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Recurrent phosphaturic mesenchymal tumour of the temporal bone causing deafness and facial nerve palsy

Published online by Cambridge University Press:  29 May 2012

M I Syed ,
M Chatzimichalis ,
M Rössle  and
A M Huber
M I Syed*
Affiliation:
Department of Otolaryngology, The Royal Infirmary, Edinburgh, Scotland, UK
M Chatzimichalis
Affiliation:
Department of Otolaryngology, University Hospital Zurich, Switzerland
M Rössle
Affiliation:
Institute of Clinical Pathology, University Hospital Zurich, Switzerland
A M Huber
Affiliation:
Department of Otolaryngology, University Hospital Zurich, Switzerland
*
Address for correspondence: Mr M I Syed, Department of Otolaryngology, The Royal Infirmary, Edinburgh E16 4SA, Scotland, UK Fax: +44 (0)131 536 3757 E-mail: [email protected]
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Abstract

Objective:

We describe the first reported case of a phosphaturic mesenchymal tumour, mixed connective tissue variant, invading the temporal bone.

Case report:

A female patient presented with increasing deafness. On examination there appeared to be a mass behind an intact tympanic membrane. Further radiological investigation showed a vascular mass occupying the middle ear, mastoid and internal auditory meatus. This was surgically resected and revealed to be a benign phosphaturic mesenchymal tumour, mixed connective tissue variant. The tumour recurred a year later, presenting as facial nerve palsy. A revision procedure was carried out; the tumour was excised with the sacrifice of a segment of the facial nerve, and a facial-hypoglossal nerve anastomosis was performed.

Conclusion:

This case report highlights the occurrence of this benign but sometimes aggressive tumour, of which both clinicians and pathologists should be aware. Early recognition of the condition remains of utmost importance to minimise the debilitating consequences of long-term osteomalacia in affected patients, and to prevent extracranial and intracranial complications caused by the tumour.

Keywords

Temporal BoneEar, MiddleFacial ParalysisMalignant Mesenchymal TumorOncogenic Osteomalacia
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 126 , Issue 7 , July 2012 , pp. 721 - 724
Copyright
Copyright © JLO (1984) Limited 2012

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