• Raúl Sánchez Muñoz, Jose L Sanmillan Blasco, Noemi Vidal Sarro, Guillermo Martin Quintero, Daniel Marti Duce, and Andreu Gabarrós Canals.
• World Neurosurg. 2024 Mar 30.

BackgroundPhosphaturic Mesenchymal Tumors (PMT) are rare mesenchymal neoplasms known for producing Tumor-induced Osteomalacia (TIO). TIO is an uncommon paraneoplastic syndrome characterized by radiographic evidence of inadequate bone mineralization and analytical abnormalites.MethodsWe sought to present a case of TIO caused by skull base PMT with intracranial extension, manifesting with pain, progressive weakness, and multiple bone fractures. Furthermore, a systematic review was performed, following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. A search was conducted in PubMed database with title/abstract keywords "Phosphaturic mesenchymal tumor" and "Osteomalacia". Search results were reviewed looking for intracranial or skull base tumors.ResultsOur systematic review included 29 reported cases of intracranial PMT. In the reviewed cases there was a significative female predominance with 22 cases (75,86%). Osteomalacia was presented in 25 cases (86,20%). Bone fractures were present in 10 cases (34,48%). The most common site of involvement was the anterior cranial fossa in 14 cases (48,27%). Surgery was performed in 27 cases (93,10%) with previous tumor embolization in 4 cases (13,79%). Total recovery of the presenting symptoms in the first year was achieved in 21 cases (72,41%). Recurrence of the disease was described in 6 cases (25%).ConclusionSkull base PMTs with intracranial extension are extremely rare tumors. Most patients are middle-aged adults with a PMT predominantly located in anterior cranial fossa. Surgery is the current treatment of choice with optimal outcome at one year follow-up, although recurrence could be present in almost 25% of the cases.Copyright © 2024 Elsevier Inc. All rights reserved.

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