomas: surgery or mammalian target of rapamycin inhibitor. The analysis of outcome of subependymal giant cell astrocytoma surgery may help characterize the patients who may benefit from pharmacotherapy.MethodsSixty-four subependymal giant cell astrocytoma surgeries in 57 tuberous sclerosis complex patients with at least a 12-month follow-up were included in the study. The tumor size, age of the patients, mutation in the TSC1 or TSC2 gene, indication for the surgery, and postsurgical complications were analyzed.ResultsThe mean age of patients at surgery was 9.7 years. Mean follow-up after surgery was 63.7 months. Thirty-seven (57.8%) tumors were symptomatic and 27 (42.2%) were asymptomatic. Patients with TSC2 mutations developed subependymal giant cell astrocytoma at a significantly younger age than individuals with TSC1 mutations. Four patients (6.2% of all surgeries) died after surgery. Surgery-related complications were reported in 0%, 46%, 83%, 81%, and 67% of patients with tumors <2 cm, between 2 and 3 cm, between 3 and 4 cm, >4 cm, and bilateral subependymal giant cell astrocytomas, respectively, and were most common in children younger than 3 years of age. The most common complications included hemiparesis, hydrocephalus, hematoma, and cognitive decline.ConclusionsOur study indicates that subependymal giant cell astrocytoma surgery is associated with significant risk in individuals with bilateral subependymal giant cell astrocytomas, tumors bigger than 2 cm, and in children younger than 3 years of age. Therefore, tuberous sclerosis complex patients should be thoroughly screened for subependymal giant cell astrocytoma growth, and early treatment should be considered in selected patients.     

Heterologous transplantation of cerebral and cerebellar astrocytomas

Summary Twenty two cases of cerebral and so-called cerebellar astrocytomas were heterologously transplanted to guinea pigs. Although the fifteen cerebral astrocytomas were selected according to optimal conditions for transplantation, all failed to grow heterologously. The cerebral astrocytomas represent varieties of fibrillary, protoplasmic and pilocytic types, and all originated in adults. Of the seven randomly selected so-called cerebellar astrocytomas, all of which occurred in children, only one grew when transplanted intracerebrally to guinea pigs. This tumor before transplantation had all the histological characteristics of a so-called cerebellar astrocytoma. The prevailing cells were elongated, spongioblast-like elements; however, other cell types, e.g., astrocytes occurred in varying numbers. The heterotransplant was dominated by elongated, mostly bipolar cells with a very characteristic cytoarchitectural arrangement. The controversy regarding classification of cerebellar astrocytomas versus cerebellar spongioblastomas was discussed. Although the name spongioblastoma is not favored, the concept of classifying the so-called cerebellar astrocytoma together with other slow growing midline gliomas as proposed by Zülch has definite merits. Heterotransplantation studies clearly demonstrate that the so-called cerebellar astrocytoma has different biological properties than cerebral astrocytomas.Supported by United States Public Health Service Research Grant No. NB-07849-04.     

Malignant cerebellar astrocytomas in childhood Experience with four cases

The most frequent type of cerebellar astrocytoma in children is the pilocytic variety, for which the treatment of choice and prognosis are now well established. In contrast, an anaplastic lesion in this site is extremely rare. The authors present four cases of high-grade astrocytoma in a cerebellar site. They discuss the best type of treatment for this pathology, with particular reference to postoperative radiotherapy and chemotherapy, in the light of their results. Received: 9 May 1997 Revised: 21 April 1998     

Malignant astrocytoma of the optic nerve in a child.

Malignant gliomas of optic nerve and chiasm are rare, rapidly fatal neoplasms of adulthood. This report documents the occurrence of a malignant astrocytoma of the optic nerve in an 11-year-old boy who 9 years previously had a cerebellar medulloblastoma treated with surgery and irradiation. This malignant optic nerve glioma followed the same aggressive clinical course as that seen in adults, with death 9 months after diagnosis despite surgery and chemotherapy. Radiation may have been an important factor in the development of this malignant tumor which is almost never seen in the pediatric age group.     

Radiologically typical pilocytic astrocytoma with histopathological signs of atypia

Pilocytic astrocytoma (PCA) is the most common CNS tumor in primary school-aged children. Herein, we report the case of a 7-month-old female child with a large cerebellar hypodense tumor on computer tomography occupying nearly the whole cerebellar hemisphere. Magnetic resonance imaging revealed characteristic features of a PCA, depicting a mass with hyperintense solid and cystic areas on T2-weighted images and with marked solid and ring-like enhancement pattern. Histopathological and immunohistochemical analysis showed partially typical features of a PCA, but also atypical features such as a high proliferation rate, hypercellularity, and focally diffuse infiltration pattern were present. However, definite signs of transformation to an anaplastic PCA were not observed. We therefore classified the tumor as pilocytic astrocytoma with signs of atypia instead of simple WHO grade I pilocytic astrocytoma. The case illustrates that neuroimaging features may give very important clues for the definite diagnosis in histopathologically atypical PCA.