Journal article
Recurrent upper limb neuropathies secondary to an epithelioid haemangioendothelioma – A rare mimic of nerve tumours
Journal of Clinical Neuroscience, Vol.73, pp.326-328
2019
Abstract
Epithelioid haemangioendothelioma (EHE) is an uncommon type of vascular tumour with intermediate malignant potential, classified as a sarcoma which occasionally involves neurovascular structures and can mimic nerve sheath tumours. EHE is difficult to distinguish from other nerve sheath tumours based on imaging, including MRI, and biopsy is often required for diagnosis. Diagnosis of EHE from biopsy often requires the use of vascular immunohistochemical stains. We present a case of left upper limb neurovascular bundle EHE presenting with proximal ulnar nerve neuropathy and subsequent median nerve neuropathy and liver, lungs and bone metastases. The tumour had been identified 20 years prior with a similar presentation of upper limb weakness and sensory disturbance, yet following surgical excision it was misdiagnosed as inflammatory fibrous tissue. Treatment with propranolol has resulted in disease stability and surgical debulking resulted in improved upper limb function. The use of beta-adrenergic receptor antagonists in EHE and other sarcomas have been shown to increase T-cell infiltration and decrease immunosuppressive PD-1 expression in neoplastic cells.
Details
- Title
- Recurrent upper limb neuropathies secondary to an epithelioid haemangioendothelioma – A rare mimic of nerve tumours
- Authors/Creators
- J.D. Triplett (Author/Creator)T.S. Khor (Author/Creator)A.G. Kermode (Author/Creator)
- Publication Details
- Journal of Clinical Neuroscience, Vol.73, pp.326-328
- Publisher
- Churchill Livingstone
- Identifiers
- 991005541546507891
- Copyright
- © 2019 Elsevier Ltd.
- Murdoch Affiliation
- Institute for Immunology and Infectious Diseases
- Language
- English
- Resource Type
- Journal article
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- Collaboration types
- Domestic collaboration
- Citation topics
- 1 Clinical & Life Sciences
- 1.118 Soft Tissue, Bone & Nerve Cancers
- 1.118.1928 Angiosarcoma
- Web Of Science research areas
- Clinical Neurology
- Neurosciences
- ESI research areas
- Neuroscience & Behavior