Andrew Hunn

Intramedullary Spinal Cord Metastases: A 20-Year Institutional Experience with a Comprehensive Literature Review

OBJECTIVES To review previous reports as well as our institutional experience to address the issues regarding patient management and also to assess the predisposing factors that might influence outcome and survival. METHODS We undertook a 20-year (1989-2009) retrospective study of a series of eight patients diagnosed with intramedullary spinal cord metastases (ISCMs) in our institute. We further reviewed 293 cases of ISCMs reported in the English literature since 1960. Characteristics regarding the site of the primary cancer, location of ISCM, the presence of other metastases, presenting neurological symptoms/signs, duration of symptoms, and the time interval from diagnosis of the primary tumor to ISCM were pooled. We analyzed the different treatment approaches, the functional outcome, and the factors influencing survival. RESULTS Lung and breast cancers appear to be the most frequent source of ISCM with cervical, thoracic, and lumbar spine being equally affected. Motor weakness predominates as the commonest symptom at presentation, followed by pain and sensory disturbance. At diagnosis, most patients with ISCM have a known primary cancer often associated with cerebral and other systemic metastases. Overall survival of ISCM is poor (median: 4 months from the time of diagnosis). Survival in surgical patients is 6 months, compared with 5 months in those conservatively managed. Clinical improvement was observed in more than one-half of those treated surgically, whereas neurological status was maintained in most patients treated conservatively. CONCLUSION ISCM is an unusual site for metastasis. Regardless of the treatment, its prognosis is generally poor as its presence often signifies end-stage cancer. However, with early diagnosis and appropriate treatment, selected patients may benefit from improved neurological outcome and quality of life.

Extracranial metastases of a glioblastoma multiforme to the pleura, small bowel and pancreas.

Extracranial metastases of glioblastoma multiforme (GBM) are rare and usually occur in the context of recurrent intracranial GBM. We present a 39-year-old man with histologically confirmed GBM. The patient remained well for nearly 2 years, with no signs of recurrent tumour. He then presented with distant recurrence within the brain at the same time as developing pneumonia and epigastric pain. A computed tomography scan of the patients abdomen and chest showed several intra-abdominal masses, including one in the head of the pancreas as well as a separate mass at the base of the left lung. A computed tomography-guided biopsy of the pancreatic mass demonstrated histological appearances identical to those of the original GBM. This unusual case raises the possibility of a link between prolonged survival with GBM and the occurrence of extracranial disease.

Atypical meningiomas - A case series

Meningiomas, in particular the Atypical (grade 2), vary greatly in their behaviour and prognosis. Over a 19 year period, we operated on 169 meningiomas (on 86 patients) and of those, 9 cases of atypical meningiomas were found which met the 2007 World Health Organization (WHO) classification. The 9 patients represented 5.3% of all meningiomas. The average presenting age was 51 years and average follow-up was 103 months with 5 patients passing away between 38 and 219 months after diagnosis. The time to first recurrence was 24 months with 1 patient suffering 12 recurrences and 2 cases having metastases. Although we had a small number of atypical meningiomas, we believe our paper highlights the unpredictable and difficult nature of these tumours.

Ventriculoperitoneal shunt blockage due to spontaneous knot formation in the peritoneal catheter : Case report

The authors report the third case of ventriculoperitoneal shunt blockage due to spontaneous knot formation in the peritoneal catheter that had been placed in a 3.5-year-old boy 8 months earlier. On surgical exploration a double knot was found 10 cm from the distal end of the peritoneal catheter. Although the underlying mechanism remains unknown, the authors used the analogy of related physical studies and true knot formation in the umbilical cord and determined the possible causes as related to the catheter, volume and configuration of the abdomen, and kinetics of the catheter movements. If further study should reveal a significantly higher incidence of this complication, the authors suggest further in vitro studies, designed to investigate the optimal characteristics and safe range of length of peritoneal catheters in different situations.

Non-neoplastic demyelinating process mimicking a disseminated malignant brain tumour.

Non-neoplastic demyelinating processes of the brain with ring enhancing lesions and mass effect on MRI imaging, mimicking malignant brain tumours, are rare phenomena. We document the case of a 32 year old male with clinical, radiological and initial histological findings, suggestive of a malignant brain tumour. Additional investigations confirmed the diagnosis of multiple sclerosis. This case is significant as the lesion could not be easily distinguished from a malignant brain tumour on imaging alone. Cases such as this illustrate the importance of considering a demyelinating process in the differential diagnosis of tumour-like brain lesions.

Pre-operative embolisation of metastatic paraganglioma of the thoracic spine

We report a 46-year-old male with metastatic functioning paraganglioma of the thoracic spine. Thoracic spine functioning paragangliomas are rare and we describe the benefits of embolisation of the lesion prior to surgical decompression.

An intraneural ganglion cyst causing unilateral hypoglossal nerve palsy

Intracranial ganglion cysts are rare. We report a patient with a rare unilateral hypoglossal nerve palsy caused by an intraneural ganglion cyst. To our knowledge, there are only four reports of ganglion/synovial cysts causing unilateral hypoglossal nerve palsy. Our aim is to present the fifth report, and to compare our findings with the others.

Seat belt syndrome with unstable Chance fracture dislocation of the second lumbar vertebra without neurological deficits

The seat belt syndrome is a recognised complication of seat belt use in vehicles. Unstable Chance fractures of the spine without neurological deficits have been reported infrequently. We describe a young woman with completely disrupted Chance fracture of the second lumbar vertebra in association with left hemidiaphragmatic rupture/hernia, multiple bowel perforations, splenic capsular tear, left humeral shaft and multiple rib fractures. These injuries which resulted from high-speed vehicle collision and led to death of one of the occupants were readily detected by trauma series imaging. The patient was successfully treated by a dedicated multidisciplinary team which adopted a staged surgical approach and prioritisation of care. There were no manifested neurological or other deficits after 1 year of follow-up. To the authors’ knowledge, this is the first report of such a case in Australasia. We discuss the challenging surgical management, highlighting the role of radiological imaging in such cases and provide a literature review.

A rare association of cerebral dural arteriovenous fistula with venous aneurysm and contralateral flow-related middle cerebral artery aneurysm.

The association of cerebral dural arteriovenous fistula (DAVF) and ipsilateral flow related aneurysm has infrequently been reported. We describe a male patient who presented with an acute haemorrhagic stroke and was found to have a large right fronto-parietal intra-parenchymal haemorrhage from the ruptured Borden type II DAVF in addition to a large venous aneurysm and a flow related intraosseous aneurysm of the contralateral middle meningeal artery (MMA) all clearly delineated by CT and DSA. He underwent emergency stereotactic evacuation of the intraparenchymal haemorrhage and successful surgical treatment of all the vascular lesions at the same time with residual neurological deficit. To our knowledge, this is the first such reported case. We discuss the challenging surgical treatment, emphasising the role of CT/DSA in management, and provide a literature review.

Adult polycystic kidney disease and arachnoid cyst formation: Case report.

A 34 year old man is reported with adult polycystic kidney disease with an associated extensive arachnoid cyst occupyingmost of his left hemicranium. The aetiology of arachnoid cyst formation is discussed.