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Case Reports
. 2014 Aug 5:2014:bcr2014205248.
doi: 10.1136/bcr-2014-205248.

Acquired von Willebrand's disease associated with epithelial myoepithelial carcinoma of the parotid salivary gland

Geoffrey Spencer Chilvers  1 , Graham Porter  1
Affiliations
Case Reports

Acquired von Willebrand's disease associated with epithelial myoepithelial carcinoma of the parotid salivary gland

Geoffrey Spencer Chilvers et al. BMJ Case Rep. .

Abstract

This is the first case report of a patient with acquired von Willebrand's disease (AvWD) secondary to epithelial myoepithelial carcinoma (EMC) of the parotid salivary gland. This patient presented to haematology following an abnormal bleeding episode with von Willebrand factor Ristocetin cofactor (VWF:RCo) <5% and VWF:Ag 13%. He was diagnosed with AvWD. At the same time he was found to have a left parotid lump which was excised following a multidisciplinary team meeting. This was histologically reported as an EMC. Following excision, unusually this patient's AvWD did not resolve. Extensive investigation looking for other causes of the AvWD did not reveal any other potential cause; therefore, due to the similar timing of onset of both pathologies it is felt that the AvWD in this case is secondary to the EMC. This case report highlights the presentation, investigation and management of AvWD and parotid lumps as well as discussing proposed pathophysiological mechanisms for AvWD.

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Figures

Figure 1
Figure 1
MRI with gadolinium enhancement of the head and neck showing the left parotid tumour.
Figure 2
Figure 2
MRI with gadolinium enhancement of the head and neck showing no recurrence of the left parotid tumour.
See this image and copyright information in PMC

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