Thalamic tumors in children

Thalamic tumors (TT) merit individual analysis and must not be confused with tumors that, while involving the entire thalamus have a different origin. We analyzed 26 patients who fulfilled our criteria of having ``strictly' TT. We examined incidence, clinical features, histology, response to treatment (mainly surgery), recurrence rate, mortality and prognosis. We considered that histology and surgical treatment were the most important items related to prognosis. Low-grade tumors (LGT) had a good prognosis, while anaplastic tumors (AT) had a discouraging one; nevertheless both must be operated on. We believe that total removal of LGT is curative and total removal of AT, even if it is not curative, can extend survival by some months. Radiotherapy and chemotherapy seemed to be of little value in our series of TT. Received: 3 October 1995     

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     

Treatment and survival of supratentorial and posterior fossa ependymomas in adults

Ependymoma is a rare primary brain or spinal cord tumor that arises from the ependyma, a tissue of the central nervous system. This study analyzed a large cohort of adult supratentorial and posterior fossa ependymoma tumors in order to elucidate factors associated with overall survival. We utilized the USA National Cancer Database to study adult World Health Organization grade II/III supratentorial and posterior fossa ependymoma patients treated between 1998 and 2011. Overall survival was estimated by the Kaplan–Meier method and factors associated with survival were determined using a multivariate Cox proportional hazards model. Among 1318 patients, 1055 (80.0%) had grade II and 263 (20.0%) anaplastic tumors located in the posterior fossa (64.3%) and supratentorial region (35.7%). Overall average age was 44.3 years, 48.0% of patients were female, 86.5% were Caucasian, and 36.8% underwent near/gross total surgical resection. Radiotherapy was given to 662 patients (50.8%) and 75 (5.9%) received chemotherapy. Older age at diagnosis (hazard ratio [HR] 1.51, p < 0.0001), high tumor grade (HR 1.82, p = 0.005), and large tumor size (HR 1.66, p = 0.008) were associated with poor survival. Females compared to males (HR 0.67, p = 0.03) and patients with posterior fossa tumors versus supratentorial (HR 0.64, p = 0.04) had a survival advantage. Our study showed that older patients, with supratentorial tumors, and high histological grade had an increased risk of mortality. A survival benefit was captured in females and patients with posterior fossa tumors. Adjuvant radiotherapy and chemotherapy did not confer a survival benefit among all patients, even after stratification by tumor grade or anatomical location.     

Intracranial neoplasms in children in Ibadan,Nigeria

Analysis of 89 intracranial tumors in children presenting at the University College Hospital, Ibadan, Nigeria, between 1960 and 1982 is reported. These tumors are commonest in the first decade of life and occur more in males than in females. Fifty-three per cent of the primary brain tumors are supratentorial. The commonest sites are cerebellum, cerebrum, and the pons. The common histological types are astrocytomas, medulloblastomas, ependymomas, and oligodendrogliomas. Craniopharyngiomas are among the most frequent supratentorial neoplasms in Ibadan children and the central nervous system involvement in cases of Burkitt's lymphoma is a frequent complication.     

Brain tumors of childhood: nosological and diagnostic problems

Brain tumors containing undifferentiated cells were selected from a series of 504 childhood brain tumors; 117 were analyzed. Most tumors were medulloblastomas, followed by cerebral neuroblastomas, pineocytomas-blastomas, ependymoblastomas, and polar spongioblastomas. Of each oncotype, the main histological features were evaluated, including differentiation and the most important prognostic factors. The terminology and different tumor entities are discussed in light of the recent PNET system. The usefulness of its application is evaluated in relation to prognosis.Supported by grant no. 87.01446.44 from the Italian National Research Council (CNR), Special Project Oncology, Rome, and from the Italian Association for Cancer Research (AIRC), MilanPresented at the 11th Meeting of the European Society for Paediatric Neurosurgery, Naples 1988     

Immunohistochemistry in childhood brain tumors: what are the facts?

In the past, contradictory results have been reported concerning the specificity of neuronal or glial cell markers. However, we have investigated this aspect in a large group of more than 550 brain tumors (among them 60 medulloblastomas). These contradictions can easily be explained by considering two basic facts. First, every neoplastic cell population, especially in embryonic tumors, diffusely infiltrates the brain tissue: non-neoplastic cells, intermingled with tumor cells, can therefore give rise to immunohistochemical and histogenetic misinterpretations. Second, different cell markers can be expressed by one and the same cell (e.g., GFAP, NSE, vimentin), making nosological interpretation of the tumor difficult, impossible, or at best rather subjective. Clear-cut marker positivity is mostly found in the differentiated tumors for which the nosological classification is already clear by the usual histological methods. Only synaptophysin seems to be a reliable marker for neurogenic cells. In embryonic brain tumors (so-called PNET), no correlations between the presence of a given cell marker and the biological behavior of the tumor have so far been detected.     

