Relative survival of patients with non-malignant central nervous system tumours: a descriptive study by the Austrian Brain Tumour Registry.

BACKGROUND: Unlike malignant primary central nervous system (CNS) tumours outcome data on non-malignant CNS tumours are scarce. For patients diagnosed from 1996 to 2002 5-year relative survival of only 85.0% has been reported. We investigated this rate in a contemporary patient cohort to update information on survival. METHODS: We followed a cohort of 3983 cases within the Austrian Brain Tumour Registry. All patients were newly diagnosed from 2005 to 2010 with a histologically confirmed non-malignant CNS tumour. Vital status, cause of death, and population life tables were obtained by 31 December 2011 to calculate relative survival. RESULTS: Overall 5-year relative survival was 96.1% (95% CI 95.1-97.1%), being significantly lower in tumours of borderline (90.2%, 87.2-92.7%) than benign behaviour (97.4%, 96.3-98.3%). Benign tumour survival ranged from 86.8 for neurofibroma to 99.7% for Schwannoma; for borderline tumours survival rates varied from 83.2 for haemangiopericytoma to 98.4% for myxopapillary ependymoma. Cause of death was directly attributed to the CNS tumour in 39.6%, followed by other cancer (20.4%) and cardiovascular disease (15.8%). CONCLUSION: The overall excess mortality in patients with non-malignant CNS tumours is 5.5%, indicating a significant improvement in survival over the last decade. Still, the remaining adverse impact on survival underpins the importance of systematic registration of these tumours.

(18)F-FDG positron emission tomography in the early diagnosis of enterocolitis: preliminary results.

Collagenous and eosinophilic colitis are rare diseases characterised by chronic watery diarrhoea. Radiographic evaluation of the gastrointestinal tract and colonoscopy are usually non-diagnostic since as many as one-third of patients will have minor abnormalities. To date a few investigators have reported increased fluorine-18 fluorodeoxyglucose ((18)F-FDG) uptake on positron emission tomography (PET) in patients with acute enterocolitis, but there have been no reports on the use of (18)F-FDG PET for the diagnosis of collagenous or eosinophilic colitis in an early clinical stage. The aim of this preliminary study was to evaluate the usefulness of (18)F-FDG PET in the early diagnosis of patients with colitis. We investigated five women (mean age 61.2+/-12.1 years) who had been diagnosed as having colitis in an early clinical stage. In all but one of the patients, the diagnosis of colitis was based on biopsy. Magnetic resonance colonography, ultrasonography and colonoscopy were performed in all but one of the patients. Two women were identified as having collagenous colitis in an early clinical stage. Another two patients had eosinophilic colitis. The morphological imaging methods, magnetic resonance colonography and ultrasonography, yielded no suspicious findings, and the results of colonoscopy similarly showed no abnormalities. One patient had colitis due to bacterial infection. In all patients (18)F-FDG PET showed a pathological increase in tracer uptake in the large bowel, suggestive of colitis. In four of the five patients, colitis was confirmed by histology, and in one, by bacterial analysis. (18)F-FDG PET was able to detect colitis in an early clinical stage, when morphological imaging methods and colonoscopy were non-diagnostic. The early performance of (18)F-FDG PET imaging in patients with possible colitis is encouraging.

Value of ultrasound-guided fine-needle aspiration biopsy of thyroid nodules in an endemic goitre area.

The aim of this study was to determine the value, advantages and limitations of ultrasound-guided fine-needle aspiration biopsy (US-FNAB) in an endemic goitre area. US-FNAB was performed on all outpatients who presented with hypoechoic and/or hypofunctional and/or growing nodules. A total of 4518 US-FNABs were performed and 718 patients from this series underwent surgery. Cytological results of the primarily performed US-FNAB of these patients were compared retrospectively with the histological results. US-FNAB results were grouped as (1) non-malignant (n = 303), (2) non-malignant follicular proliferation (n = 177), (3) malignancy cannot be ruled out (n = 133), (4) malignant (n = 61), (5) inadequate (n = 34), and (6) sampling error; biopsy of a non-malignant nodule (n = 10). Nodules as small as 5 mm in diameter could be biopsied, gaining representative material. US-FNAB found a malignant or suspicious cytology in 65 out of 87 cases with malignant histology (74.71%). Diagnosis of early tumour stages was often possible: 12 of 18 thyroid carcinomas biopsied and smaller than 10 mm in diameter had malignant or suspicious cytology (groups 3 and 4). US-FNAB was performed incorrectly within non-malignant nodules in ten patients (1.39%) with multinodular goitre (ten papillary carcinomas, nine smaller than 10 mm). Regarding the cytology of groups 1 and 2 as benign and those of groups 3 and 4 as malignant, US-FNAB performance was as follows: sensitivity 87.84%, specificity 78.50%, negative predictive values 98.13%, positive predictive values 33.51% and accuracy 79.53%. Biopsies with inadequate material were obtained in 4.73% of all biopsies. No major adverse effects occurred. Re-biopsies in 61 cases did not alter the cytological outcome in those cases where adequate material was obtained. US-FNAB is a valuable method in the pre-operative assessment of thyroid nodules in order to select patients for surgery, as malignancy can often be detected even in early tumour stages. However, even with ultrasonographic guidance, the minimal tumour size detectable by US-FNAB is around 5 mm. The cytological interpretation in cases with regression and microfollicular proliferation also sets limits on the method. However, patients with non-malignant cytologies can be followed up safely by sonography due to the high NPV of US-FNAB as long as thyroid nodules do not become larger. Re-biopsies seem to be of limited value as long as adequate material was obtained by US-FNAB.

Intraspinal primitive neuroectodermal tumour: report of two cases and review of the literature.

Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sacral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, and died 29 months after the first diagnosis. In a 17-year-old male adolescent, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinical, radiological, histopathological and cytogenetic findings of both patients are presented and the relevant literature is reviewed. Particular attention is given to the histogenetic relationship between peripheral primitive neuroectodermal tumour and Ewing's sarcoma.

Cerebral primitive neuro-ectodermal tumour following treatment of a unilateral retinoblastoma.

Cerebral primitive neuro-ectodermal tumour (PNET) occurring as a second primary malignancy in childhood is exceedingly rare. We present a 7-year-old boy who developed a proven supratentorial PNET five years after enucleation and radio-/chemotherapy for a sporadic, unilateral retinoblastoma with optic nerve invasion. The association with this malignant eye disease as well as the effect of irradiation and multi-agent chemotherapy on second tumour induction are evaluated.

Cytomorphology of anaplastic ependymoma in a pleural effusion. A case report.

In a 42-year-old women a recurrent anaplastic ependymoma of the frontal lobe developed intracerebral metastases and, after implantation of an atrioventricular shunt, also metastasized to the lung, pleural cavity and mediastinal lymph nodes. In routinely stained smear preparations of the pleural effusion sediment, the typical cytologic features of an anaplastic ependymoma were found--papilliform cell clusters with acellular axes ("pseudorosettes"), incomplete true ependymal rosettes, and epithelioid and tanycytoid tumor cells with signs of anaplasia. A peculiar matrix material was a characteristic feature of this case and, to our knowledge, was not reported previously. It consisted of refringent, fibrillar strands composed of tightly packed, roundish to rodshaped granules. This matrix seemed to be associated with the cytoplasmic processes of the tumor cells.