Recurrent upper limb neuropathies secondary to an epithelioid haemangioendothelioma – A rare mimic of nerve tumours

James D. Triplett, Tze S. Khor, Allan G. Kermode

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalJournal of Clinical Neuroscience
DOIs
Publication statusE-pub ahead of print - 7 Nov 2019

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Epithelioid Hemangioendothelioma
Upper Extremity
Nerve Sheath Neoplasms
Neoplasms
Sarcoma
Blood Vessels
Median Neuropathy
Ulnar Neuropathies
Biopsy
Ulnar Nerve
Adrenergic Antagonists
Median Nerve
Receptors, Adrenergic, beta
Immunosuppressive Agents
Diagnostic Errors
Propranolol
Coloring Agents
Neoplasm Metastasis
T-Lymphocytes
Bone and Bones

Cite this

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title = "Recurrent upper limb neuropathies secondary to an epithelioid haemangioendothelioma – A rare mimic of nerve tumours",
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.",
keywords = "Epithelioid haemangioendothelioma, Magnetic resonance imaging, Mononeuropathy, Upper limb, Vascular tumour",
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AB - 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.

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