Radiation therapy in the management of childhood brain tumors

Radiation therapy (RT) still plays a major role in the management of intracranial malignancies, together with surgical resection and, more recently, chemotherapy. This is a review of the experience with fractionated external beam RT. In medulloblastomas, combined modalities currently achieve a 5-year survival in excess of 70% in low-risk subgroups and 40% in the subgroups considered to be high risk. For the past decade, the emphasis has been on the quality of life in cured children. Recent advances have mainly aimed at limiting the toxicity of the ”prophylactic” craniospinal irradiation by testing dose reductions and altered fractionations. Technical innovations have also greatly benefited gliomas: modern techniques dealing with 3D CT and MRI-based treatment combined with stereotactic positioning of the patients, achieve a high degree of conformity that might improve both local control and long-term neurocognitive and growth sequelae. Received: 21 March 2000     

CT and MRI aspects of supratentorial hemispheric tumors of childhood and adolescence

The neuroradiological features of supratentorial hemispheric tumors (SHTs) were studied in 27 patients whose ages ranged from 11 months to 18 years. Astrocytomas constitued 10 of the 27 SHTs. On computed tomography low-grade astrocytomas were in most cases hypodense; after intravenous administration of contrast medium, pilocytic astrocytomas enhanced, whereas fibrillary astrocytomas did not. Gd-DPTA-enhanced magnetic resonance imaging was the most useful technique for the assessment of recurrences. Atypical imaging features were observed in one glioblastoma and in oligodendrogliomas (in half of the cases no calcifications were found). Gangliogliomas were surprisingly rather frequent in our series (5/27) and appeared in three cases as low-density, well-circumscribed lesions, not calcified and without edema and mass effect, while in two cases they had pronounced perifocal edema without clear demarcation. A rare desmoplastic infantile ganglioglioma was observed. The two meningiomas showed malignant behavior.     

Biological aspects of brain tumors in infancy and childhood

Notwithstanding the definitive systematization of theclinical features of childhood brain tumors, manybiological laws governing this vast area of pathology still escape us. There are no sure explanations for the fickleness of supratentorial/subtentorial distribution in fetal life and in the first 15 years of life — something not found in later years. Another focus of discussion is the tendency of brain tumors in infancy to concentrate along the midline due to the fact that most infantile neuroectodermal tumors arise from the phylogenetically older structures of the CNS (periventricular regions, brainstem, cerebellum), in contrast to tumors of adulthood. Neuroepithelial tumors, much more frequent in childhood than in later years, exhibit substantial histological differences in infancy: mixed gliomas and primitive neuroectodermal tumors (not otherwise specified, or with astrocytic, ependymal, oligodendrocytic ... cells) are the best examples. As to the question of whether a given oncotype exhibits different biological behavior according to patient age, there is no single answer: some malignant tumors (medulloblastomas, ependymomas, neuroblastomas) are more aggressive in infancy, in line with Collins' law, while others (optic gliomas) offer a better prognosis in younger patients. The most peculiar and disquieting aspect of brain tumors in infancy/childhood, however, is what emerged from a recent epidemiological survey. The survey was conducted on the close relatives of a boy with a brain tumor from point of view of a possible second malignancy in this boy. Since a brain tumor is a rare occurrence in the very young, it may signal heightened susceptibility to malignancy in the individual, extending to other tumors in the patient himself and even to other members of his family.This paper was presented in part at the XIth Congress of the European Society for Paediatric Neurosurgery, Naples, 1988     

MRI studies after treatment of brain tumors in childhood and adolescence

Forty-seven children and adolescents with brain tumors were examined by magnetic resonance imaging (MRI) after tumor resection. The typical changes and complications after surgery and chemotherapy, as well as the corresponding MRI findings, are discussed. Typical examples of boundary-layer lesions, tumor recurrences, hydrocephalus, porencephalic cysts, and hygromas are given.     

