Mark A J Dexter

Quality of Life in Postoperative Vestibular Schwannoma Patients

To quantify the postoperative quality of life (QOL) in patients following vestibular schwannoma surgery in a new multidisciplinary skull base unit.

Usefulness of smears in intra-operative diagnosis of newly described entities of CNS

The recent edition of World Health Organisation (WHO) Classification of Tumours of the Central Nervous System has incorporated a substantial number of important changes. It has recognised several new entities, many of which are rare. Intra‐operative diagnosis of these tumours can be difficult with the freezing artefact that often cripples brain frozen sections. In many instances intra‐operative smears are extremely useful adjuvants in neuropathological diagnosis. In this article, we describe intra‐operative smear findings of three of the newly described tumours. Their characteristic cytologic features are illustrated along with differentiating features from the common mimics, together with a general approach to brain smears. The entities we discuss here are papillary glioneuronal tumour, papillary tumour of the pineal region and angiocentric glioma. All three tumours share at least focal pseudo‐papillary/vasculocentric architecture. Smears from papillary glioneuronal tumour demonstrated dual population of cells in a neuropil background, whereas papillary tumour of the pineal region and angiocentric glioma comprise a single population of cells. These two tumours can further be differentiated based on their cell morphology and background.

Extraventricular neurocytoma with atypical features and ganglionic differentiation

Extraventricular neurocytoma (EVN) is an unusual variant of central neurocytoma located outside the ventricular system. Both tumours share a similar histology characterised by monotonous populations of round-to-oval cells with scanty cytoplasm separated by neuropil and branching capillaries. We report an EVN arising from the right frontal lobe near the olfactory tract in a 34-year-old male with worsening chronic epilepsy. Our patients tumour exhibited many uncommon features including ganglionic differentiation, increased mitotic activity and a high proliferative index. We discuss the important differential diagnoses given the site of the tumour as well as the differentiating features from olfactory neuroblastoma, oligodendroglioma, anaplastic ganglioglioma and supra-tentorial primitive neuroectodermal tumour.

Histiocytic sarcoma of the brain

Histiocytic sarcoma is a rare malignant neoplasm of the lympho-hematopoietic system that usually occurs in the skin, lymph nodes and intestinal tract. We present a 36-year-old woman with a rare histiocytic sarcoma with isolated central nervous system (CNS) involvement of multifocal circumscribed lesions. Biopsy of the brain lesions showed diffuse proliferation of pleomorphic histiocytes that were immunopositive for CD45, CD68 and CD163. Various cytokeratins and markers of lymphoma, melanoma, germ cell tumours and primary CNS tumours were negative. Examination of bone marrow trephine and a whole-body positron emission tomography scan showed no evidence of involvement of any other organ systems, thus establishing the primary nature of the lesion. The neoplastic cells uniquely showed eosinophilic globules within the cytoplasm, which were positive for CD68. These globules were shown by electron microscopy to be collections of lysosomes. A thorough discussion of the differential diagnosis and literature review is included.

The surgical management of contralateral anterior circulation intracranial aneurysms

This study reviews the surgical management of contralateral anterior circulation aneurysms in patients with bilateral intracranial aneurysms repaired following a unilateral craniotomy. Between 1993 and 1999, 27 patients had 88 intracranial aneurysms repaired. Eleven patients presented following subarachnoid haemorrhage. Excluding midline aneurysms, 31 anterior circulation aneurysms were contralateral to the craniotomy and all were repaired at the same time that ipsilateral or midline aneurysms were repaired. Morbidity included one death and one case of loss of unilateral vision directly attributable to surgery and two cases of cerebral infarction due to vasospasm. No new neurological deficit or mortality could be directly attributed to the repair of a contralateral aneurysm. The repair of all accessible aneurysms, including those contralateral to the craniotomy, during one session avoids the risk of haemorrhage from incidental or unrecognised ruptured aneurysms (particularly during the aggressive treatment of vasospasm), avoids a second craniotomy, decreases overall hospitalisation and can improve visualisation of carotid-ophthalmic aneurysms.

Intraventricular rhabdoid meningioma

Rhabdoid meningioma is a rare variant of meningioma, often found in tumour recurrences. We report a 55-year-old woman with a history of intraventricular fibroblastic meningioma, who developed headache and tinnitus 5 years after complete resection of the initial tumour. Imaging confirmed a recurrent tumour in the intraventricular location. Histological analysis revealed rhabdoid meningioma. We reviewed the literature and were unable to find any previously reported cases of intraventricular rhabdoid meningioma.

