Syed M I, Chatzimichalis M, Rössle M, Huber A M
Department of Otolaryngology, The Royal Infirmary, Edinburgh, Scotland, UK.
J Laryngol Otol. 2012 Jul;126(7):721-4. doi: 10.1017/S0022215112000989. Epub 2012 May 29.
We describe the first reported case of a phosphaturic mesenchymal tumour, mixed connective tissue variant, invading the temporal bone.
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.
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.
我们描述了首例有文献报道的侵犯颞骨的混合性结缔组织变体的磷酸酶分泌性间叶肿瘤病例。
一名女性患者出现听力逐渐下降。检查发现鼓膜完整，但鼓膜后似乎有肿物。进一步的影像学检查显示，一个血管性肿物占据了中耳、乳突和内耳道。该肿物经手术切除，病理显示为良性磷酸酶分泌性间叶肿瘤，混合性结缔组织变体。一年后肿瘤复发，表现为面神经麻痹。进行了翻修手术；切除肿瘤时牺牲了一段面神经，并进行了面-舌下神经吻合术。
本病例报告强调了这种良性但有时具有侵袭性的肿瘤的发生情况，临床医生和病理学家都应予以关注。尽早识别该疾病对于将受影响患者长期骨软化症的致残后果降至最低，以及预防肿瘤引起的颅外和颅内并发症至关重要。