Paravertebral malignant tumors of childhood: analysis of 28 pediatric patients

Purpose  To evaluate the clinical features and treatment results of the primary paravertebral malignant tumors (PMTs) in our department. Methods  Medical records of 28 children with primary PMTs treated between 1988–2007 were analyzed retrospectively. Results  Primary PMTs constituted 4.8% of the cancer cases in our department. Tumor diagnoses were mostly neuroblastoma (46.4%) and soft tissue sarcomas (35.7%). These cases presented with pain (64.3%), motor dysfunction (42.8%), sphincter dysfunction (35.7%), palpable mass (32.1%), and sensory deficits (7.1%). All tumors were extradural. Physical examination revealed motor deficits (53.6%), deep tendon reflex alterations (53.6%), sphincter dysfunction (35.7%), pathologic reflexes (25%), abnormal cutaneous reflexes (25%), and sensory deficits (17.8%). Sixteen had cord compression (CC; 13 clinical, three radiological CC). Eleven of them presented with advanced disease. Seven were managed by surgical departments by primary surgery (three unresponsive). Others were managed by pediatric oncology: five with corticosteroids ± chemotherapy (one unresponsive), one with radiotherapy (RT), and two with surgery for the clinical CC. Surgery was tumor excision in nine, laminectomy in nine, laminotomy in one, and delayed surgery after chemotherapy in two cases. In chemotherapy and surgery groups, there were neurologic sequela associated with the advanced disease at diagnosis in 38% and 37%, respectively. At 3-year median follow-up, nine patients died, 17 are alive (four with neurologic sequela), and two are lost of follow-up. Conclusion  Majority of cases presented with advanced disease. Late referral is the major cause of morbidity and mortality. The CC caused by PMTs should be initially managed with corticosteroids ± chemotherapy to avoid the adverse late effects of RT and surgery.     

A comparative study of CT and MRI in midline tumors of childhood and adolescence

In order to compare the validity of CT and MRI, we evaluated results of these studies in 40 children and adolescents suffering from supratentorial and infratentorial midline tumors. Plain and enhanced CT scans were compared with MRI for sensitivity, specificity and capacity to delineate the tumor. These parameters were evaluated by three independent investigators on a scale with four grades of accuracy. The results demonstrate greater sensitivity and better delineation of the tumor with MRI, but greater specificity in diagnosis of tumors with CT studies.Presented at the XIth Meeting of the European Society for Paediatric Neurosurgery, Naples, 1988     

Early outcomes of supratentorial cranial surgery for tumor resection in older patients

With longevity increasing in the United States, more older individuals are presenting with supratentorial brain tumors. Despite improved perioperative management, there is persistent disparity in surgical resection rates among patients aged 65 years or older. We aim to assess the effects of advanced age (≥65 years) on 30-day outcomes in patients with supratentorial tumors who underwent craniotomy for supratentorial tumor resection. Data obtained in adults who underwent supratentorial tumor resections was extracted from the prospectively-collected American College of Surgeons: National Surgical Quality Improvement Program (NSQIP; 2012–2018) database. Using multivariate regression, we compared odds of major and minor complications; prolonged length-of-stay (LOS); discharge anywhere other than home; and 30-day readmission, reoperation, and mortality rates between patients aged 18–64 years (the control cohort) and those 65–74 years or ≥75 years of age. Of the 14,234 patients who underwent craniotomy for supratentorial tumors and met inclusion criteria, 30.7% were ≥65 years of age; 71.4% of these were 65–74 years and 28.6% were ≥75 years old. Compared to the control group, both older subpopulations had more medical comorbidities. Both older subgroups had increased odds of major complications and prolonged LOS relative to the control group. Older patients had greater odds of mortality at 30 days. Advanced age, defined as ≥65 years, was significantly associated with higher odds of complications, prolonged LOS, and mortality within the 30-day post- operative period after adjusting for potential confounders. Age is one important consideration when prospectively risk-stratifying patients to minimize and mitigate suboptimal perioperative outcomes.     

Brain tumors in childhood: Statistical analysis of cases from the brain tumor registry of Japan

