Object. A multidisciplinary team devised a protocol for long-term care of patients with skull base chordomas. In this study they describe their approach. Methods. Forty-two patients presented between 1986 and 1998 and were treated by maximum surgical cytoreduction and photon radiation therapy. Tumor volume—doubling time determined on the basis of magnetic resonance imaging, immunostaining, and cell proliferation (Ki67 labeling index [LI]) studies indicated growth rates of individual chordomas. The best outlook was associated with the greatest extent of tumor removal achieved during the first operation. There were no deaths associated with patients who underwent first-time surgery, but there was a 7.1% mortality rate associated with those who underwent subsequent operations. Cerebrospinal fluid leaks, additional cranial nerve palsies, and pharyngeal wound problems were the most difficult management problems encountered after second and subsequent surgeries. The time interval between operations was usually between 2 years and 3 years after the first surgery; very few patients required a second surgery, with a quiescent period in excess of 5 years. Life-table 5- and 10-year survival rates were 77% and 69%, respectively. Conclusions. The authors believe that this series of skull base chordomas provides new insights into the management of these lesions, particularly with regard to techniques that increase survival times and studies that aid in formulating prognoses.

The presentation and results of treatment are reviewed for 38 patients with skull base chordoma treated at the National Hospital for Neurology and Neurosurgery between 1958 and 1988. With few exceptions, previous studies have combined results for clival and sacral chordomas, or for chordomas and other similar tumours such as chondrosarcoma, and thus it is difficult to be specific about effects of therapy. This study included histological review using immunohistochemistry to confirm diagnosis. Analysis of the survival data for our patients suggests that there are two subgroups with distinct survival patterns: one group with high mortality within the first 5 years, and a second group with an indolent disease process and near normal life expectancy. The age of the patients at presentation ranged from 7 to 78 years, with a mean of 44.3 years. Male: female distribution was 6:5. The commonest presentation was with cranial nerve palsy (94%) or with headache (60%). The most frequently involved cranial nerve was the VIth (60%), followed by the IXth and Xth (40% each). Comparing our results with those of 50 years ago, there was little improvement in the outlook for these patients, despite improvements in surgical approaches and the use of radiotherapy. The promising results in skull base tumours using proton therapy must be treated with caution until definite criteria for diagnosis and outcome have been established. There is a case for a multicentre prospective study of this disease.

We describe three unusual tumours characterized by a mixture of glial and neuronal differentiation, involvement of the posterior fossa and formation of rosettes. Mixed glial-neuronal tumours of the posterior fossa are rare and poorly described neoplasms. However, several distinctive entities have appeared in the literature over recent years under a variety of different names. Our cases demonstrate the morphological features of the 'rosette-forming glioneuronal tumour of the fourth ventricle', a recently identified tumour characterised by its unique location, neurocytic pseudo-rosette formation and the presence of a low grade astrocytoma component. The long term prognosis of these tumours remains unclear. However, the clinical data available including the cases presented here, along with the histological features, suggest that these are low grade tumours with a good prognosis after surgical resection.

Eighty-one children with malignancies and their families were investigated for the psychosocial changes that take place during the course of the disease. Seventeen patients were in the initial phase of treatment, 24 were in first remission, 14 were long-term survivors already off therapy, 11 were in relapse, and 15 children died 1-5 years before this study. Detailed personal interviews with the parents showed profound changes in the families' life and severe problems in adapting to the new situation. Marital problems, neglecting the healthy siblings, and a loss of interest in work occurred in the majority of parents, especially in mothers. Younger siblings suffered more from the strains imposed on the family than did elder ones. Psychosocial care is felt to be helpful for all families in adjusting to the altered circumstances and emotional upheavals.

The aim of the study was to assess the relationship between the rate of clinical tumour growth and various histological features, including Ki67 labelling index, in skull base chordoma. Cases of skull base chordoma from 19 patients (six female, 13 male; age range 8-63 years) were reviewed and the diagnosis confirmed based on histological and immunohistochemical features. In each biopsy cellularity, pleomorphism, mitotic activity, apoptotic bodies, necrosis and inflammatory cell infiltrate were graded and Ki67 labelling index (LI) calculated as a measure of proliferation. Tumour doubling time was assessed by quantitative analysis of tumour volumes in post-operative magnetic resonance images and correlated with age, sex, histological parameters and Ki67 LI. It was shown that increasing patient age, the presence of mitotic figures or a Ki67 LI in excess of 6% were associated with faster growing tumours.