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


Syed, M I; Chatzimichalis, M; Rössle, M; Huber, A M (2012). Recurrent phosphaturic mesenchymal tumour of the temporal bone causing deafness and facial nerve palsy. The Journal of Laryngology and Otology, 126(7):721-4.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Otorhinolaryngology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2012
Deposited On:18 Sep 2012 12:30
Last Modified:07 Dec 2017 15:04
Publisher:Cambridge University Press
ISSN:1748-5460
Free access at:Official URL. An embargo period may apply.
Publisher DOI:https://doi.org/10.1017/S0022215112000989
Official URL:http://journals.cambridge.org/download.php?file=%2F1859_84361B3A23E578B6E2CC9ADB76717E9F_journals__JLO_JLO126_07_S0022215112000989a.pdf&cover=Y&code=71071067985053fe50aadfcbedad50cd
PubMed ID:22642807

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