Late onset Rasmussen's encephalitis with triple pathology

Rasmussens encephalitis is a devastating illness characterized by intractable focal seizures due to chronic localised encephalitis. We report on a rare variant of delayed onset Rasmussens encephalitis with triple pathology. A 27-year-old male, who was initially diagnosed with seizures when he was 16 years old, presented with focal seizures that became refractory to multiple anticonvulsants. Multiple investigations, including subdural electrode monitoring, revealed foci of onset in the right frontotemporal region. The patient underwent right front-temporal lobectomy. Post-operatively, the seizures became more severe and he developed new epilepsia partialis continua. Treatment with intravenous immunoglobulin was unsuccessful. He subsequently underwent a right hemispherectomy that rendered him seizure free. The three pathologies identified were old ischemic changes, type II cortical dysplasia and stage II Rasmussens encephalitis.

Microvascular decompression for elderly patients with trigeminal neuralgia

Microvascular decompression (MVD) has been demonstrated to be an excellent surgical treatment approach in younger patients with trigeminal neuralgia (TN). However, it is not clear whether there are additional morbidity and mortality risks for MVD in the elderly population. We performed a systematic literature review using six electronic databases for studies that compared outcomes for MVD for TN in elderly (cut-off ⩾60, 65, 70years) versus younger populations. Outcomes examined included success rate, deaths, strokes, thromboembolism, meningitis, cranial nerve deficits and cerebrospinal fluid leaks. There were 1524 patients in the elderly cohort and 3488 patients in the younger cohort. There was no significant difference in success rates in elderly versus younger patients (87.5% versus 84.8%; P=0.47). However, recurrence rates were lower in the elderly (11.9% versus 15.6%; P=0.03). The number of deaths in the elderly cohort was higher (0.9% versus 0.1%; P=0.003). Rates of stroke (2.5% versus 1%) and thromboembolism (1.1% versus 0%) were also higher for elderly TN patients. No differences were found for rates of meningitis, cranial nerve deficits or cerebrospinal fluid leak. MVD remains an effective and reasonable strategy in the elderly population. There is evidence to suggest that rates of complications such as death, stroke, and thromboembolism may be significantly higher in the elderly population. The presented results may be useful in the decision-making process for MVD in elderly patients with TN.

Diagnostic pitfall in the diagnosis of mesenchymal chondrosarcoma arising in the central nervous system.

Mesenchymal chondrosarcoma is a rare aggressive neoplasm typically affecting the bones of young adults. It may also arise in somatic soft tissue, the CNS and other organs. It has a characteristic biphasic histological pattern composed of highly undifferentiated small round cells and islands of well‐differentiated hyaline cartilage. We report a case of mesenchymal chondrosarcoma arising from the right tentorium cerebelli in a 21‐year‐old woman with symptoms relating to mass effect. Histological examination demonstrated a purely small round cell appearance in a specimen obtained during partial resection at an outside institution, leading to an erroneous diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (PNET). The diagnosis of mesenchymal chondrosarcoma was made only after tissue obtained during a definitive complete macroscopic removal involving the regional tentorium cerebelli, transverse and sigmoid dural venous sinuses which showed a prominent cartilaginous component. We discuss the features of mesenchymal chondrosarcoma arising in the CNS, the important differential diagnoses of small round‐cell tumors within the CNS, and the differentiating features of mesenchymal chondrosarcoma from Ewing sarcoma/PNET, medulloblastoma, hemangiopericytoma, monophasic synovial sarcoma and atypical teratoid/rhabdoid tumour.

The Vestibular Schwannoma Surgery Learning Curve Mapped by the Cumulative Summation Test for Learning Curve

Objective To demonstrate and quantify the learning curve for microsurgical excision of vestibular schwannoma in a newly formed neurootologic team by using the cumulative summation test for learning curve (LC-CUSUM). To secondarily identify the factors influencing postoperative facial nerve outcome. Study Design Retrospective review. Setting Tertiary referral center. Patients Between 1999 and 2011, 153 consecutive cases of vestibular schwannoma excision. Intervention One-hundred and fifty-three patients underwent excision of vestibular schwannoma. Main Outcome Measures Facial nerve outcomes were assessed using the House-Brackmann (HB) facial nerve grading system. Postoperative facial nerve outcomes at 12 months were analyzed using the LC-CUSUM method with HB Grades I to III being defined as successful outcomes. The factors that influence postoperative facial nerve outcome were analyzed. Results The constructed learning curve shows a gradual improvement in facial nerve outcomes. The learning curve crossed the derived LC-CUSUM barrier at the 56th procedure, indicating that sufficient evidence had accumulated to demonstrate that the surgeon had achieved optimal outcomes at this point. Tumor size (p = 0.008) and surgical approach (p = 0.005) were 2 additional significant factors influencing postoperative facial nerve outcome. Conclusion The learning curve is evident in this series of microsurgical excisions of vestibular schwannoma. A newly formed team needs to perform at least 56 cases to gain sufficient experience to accomplish optimal results. Position along the learning curve, tumor size, and familiarity with a preferred surgical approach are the factors, which dominated facial nerve outcome. We recommend the use of LC-CUSUM test for learning curve analysis.