Recurrent phosphaturic mesenchymal tumour of the temporal bone causing deafness and facial nerve palsy.
OBJECTIVE
We describe the first reported case of a phosphaturic mesenchymal tumour, mixed connective tissue variant, invading the temporal bone.


METHODS
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.


CONCLUSIONS
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.
Department of Otolaryngology, The Royal Infirmary, Edinburgh, Scotland, UK. iqbalms@hotmail.com
M I
MI
Syed
M
M
Chatzimichalis
M
M
Rössle
A M
AM
Huber
D000368:Aged; D000714:Anastomosis, Surgical; D003638:Deafness / Q000209:etiology*; D004432:Ear, Middle / Q000473:pathology / Q000601:surgery; D005154:Facial Nerve / Q000601:surgery; D005155:Facial Nerve Diseases / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D005260:Female; D006801:Humans; D007002:Hypoglossal Nerve / Q000601:surgery; D017674:Hypophosphatemia / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008637:Mesenchymoma / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D009364:Neoplasm Recurrence, Local; D009372:Neoplasms, Connective Tissue / Q000150:complications / Q000473:pathology* / Q000601:surgery; D010018:Osteomalacia; D013506:Otologic Surgical Procedures / Q000379:methods; D010257:Paraneoplastic Syndromes; D012086:Reoperation; D012888:Skull Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000601:surgery; D013701:Temporal Bone / Q000473:pathology / Q000601:surgery