Cerebellar Pilocytic Astrocytoma Research Articles

Superficial siderosis 20 years after brain tumour

A 6-year-old girl presented in April, 1962, with headache, vomiting, and an unsteady gait. She had left-sided ataxia and bilateral papilloedema. Skull radiograph showed accentuation of the convolutional pattern and ventriculography showed symmetrical hydrocephalus with blockage at the upper end of the aqueduct. After insertion of a ventricular drain, a posterior fossa craniectomy was performed and a left cerebellar pilocytic astrocytoma was resected on May 16, 1962. Since all the margins were not clear of tumour, she received radiotherapy (4000 rads).

Quality-of-life assessment in patients who had been surgically treated for cerebellar pilocytic astrocytoma in childhood.

After radical surgery for childhood cerebellar astrocytomas, patients are considered to be cured. Long-term follow up demonstrates that these patients survive, with most of them leading a normal life. The study reported here was aimed at assessing the quality of life (QOL) of these adults, which is defined as a person's sense of well-being, as derived from his or her current experience of life as a whole. Twenty patients who had undergone surgery between 1970 and 1985 were enrolled in the study. In four patients ventriculoperitoneal shunts were in place; two of these patients had required more than six shunt revisions. At present, all patients have clear neuroimaging studies and their Karnofsky Performance Scale (KPS) scores are as follows: 70 in three, 80 in seven, 90 in six, and 100 in four. A QOL questionnaire was administered to the patients and to a control group consisting of 20 healthy volunteers of matching age and sex. The chi-square test was applied to compare patients and controls. Traditional questions on the level of education, work, whether the patients have their own families, and whether they possessed a driver's license were asked at the end of the questionnaire. In all the dimensions assessed except one (sex life), the difference between patients and control volunteers was significant, socializing and adolescence being the most striking ones. This was also true when the three patients with the lowest KPS scores and the worst QOL results were excluded. By traditional standards, these patients appear to fare quite well. Nevertheless, their self-reported life experience is unsatisfying when compared with the control group. The authors conclude that psychosocial factors are critical to complete recovery and the QOL of children who undergo successful operations for benign cerebellar astrocytoma.

Proliferative activity as measured by MIB-1 labeling index and long-term outcome of cerebellar juvenile pilocytic astrocytomas.

Proliferative activity of cerebellar juvenile pilocytic astrocytomas (CJPA) and its significance for prognosis was retrospectively investigated. Forty-four consecutive children operated between 1981 and 1997 with a mean age of 8.3 years (3 months to 20 years) were reviewed. Clinical and radiological follow-up was available for 38 patients ranging from 0 to 18 yrs (mean 6.3 years). Proliferative activity was determined by MIB-1 immunohistochemistry on sections of resected tumor specimen. Total resection was achieved in 35 children (79.5%), subtotal in 9 (20.5%). Currently, 31 are tumor-free, 6 have stable remnants, one developed spinal seeding and one died. Radiology revealed a cystic mural node type tumor in 27 patients (61.4%), a solid lesion with a small cyst in 5 patients (11.4%), and a solid tumor in 12 patients (27.3%). Mean MIB-1 labeling index (LI) of all tumors was 4.4% (range 0.6-12%, SD = 2.7) and did not correlate with age, gender, localization, amount of resection, follow-up status, histological appearence or grade of neovascularization, but showed a significant correlation to radiological types: 6.9% in solid tumors versus 3.7% in the cystic mural node type (p = 0.0037). Five year progression-free survival (PFS) of all patients was 84.4%, differences between subgroups of MIB-1 <5% (27 patients, PFS = 87.4%) and MIB-1 >5% (13 patients, PFS = 76.3%) were not significant. CJPA showed a remarkable high MIB-1 LI, but no significant correlation to PFS in this series. Nevertheless, radiologically solid tumors demonstrated a significantly higher MIB-1 LI and thus may need further investigation for possible increased ability of regrowth.

Transient peduncular hallucinations secondary to brain stem compression by a cerebellar pilocytic astrocytoma

Almost all peduncular hallucinations have been described in patients with intrinsic lesions of the midbrain. An, as yet, unreported case of peduncular hallucinosis caused by posterior compression of the midbrain by a cerebellar pilocytic astrocytoma is described. The hallucinations and associated symptoms only ceased after removal of the tumour.

Cerebellar pilocytic astrocytoma with leptomeningeal dissemination: Case report

The authors present an extremely rare case of histologically benign cerebellar pilocytic astrocytoma that had already disseminated to the leptomeninges at the initial presentation.

