Paraneoplastic Paralysis in a Patient With Malignant Fibrous Histiocytoma

SummaryParaneoplastic movement disorders are part of the spectrum of paraneoplastic syndromes caused by the production of onconeural antibodies such as anti-Hu by underlying tumours. These attack specific neurons depending on receptor aetiology. We report the case of a 53-year-old man who presented 8 years previously with symptoms of upper limb weakness, light headedness, dizziness and falls. His condition followed a progressive course. Two years after onset he had right-sided weakness, diplopia and generalised dystonia. Initial investigations identified a positive anti-Hu antibody, but an extensive search for a primary tumour was negative. A malignant fibrous histiocytoma in his right gluteal fold was subsequently identified. At this stage he was bed bound with severe ataxia, dystonia and spasticity. Following surgical excision and treatment with high dose steroids and pulse immunoglobulin, further progression was arrested and minor improvements occurred. He can now ambulate with bilateral assistance but remains severely disabled. BACKGROUND

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Malignant Fibrous Histiocytoma of the Thoracic Spine Manifesting as Rapidly Progressive Compressive Myelopathy -Case Report-

Ochiai

Kawano

Shimao

et al. 2009

Neurol. Med. Chir.(Tokyo)

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A 75-year-old woman presented with an extremely rare case of malignant fibrous histiocytoma (MFH) arising in the thoracic spine, causing compression fracture and manifesting as rapidly progressive compressive myelopathy resulting in progressive gait disturbance and weakness of the bilateral legs. Computed tomography and magnetic resonance imaging demonstrated a solid enhanced intravertebral mass destroying the vertebral bodies at the T7 and T8 levels. Maximum resection of the lesion was performed through wide laminectomy from T7 through T9, followed by vertebroplasty using calcium phosphate paste and posterior fixation with a spinal fixation system. Histological examination showed proliferation of pleomorphic spindle-shaped cells having large irregular nuclei with hyperchromasia with storiform pattern and collagenous stroma, consistent with a diagnosis of MFH. This case illustrates the possibility of rapid maximum decompression surgery from the posterolateral side if MFH manifests as progressive transverse myelopathy.

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Paraneoplastic Paralysis in a Patient With Malignant Fibrous Histiocytoma

Cited by 3 publications

References 13 publications

Paraneoplastic brainstem encephalitis in a patient with malignant fibrous histiocytoma and atypical anti-neuronal antibodies

Paraneoplastic brainstem encephalitis in a patient with malignant fibrous histiocytoma and atypical anti-neuronal antibodies

Paraneoplastic ataxia and dystonia secondary to a malignant fibrous histiocytoma

Malignant Fibrous Histiocytoma of the Thoracic Spine Manifesting as Rapidly Progressive Compressive Myelopathy -Case Report-

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