Malignant vestibular schwannoma with intracerebral metastasis.

BACKGROUND: Malignant change in vestibular schwannoma is rare and intracranial metastatic deposits have not been reported. CLINICAL PRESENTATION: We report the case of a 64 year old woman with a benign vestibular schwannoma who underwent translabyrinthine excision in 1996 and Gamma Knife® radiosurgery (Elekta AB, Stockholm, Sweden) (GKRS) in 2006. She presents 10 years after GKRS with progressive neurological deterioration. Histopathologic analysis confirms a malignant peripheral nerve sheath tumour, WHO grade IV with subsequent metastatic spread to the left thalamus confirmed on biopsy. CONCLUSION: We report a rare case of a vestibular schwannoma metastasizing with histological confirmation. It also reminds us of malignant conversion of a benign vestibular schwannoma following GKRS and subsequent aggressive behaviour, with poor prognosis.

Meningioma with rhabdoid features combined with meningioangiomatosis in infancy: a novel combination.

Meningioangiomatosis is a rare histologically distinct abnormality that is occasionally associated with intracranial meningioma. The rhabdoid variant of meningioma is also uncommon and is classified as a World Health Organization Grade III tumour. We report a case of meningioangiomatosis in conjunction with a meningioma with prominent rhabdoid features, in an infant male who underwent complete surgical resection of the lesion. The patient has been followed up for 6 years with no disease recurrence. To our knowledge, this is the first report in the literature describing meningioangiomatosis combined with a meningioma with rhabdoid features.

The effect of the revised WHO classification on the incidence of grade II meningioma.

Introduction: A retrospective study over a three-year period at University Hospital of Wales (UHW) of the incidence of atypical cranial grade II meningioma both pre and post 2016 revision of The World Health Organisation (WHO) classification of tumours of the central nervous system.Subjects: All available histology from January 2015 to December 2017 of patients with cranial meningiomas.Method: Institutional online reporting system Welsh Clinical Portal to identify patients and view histology reports.Results: Overall 164 patient histology results were analysed (median age 59, age range 23-82, 74% females). There were 55 patients in 2015: 69.1% grade I, 29.1% grade II and 1.8% grade III. There were 109 patients in 2016/17; 68.8% grade I, 29.4% grade II, 1.8% grade III.Discussion: There is significant variability in the reported incidence of grade II meningioma, likely due to variation in local interpretation of diagnostic criteria. Neuropathologists at our institution have reported brain invasion as grade II prior to 2016. This was due to compelling published evidence that brain invasive meningiomas have recurrence and mortality rates similar to that of grade II meningioma as defined using other criteria. The new 2016 WHO criteria now recognise this specifically. As other institutions adapt to the amended 2016 guidelines we anticipate that there will be a greater consensus in line with our incidence rates of grade II meningioma.

Pineal parenchymal tumours of intermediate differentiation - An evidence-based review of a new pathological entity.

Pineal region lesions are uncommon, and pineal parenchymal tumours (PPT) account for 20-30% of tumours in this area of which pineocytomas (PCs) and pineoblastomas (PBs) are more prevalent. In 2007, the World Health Organisation (WHO) reclassified PPT from two subgroups (PC and PB) into four, including pineal parenchymal tumours of intermediate differentiation (PPTID). PPTID have been further divided into low- and high-grade lesions (WHO II and III), but due to their rarity have proven difficult lesions to diagnose and a paucity of literature means their optimal treatment options are a challenge to define. This article is a review of the literature of PPTID highlighting diagnostic criteria, a discussion on the role of surgery and radiotherapy, including treatment paradigms and reported outcomes for these problematic neoplasms.

Dense calcification in a GH-secreting pituitary macroadenoma.

UNLABELLED: A 30-year-old female presented with a history of secondary amenorrhoea, acromegalic features and progressive visual deterioration. She had elevated serum IGF1 levels and unsuppressed GH levels after an oral glucose tolerance test. Magnetic resonance imaging revealed a heterogeneously enhancing space-occupying lesion with atypical extensive calcification within the sellar and suprasellar areas. Owing to the extent of calcification, the tumour was a surgical challenge. Postoperatively, there was clinical, radiological and biochemical evidence of residual disease, which required treatment with a somatostatin analogue and radiotherapy. Mutational analysis of the aryl hydrocarbon receptor-interacting protein (AIP) gene was negative. This case confirms the relatively rare occurrence of calcification within a pituitary macroadenoma and its associated management problems. The presentation, biochemical, radiological and pathological findings are discussed in the context of the relevant literature. LEARNING POINTS: Calcification of pituitary tumours is relatively rare.Recognising calcification in pituitary adenomas on preoperative imaging is important in surgical decision-making.Gross total resection can be difficult to achieve in the presence of extensive calcification and dictates further management and follow-up to achieve disease control.

Cerebellar pilocytic astrocytoma in adults: a management paradigm for a rare tumour.

BACKGROUND: Pilocytic astrocytoma is one of the commonest subtypes of glioma to affect children. However, they are rarely diagnosed in patients over the age of 18 years. In adults, these tumours appear more frequently supra-tentorially than in the cerebellum and some reports suggest a different clinical course in adults. We reviewed ten patients aged 18 or over who had been operated on for cerebellar pilocytic astrocytoma to assess the impact of tumour biology and extent of resection on outcome in adults. METHOD: Patients were identified from a neuropathology database and a retrospective chart review of ten patients was performed. Recorded data included patient demographics, tumour location, presenting features, radiological appearance, extent of surgical resection, tumour recurrence and Ki-67 proliferation index. RESULTS: Nine patients were men and one patient was a woman. Median follow up is 41.5 months (range 15-334 months). Complete surgical resection was achieved in nine of the patients operated in our institution. One patient had prior subtotal resection elsewhere. Tumour recurrence was seen only in the two patients with subtotal resection, at 7 and 25 years. Ki-67 ranged from <1 to 10% and appears to have no correlation to recurrence. No patients in this series had adjuvant treatment. CONCLUSIONS: Cerebellar pilocytic astrocytomas in adults should be treated with macroscopic complete surgical resection whenever possible. If this is achieved, long-term survival rates are excellent, whereas subtotal resection carries a high risk of tumour recurrence. Ki-67 is less important prognostically than the extent of initial resection.