Inhibition of YAP/TAZ-driven TEAD activity prevents growth of NF2-null schwannoma and meningioma.

Schwannoma tumours typically arise on the eighth cranial nerve and are mostly caused by loss of the tumour suppressor Merlin (NF2). There are no approved chemotherapies for these tumours and the surgical removal of the tumour carries a high risk of damage to the eighth or other close cranial nerve tissue. New treatments for schwannoma and other NF2-null tumours such as meningioma are urgently required.

Bickerstaff brainstem encephalitis: clinical, neurophysiological, laboratory and postmortem findings of a case presenting as encephalomyelitis.

Our patient was admitted to hospital with a 1-week history of an upper respiratory tract infection and a rapidly progressive encephalopathy dominated by brainstem features and widespread areflexia. Her antiganglioside antibodies and electroencephalography were consistent with Bickerstaff brainstem encephalitis (BBE), and her postmortem examination revealed a predominantly florid brainstem encephalitis and myelitis. Her sputum and throat swabs isolated and respectively, the former being the most probable trigger of BBE.

Ketogenic Metabolic Therapy, Without Chemo or Radiation, for the Long-Term Management of -Mutant Glioblastoma: An 80-Month Follow-Up Case Report.

Successful treatment of glioblastoma (GBM) remains futile despite decades of intense research. GBM is similar to most other malignant cancers in requiring glucose and glutamine for growth, regardless of histological or genetic heterogeneity. Ketogenic metabolic therapy (KMT) is a non-toxic nutritional intervention for cancer management.

EBV+ HHV-8+ Multicentric Castleman Disease With Plasmablastic Aggregates in an HIV+ Man: An Evolving Clinicopathologic Entity.

We report a case of EBV+ and HHV-8+ multicentric Castleman disease with plasmablastic aggregates in an HIV-positive individual. A 41-year-old man presented in early 2015 with fevers, sweats, weight loss, intractable itching, and on subsequent testing was found to be HIV positive. Investigations showed cervical lymphadenopathy and splenomegaly.

Merlin controls the repair capacity of Schwann cells after injury by regulating Hippo/YAP activity.

Loss of the Merlin tumor suppressor and activation of the Hippo signaling pathway play major roles in the control of cell proliferation and tumorigenesis. We have identified completely novel roles for Merlin and the Hippo pathway effector Yes-associated protein (YAP) in the control of Schwann cell (SC) plasticity and peripheral nerve repair after injury. Injury to the peripheral nervous system (PNS) causes a dramatic shift in SC molecular phenotype and the generation of repair-competent SCs, which direct functional repair.

Activation of multiple growth factor signalling pathways is frequent in meningiomas.

A minority of meningiomas are difficult to treat with surgery or radiotherapy, and chemotherapeutic alternatives are limited. This study aims to better understand pathways that are active in meningiomas, in order to direct future treatment strategies. We investigated the expression and activation of multiple growth factor receptors, their ligands and downstream signalling pathways in 30 meningiomas using immunohistochemistry.

A tale of the unexpected: Amyloidoma associated with intracerebral lymphoplasmacytic lymphoma.

Amyloidoma is a rare cause for intracranial space-occupying lesions diagnosed on brain imaging. Histology of excised tissue usually reveals the presence of a discrete, λ-light chain secreting plasmacytoma adjacent to an amyloid mass comprising aggregated monoclonal immunoglobulin light chains. We described a patient with intracerebral amyloidoma associated with a localised lymphoplasmacytic lymphoma and no systemic paraproteinaemia, tumour or amyloid deposits.

Loss of SOX10 function contributes to the phenotype of human Merlin-null schwannoma cells.

Loss of the Merlin tumour suppressor causes abnormal de-differentiation and proliferation of Schwann cells and formation of schwannoma tumours in patients with neurofibromatosis type 2. Within the mature peripheral nerve the normal development, differentiation and maintenance of myelinating and non-myelinating Schwann cells is regulated by a network of transcription factors that include SOX10, OCT6 (now known as POU3F1), NFATC4 and KROX20 (also known as Egr2). We have examined for the first time how their regulation of Schwann cell development is disrupted in primary human schwannoma cells.

Expression of c-Jun and Sox-2 in human schwannomas and traumatic neuromas.

Aims: Schwann cells myelinate axons of the peripheral nervous system. This process of myelination is regulated by various transcription factors. c-Jun and Sox-2 are negative regulators of myelination and control Schwann cell differentiation and plasticity.

Occurrence and distribution of pilomyxoid astrocytoma.

Purpose: To know the occurrence and distribution of Pilomyxoid Astrocytomas amongst tumours previously diagnosed histologically as Pilocytic Astrocytoma and to assess the clinical impact of this new entity.

Methods: Retrospective Diagnostic review of all cases histologically diagnosed as WHO Grade I Astrocytoma at a single Neurosurgical unit between 1990 and 2003.

Results: Of a total of 91 cases identified, 9 were found to have Pilomyxoid histology.

Metastatic malignant melanoma within meningioma with intratumoral infarct: report of an unusual case and literature review.

We report an unusual case of a patient with a sphenoid wing meningioma that after a few years of static radiological appearance presented with sudden deterioration following rapid growth of tumor with intratumoral infarct. The patient underwent surgery and malignant melanoma deposits within the meningioma were demonstrated on histopathological examination. She had a history of a malignant melanoma (MM) excised from the left forearm 10 months ago with no evidence of recurrence.

Paediatric intramedullary spinal cord cavernous malformations: case report and review of the literature.

Cavernous malformations are vascular lesions which can occur throughout the entire neuraxis. This term is synonymous to cavernous angioma, cavernous haemangioma, and cavernoma. They comprise of closely packed, capillary-like vascular channels, without intervening neural tissue.

Primitive neuroectodermal tumour arising within low grade astrocytoma: transformation, de novo or radiation induced? Report of three cases and review of literature.

The transformation from low grade to aggressive astrocytoma is well known. However, the development of a completely different tumour such as a primitive neuroectodermal tumour (PNET) within a low grade astrocytoma (LGA) is rare. Only two cases have been reported to date.

Cervical spine metastasis from adrenal pheochromocytoma.

Metastatic tumours are the most common type of tumours in the spine of which only 10% occur in the cervical spine. We report a rare presentation of metastatic pheochromocytoma of the cervical spine which was successfully treated operatively. Adequate precautions should be taken during surgery as the tumour is very vascular.

A rare case of recurrent secretory meningioma with malignant transformation.

A 72-year-old woman previously operated for a sphenoid-ridge meningioma, now presented with double vision. Histology showed a secretory meningioma with an epithelial-appearing, malignant component. Malignant transformation in a secretory meningioma is not known.

Twenty year review of histopathological findings in enucleated/eviscerated eyes.

Background/aims: To evaluate the need for routine histopathological analysis of enucleated/eviscerated eyes and changes in indications for eye removal.

Methods: Retrospective review of all enucleation/evisceration histopathology reports over 20 years. Clinical history was correlated with pathological findings.