A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base

Objectives: To describe a rare case of infratemporal phosphaturic mesenchymal tumor (PMT), a “nonphosphaturic” variant in the infratemporal skull base, in a 5-year-old boy. Methods: This was a retrospective study of the patient's clinical and biochemical features, computed tomography (CT), maxi...

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Autores principales:	Jin-Feng Liang, Zu-yun Li, Dong-Xiao Nong, An-zhou Tang
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Phosphaturic mesenchymal tumor (PMT) “Nonphosphaturic” variant Fibroblast growth factor 23 (FGF-23) Oncogenic osteomalacia Infratemporal skull base Otorhinolaryngology RF1-547
Acceso en línea:	https://doaj.org/article/c85f9c74963948f482557806ba4d870c
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spelling	oai:doaj.org-article:c85f9c74963948f482557806ba4d870c2021-11-12T04:44:20ZA pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base2468-548810.1016/j.xocr.2021.100377https://doaj.org/article/c85f9c74963948f482557806ba4d870c2021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2468548821001181https://doaj.org/toc/2468-5488Objectives: To describe a rare case of infratemporal phosphaturic mesenchymal tumor (PMT), a “nonphosphaturic” variant in the infratemporal skull base, in a 5-year-old boy. Methods: This was a retrospective study of the patient's clinical and biochemical features, computed tomography (CT), maxillofacial magnetic resonance imaging (MRI), histopathology as well as surgical treatment and follow-up. Results: The boy suffered from a right preauricular mass and ipsilateral hearing loss. Temporal bone CT and maxillofacial MRI demonstrated a circumscribed infratemporal skull base lesion. Histomorphologically, the lesion demonstrated typical microscopic features of PMTs and was positive for vimentin, smooth muscle actin, and FGF-23. However, there was no clinical or biochemical evidence of tumor-induced osteomalacia (TIO), and ultimately a diagnosis of a “nonphosphaturic” variant of PMTs was made. No postoperative recurrence was observed during follow-up. Conclusions: PMTs are rare and have a low incidence among children. They are histologically distinctive tumors associated with phosphaturia and TIO. So-called “nonphosphaturic” variants, occurring without known phosphaturia, are even rarer. Pathologists and clinicians need to be aware of the presence of a PMT with an absence of osteomalacia symptoms. Wide surgical resection and long-term follow-up are necessary. Intraoperative functional and aesthetic concerns should be emphasized as well, particularly in pediatric patients.Jin-Feng LiangZu-yun LiDong-Xiao NongAn-zhou TangElsevierarticlePhosphaturic mesenchymal tumor (PMT)“Nonphosphaturic” variantFibroblast growth factor 23 (FGF-23)Oncogenic osteomalaciaInfratemporal skull baseOtorhinolaryngologyRF1-547ENOtolaryngology Case Reports, Vol 21, Iss , Pp 100377- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Phosphaturic mesenchymal tumor (PMT) “Nonphosphaturic” variant Fibroblast growth factor 23 (FGF-23) Oncogenic osteomalacia Infratemporal skull base Otorhinolaryngology RF1-547
spellingShingle	Phosphaturic mesenchymal tumor (PMT) “Nonphosphaturic” variant Fibroblast growth factor 23 (FGF-23) Oncogenic osteomalacia Infratemporal skull base Otorhinolaryngology RF1-547 Jin-Feng Liang Zu-yun Li Dong-Xiao Nong An-zhou Tang A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base
description	Objectives: To describe a rare case of infratemporal phosphaturic mesenchymal tumor (PMT), a “nonphosphaturic” variant in the infratemporal skull base, in a 5-year-old boy. Methods: This was a retrospective study of the patient's clinical and biochemical features, computed tomography (CT), maxillofacial magnetic resonance imaging (MRI), histopathology as well as surgical treatment and follow-up. Results: The boy suffered from a right preauricular mass and ipsilateral hearing loss. Temporal bone CT and maxillofacial MRI demonstrated a circumscribed infratemporal skull base lesion. Histomorphologically, the lesion demonstrated typical microscopic features of PMTs and was positive for vimentin, smooth muscle actin, and FGF-23. However, there was no clinical or biochemical evidence of tumor-induced osteomalacia (TIO), and ultimately a diagnosis of a “nonphosphaturic” variant of PMTs was made. No postoperative recurrence was observed during follow-up. Conclusions: PMTs are rare and have a low incidence among children. They are histologically distinctive tumors associated with phosphaturia and TIO. So-called “nonphosphaturic” variants, occurring without known phosphaturia, are even rarer. Pathologists and clinicians need to be aware of the presence of a PMT with an absence of osteomalacia symptoms. Wide surgical resection and long-term follow-up are necessary. Intraoperative functional and aesthetic concerns should be emphasized as well, particularly in pediatric patients.
format	article
author	Jin-Feng Liang Zu-yun Li Dong-Xiao Nong An-zhou Tang
author_facet	Jin-Feng Liang Zu-yun Li Dong-Xiao Nong An-zhou Tang
author_sort	Jin-Feng Liang
title	A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base
title_short	A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base
title_full	A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base
title_fullStr	A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base
title_full_unstemmed	A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base
title_sort	pediatric case of a phosphaturic mesenchymal tumor: a “nonphosphaturic” variant in the infratemporal skull base
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/c85f9c74963948f482557806ba4d870c
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A pediatric case of a phosphaturic mesenchymal tumor: A “nonphosphaturic” variant in the infratemporal skull base

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