Cerebellar pilocytic astrocytoma: a treatment protocol based upon analysis of 73 cases and a review of the literature.

In a retrospective study of 73 patients operated on for cerebellar pilocytic astrocytomas, results of treatment, outcome and biological behaviour of residual tumour were analysed. Complete tumour resection proven by CT or MRI scans within 1 year after surgery was achieved only in 69% of cases. In 31% of cases the surgeon's opinion on the extent of surgical resection was not borne out by the result of postoperative neuroimaging. Progression of residual tumour or tumour recurrence appeared in 19% of patients. 1 patient showed metastatic spread along the craniospinal axis, and in 1 patient malignant degeneration appeared during follow-up. Stable residual tumour or regression of residual tumour was seen in 14% of patients. Outcome after surgical treatment, which was combined with irradiation in 10 patients (14%), was favourable in 80% and unfavourable in 20% of patients. This outcome of treatment was not influenced by a second operation for progression of residual tumour or recurrent tumour. Characteristics of patients with tumour progression after the first operation did not differ from those of the whole group. There were 17 reoperations for residual or recurrent tumour, 10 of which took place within 4 years after the initial surgical treatment. Surgery-related morbidity was 15% and mortality 4%. Irradiation to residual tumour in 8 patients was followed by complete regression in 1 patient, progression in 4 patients and no changes in 1 patient. For the remaining 2 patients the effect of irradiation on the residual tumour is unknown. Factors that determine the prognosis are discussed on the basis of this retrospective analysis and the data from the literature. It is concluded that optimal treatment for a cerebellar pilocytic astrocytoma does not consist solely in surgery with the aim of total tumour removal and careful tumour handling in order to avoid spread of tumour cells and subsequent metastases and additional radiation therapy is strictly selected cases, but also in posttreatment follow-up based on direct postoperative neuroimaging, preferably by MRI. An algorithm for postoperative follow-up management is presented.

Chromosomal abnormalities in 47 pediatric brain tumors

The karyotypes of 47 pediatric brain tumors (14 cerebellar pilocytic astrocytomas, six cerebral pilocytic astrocytomas, seven anaplastic astrocytomas and glioblastomas, nine medulloblastomas [PNETs], one cerebral neuroblastoma, four ependymomas, and seven miscellaneous other neoplasms) are presented. Most of the pilocytic astrocytomas and ependymomas had normal karyotypes. In contrast, the majority of the anaplastic astrocytomas-glioblastomas were abnormal. The abnormalities included losses and structural abnormalities of chromosomes 9, 13, and 17, and double minutes. There were no losses of chromosomes 10 and 19q or gains of chromosome 7, which are among the most common abnormalities of adult glioblastomas. The chromosomal abnormalities in the medulloblastomas were similar to those reported in the literature but less frequent. Four tumors (choroid plexus papilloma, meningioma, cerebral malignant rhabdoid tumor, and immature teratoma) had losses of chromosome 22.

Pilocytic cerebellar astrocytoma in adults: Case report

A case of cerebellar pilocytic astrocytoma is reported. This tumor occurs typically in the first two decades of life and is seldom reported in adults. The 42-year-old patient presented with occipital headaches, nausea, and unsteady gait. Nystagmus and right dysmetria were noted. A CT scan showed a hypodense, nonenhancing, voluminous, right hemispheric cerebeller cyst. Magnetic resonance imaging showed a nodule in the wall of the cyst which became hyperintense with gadolinium. The mass was resected through a small occipital craniotomy. Neuropathological examination revealed a juvenile pilocytic astrocytoma.

Immunohistochemical demonstration of vimentin in human cerebral tumors.

The distribution of vimentin (VIM) has been histochemically investigated in 53 cerebral tumors and compared in gliomas to that of glial fibrillary acidic protein (GFAP). In gliomas VIM is less positive than GFAP, but shows the same distribution. It cannot be considered as indicating immaturity of glial tumor cells. VIM is also positive in glial processes of cerebellar pilocytic astrocytomas, in Schwann cells of neurinomas and in endothelial cells of all oncotypes. In medulloblastomas, VIM decorates reactive glia cells. A diffuse positive reaction has been observed in meningiomas. In hemangioblastomas, besides intervascular and endothelial cells, groups of polygonal cells are intensely positive for both VIM and GFAP. The interpretation of VIM in cerebral tumors is largely based on the distribution patterns of this intermediate filament in the developing CNS of rodents.