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

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.

CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report

“…The PMT-MCT frequently has an infiltrating growth pattern, as reported in the present cases, and this may explain the high rates of local recurrence ( 44 , 60 – 64 ). Early, complete surgical resection remains the definitive treatment of PMT-MCTs.…”

Sinonasal Phosphaturic Mesenchymal Tumor: Case Report and Systematic Review

“…However, not all PMTs cause this paraneoplastic syndrome (Table 1). 2,14,19,33,34,42,43 In a large review of 109 mesenchymal tumors by Folpe et al, only three PMTs did not demonstrate TIO. 2 Given the sparse case reports of PMTs without TIO in the literature, it is plausible that these cases are underreported.…”

“…There are 17 cases of sinonasal PMT reported in the literature, and these are summarized in Table 1. 1,4,7–35 Histopathologically, PMTs are composed of spindle-shaped or stellate cells with low nuclear grade embedded in a mesenchymal myxoid or myxochondroid matrix with “grungy” or flocculent calcification. 2 A notable feature of PMT is the rich intrinsic microvascular supply, which mimics hemangiopericytoma.…”

Sinonasal Phosphaturic Mesenchymal Tumor: A Rare and Misinterpreted Entity

“…Only 17 prior sinonasal cases have been reported in the literature and are outlined in Table 1 . 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 Tumor-induced osteomalacia is typically caused by a wide range of benign and malignant mesenchymal tumors such as giant cell tumor, nonossifying fibroma, osteoblastoma, and chondroma. 38 …”