This paper attempts to summarize and statistically analyze 2,361 pediatric brain tumors out of a total of 20,192 human brain tumors from about 250 institutes in Japan during the period 1969–1978 in order to obtain an idea of their incidence and results of treatment. This report uses the classification and nomenclature adopted by UICC (Unio Internationalis Contra Cancrum) in 1965. Survival rate was computed as relative survival rate by the method reported by Cutler in 1964 [3]. Interestingly, germinomas (7.8%) are still frequent tumors in Japan. Astrocytoma (19.7%), medulloblastoma (16.6%), craniopharyngioma (12.5%), germinoma (7.8%) and ependymoma (6.6%) occurred in that order of frequency. Five-year survival rate of medulloblastoma was 35.7% in subtotal and 55.3% in total removal. Radiotherapy was an effective therapeutic adjunct for the treatment of medulloblastoma. The germinoma prognosis was good, with a 5-year survival rate of 42.4% without and 69.1% with radiation therapy, revealing radiotherapy as a statistically significant treatment. Radiotherapy, with or without shunt, is thus the treatment of choice. The survival rate of patients with craniopharyngiomas was also high, with a 5-year survival of approximately 75% in partial and 76.3% in total removal. Most of the craniopharyngiomas were treated by partial removal of the tumor, with or without radiotherapy. Patients treated with radiotherapy survived a little longer than patients without.A part of this paper was presented at the 13th Annual Meeting of the International Society for Pediatric Neurosurgery     

1267例儿童神经系统肿瘤的流行病学

目的回顾性分析儿童神经系统肿瘤的流行病学特点。方法2001年1月至2005年12月五年间,获得组织病理学诊断的儿童(年龄≤15岁)原发性神经系统肿瘤1267例,按照WHO 2000年新分类,分析患者性别、肿瘤部位和组织学类型、分级与年龄的相关性。结果脑和椎管肿瘤分别为1177例(92.9%)和90例(7.1%),均以低级别肿瘤为主(65.20h,和76.7%),总的男女性别之比为1.6:1。脑肿瘤以幕上居多(60.4%),而750例神经上皮性脑肿瘤以幕下居多(57.3%)。常见的五类脑肿瘤是星形细胞肿瘤(29.9%)、颅咽管瘤(19.8%)、髓母细胞瘤(15.7%)、生殖细胞肿瘤(GCT)(7.5%)和室管膜肿瘤(5.9%),男性比例最高的是GCT和髓母细胞瘤。33.8%的星形细胞肿瘤发生于青春期;颅咽管瘤除婴幼儿组外,各年龄组发病几乎均等;髓母细胞瘤多见于7—9岁(33.0%),而在婴幼儿和青春期发病较低;GCT在2岁内无患病者,10—15岁占71.6%。室管膜肿瘤以幕下为主(77.1%),婴幼儿期的发生比例最高。椎管肿瘤常见的是星形细胞瘤(17.8%)、室管膜肿瘤和脂肪瘤(均为15.6%)。结论本研究基于WHO新分类,反映了国内儿童神经系统肿瘤的流行病学特点。     

Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome

Objective Brainstem tumors (BSTs) are usually gliomas and are divided into diffuse BSTs (DBSTs) and focal BSTs (FBSTs). The aim of this study is to investigate the different outcomes of these two entities.Methods Thirty-one patients with BSTs were admitted to our institution from 1995 to 2003. Patients with DBSTs were treated with locoregional radiotherapy (1.8 Gy/day for 54 Gy) and weekly vincristine for radiosensitization (1.5 mg/sm for six total doses). Patients with FBSTs underwent surgical resection. Chemotherapy and/or radiotherapy were considered in progression.Results and conclusions Fourteen patients were diagnosed as having DBSTs. The responses to treatment were ten cases of partial response, three of stable disease, and one of progressive disease. General and/or neurological symptoms improved in more than 80% of patients. The median time from diagnosis to progression and to death were, nonetheless, 8 (range of 3–13) and 13 (range of 4–25) months, respectively, with a 2-year overall survival rate of 12.3% [standard error (SE) 11.2]. Seventeen patients were diagnosed as having FBSTs. Gross total removal was achieved in 4/17 cases, subtotal removal in 7/17, and partial removal in 6/17. There was one surgery-related death. Eight out of 17 patients had adjuvant chemo- and/or radiotherapy after progression: 6/8 are without neurological symptoms and 2/8 have died due to tumor progression. The 4-year overall and disease-free survival rates are 87.4 (SE 8.4) and 58.8% (SE 11.9), respectively, the extent of resection being the most important prognostic factor (p=0.012). DBSTs continue to carry a dismal prognosis, thus demanding new treatment modalities; FBSTs can be treated surgically and patients benefit from a better prognosis.     

以神经系统症状首发的恶性肿瘤37例临床分析

恶性肿瘤以神经系统症状起病的并非少见,但许多原发肿瘤症状不表现或隐匿.常以神经系统症状就诊从而被误诊.故现将自2001年10月至2006年11月南京军区总医院神经内科收治的以神经系统症状首发且均经CT、MRI或活检等检查后确诊的37例恶性肿瘤患者的临床资料报道如下,以期提高其诊断正确率.