We report a rare case of a giant extradural and intraabdominal ganglioneuroblastoma in a young Malay girl who presented to a paediatrician initially at 5 days of life with a palpable abdominal mass. Unfortunately, the parents refused any form of surgical intervention until the child was 3 years old. She subsequently underwent vascular embolisation followed by the removal of this large tumour both via the abdomen and through a laminectomy approach and subsequently refused chemotherapy. The c-myc amplication in this patient was absent and there were no chromosomal aberrations, During the 2 year folow-up the patient remained well, and ambulatory with no tumour recurrence.
A 28-year-old Malay man presented with progressive paraparesis over a period of 6 months. Magnetic resonance imaging of the spine revealed a thoracic intramedullary spinal cord tumor at the T-7 level with homogeneous enhancement following intravenous gadolinium administration. Laminectomy and partial decompression of the tumor was performed. Histological examination of the tumor revealed features of spindle cell hemangioendothelioma. The patient was managed with limited field radiotherapy followed by systemic interferon therapy. Good neurological improvement was seen subsequently. The patient has survived 48 months with growth restraint at the primary site, although residual neurological deficit persists. Immunotherapy should be considered as a treatment modality for intramedullary hemangioendothelioma of the spinal cord after surgery and radiotherapy.
Cystic spinal or ancient schwannoma is a rare form of intradural tumour especially in the conus medullaris region. Due to its indolent behavior and benign course, the diagnosis of schwannoma is always a challenge and the imaging findings can be misleading. Our patient presented with chronic mild sciatica pain without any other neurological symptom. MRI of the spine showed intradural tumour located at the conus medullaris region with nerves compression and was reported as ependymoma. L3-L5 laminectomy and total excision of tumour was performed without any neurological complication. We concluded that ancient schwannoma of the conus medullaris is a rare benign tumour that can present with minimal non-specific neurological symptom that lead to misdiagnosis. Radical tumour excision is safe with an excellent outcome.
Primary intramedullary germinoma arising in the cervical spinal cord is a very rare entity. We present one such case arising in a young man who presented with radiculopathy and myelopathy, which was partially excised. Upon histological confirmation, he was treated successfully with radiotherapy alone. To our knowledge, this is only the second reported case worldwide which is histologically confirmed. Although extremely rare, differential diagnosis for intramedullary spinal cord tumor should include germinoma.
Giant nerve sheath tumour is a rare tumour originating from the nerve sheath. It differs from the conventional nerve sheath tumour only by the size these tumours can reach. There are two main type of tumours that occur in the nerve sheath which include neurofibroma and schwannoma. The current views are that schwannomas arise from the progenitor of the schwann cell. Whereas the neurofibroma series probably arise from a mesenchymal origin closer to a fibroblast. We report on six cases of nerve sheath tumour that occur in the spinal and paraspinal region that presented to us over a 5 year period.
A 28-year old Malay man with evidence of an upper motor neuron cord lesion was diagnosed to have a C7 to T2 spinal arterio-venous malformation and associated cutaneous vascular lesion. He finally agreed for treatment after 5 years of progressive spastic right lower limb weakness leading to inability to mobilize. A two staged intravascular procedure was done followed by surgery with recovery of ASIA impairment scale grade B.
The incidence of neurenteric cyst (NC) is rare, accounting for 0.3%-1.3% of all spine tumors. The occurrence of quadriplegia caused by NC is even scarcer. Herein we report on a young girl with a rare NC over the C2-C5 spinal cord, which led to the morbidity of permanent quadriplegia despite early surgical intervention. This case highlights the rare morbidity of cervical cord NC presenting with permanent quadriplegia that failed to respond despite early surgical excision.
One hundred and forty-one patients with non-traumatic paraplegia were reviewed. The common causative factors and the problems arising were evaluated. Management of the clinical problems were described. The need for prevention and early treatment has been stressed.
This study reviewed the epidemiology of brain and spinal tumours in Sarawak from January 2009 till December 2012. The crude incidence of brain tumour in Sarawak was 4.6 per 100,000 population/year with cumulative rate 0.5%. Meningioma was the most common brain tumour (32.3%) and followed by astrocytoma (19.4%). Only brain metastases showed a rising trend and cases were doubled in 4 years. This accounted for 15.4% and lung carcinoma was the commonest primary. Others tumour load were consistent. Primitive neuroectodermal tumour (PNET) and astrocytoma were common in paediatrics (60%). We encountered more primary spinal tumour rather than spinal metastases. Intradural schwannoma was the commonest and frequently located at thoracic level. The current healthcare system in Sarawak enables a more consolidate data collection to reflect accurate brain tumours incidence. This advantage allows subsequent future survival outcome research and benchmarking for healthcare resource planning.
An unusual case of syringomyelia secondary to a cauda equina meningioma involving the conus medullaris is described. The tumour was totally removed with decompression of an adjacent cyst and syrinx resulting in resolution of the symptoms and radiological appearance.