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.
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.
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.
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.