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Malignant Phosphaturic Mesenchymal Tumor

MedGen UID:
824907
•Concept ID:
C3839061
•
Neoplastic Process
Synonym: Phosphaturic mesenchymal tumor, malignant
SNOMED CT: Phosphaturic mesenchymal tumor, malignant (703650004)

Definition

A phosphaturic mesenchymal tumor characterized by the presence of nuclear atypia, high mitotic activity, increased cellularity, marked pleomorphism, and necrosis. It usually develops in lesions that have recurred locally and metastasizes to other sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Phosphaturic Mesenchymal Tumor

Professional guidelines

PubMed

Diagnosis and management of tumor-induced osteomalacia: a single center experience.
Hacisahinogullari H, Tekin S, Tanrikulu S, Saribeyliler G, Yalin GY, Bilgic B, Isik EG, Salduz A, Tuncer S, Gul N, Uzum AK, Aral F, Tanakol R, Selcukbiricik OS
Endocrine 2023 Nov;82(2):427-434. Epub 2023 Jul 22 doi: 10.1007/s12020-023-03450-3. PMID: 37480497
Clinical Challenges in Diagnosis, Tumor Localization and Treatment of Tumor-Induced Osteomalacia: Outcome of a Retrospective Surveillance.
Hidaka N, Koga M, Kimura S, Hoshino Y, Kato H, Kinoshita Y, Makita N, Nangaku M, Horiguchi K, Furukawa Y, Ohnaka K, Inagaki K, Nakagawa A, Suzuki A, Takeuchi Y, Fukumoto S, Nakatani F, Ito N
J Bone Miner Res 2022 Aug;37(8):1479-1488. Epub 2022 Jul 1 doi: 10.1002/jbmr.4620. PMID: 35690913
Surgical Treatment and Outcomes for Sinonasal and Skull Base Phosphaturic Mesenchymal Tumors.
Tang R, Mao S, Lin H, Ye HB, Li DW, Chen ZN, Su KM, Zhang WT
Otolaryngol Head Neck Surg 2020 May;162(5):674-682. Epub 2020 Mar 3 doi: 10.1177/0194599820904055. PMID: 32122245

Recent clinical studies

Etiology

Clinical Characteristics of Malignant Phosphaturic Mesenchymal Tumor Causing Tumor-Induced Osteomalacia.
Abate V, Vergatti A, De Filippo G, Damiano V, Menale C, D'Elia L, Rendina D
J Clin Endocrinol Metab 2024 Feb 20;109(3):e1006-e1011. doi: 10.1210/clinem/dgad690. PMID: 38006315
Genetic profiling of a chondroblastoma-like osteosarcoma/malignant phosphaturic mesenchymal tumor of bone reveals a homozygous deletion of CDKN2A, intragenic deletion of DMD, and a targetable FN1-FGFR1 gene fusion.
Saba KH, Cornmark L, Rissler M, Fioretos T, Åström K, Haglund F, Rosenberg AE, Brosjö O, Nord KH
Genes Chromosomes Cancer 2019 Oct;58(10):731-736. Epub 2019 May 15 doi: 10.1002/gcc.22764. PMID: 31066955

Diagnosis

Treatment and Diagnose of Spinal Phosphaturic Mesenchymal Tumor: A Case Report and a Systematic Literature Review.
Chen D, Zhang L, Zhang J, Yin M, Gao X, Huang Q, Li L, Yang X
World Neurosurg 2024 Apr;184:65-73. Epub 2024 Jan 12 doi: 10.1016/j.wneu.2024.01.032. PMID: 38218444
Clinical Characteristics of Malignant Phosphaturic Mesenchymal Tumor Causing Tumor-Induced Osteomalacia.
Abate V, Vergatti A, De Filippo G, Damiano V, Menale C, D'Elia L, Rendina D
J Clin Endocrinol Metab 2024 Feb 20;109(3):e1006-e1011. doi: 10.1210/clinem/dgad690. PMID: 38006315
Malignant phosphaturic mesenchymal tumor-ossifying fibroma-like subtype: a case report and literature review.
Qin H, Zeng H, Li H, Yuan S, Yang J
BMC Musculoskelet Disord 2021 Aug 10;22(1):677. doi: 10.1186/s12891-021-04558-1. PMID: 34376178Free PMC Article
Malignant phosphaturic mesenchymal tumor with pulmonary metastasis: A case report.
Qiu S, Cao LL, Qiu Y, Yan P, Li ZX, Du J, Sun LM, Zhang QF
Medicine (Baltimore) 2017 Apr;96(17):e6750. doi: 10.1097/MD.0000000000006750. PMID: 28445300Free PMC Article
Malignant phosphaturic mesenchymal tumor of the larynx.
Sidell D, Lai C, Bhuta S, Barnes L, Chhetri DK
Laryngoscope 2011 Sep;121(9):1860-3. Epub 2011 Jun 30 doi: 10.1002/lary.21916. PMID: 21721013

Prognosis

Genetic profiling of a chondroblastoma-like osteosarcoma/malignant phosphaturic mesenchymal tumor of bone reveals a homozygous deletion of CDKN2A, intragenic deletion of DMD, and a targetable FN1-FGFR1 gene fusion.
Saba KH, Cornmark L, Rissler M, Fioretos T, Åström K, Haglund F, Rosenberg AE, Brosjö O, Nord KH
Genes Chromosomes Cancer 2019 Oct;58(10):731-736. Epub 2019 May 15 doi: 10.1002/gcc.22764. PMID: 31066955
Malignant phosphaturic mesenchymal tumor with pulmonary metastasis: A case report.
Qiu S, Cao LL, Qiu Y, Yan P, Li ZX, Du J, Sun LM, Zhang QF
Medicine (Baltimore) 2017 Apr;96(17):e6750. doi: 10.1097/MD.0000000000006750. PMID: 28445300Free PMC Article
Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia.
Ogose A, Hotta T, Emura I, Hatano H, Inoue Y, Umezu H, Endo N
Skeletal Radiol 2001 Feb;30(2):99-103. doi: 10.1007/s002560000306. PMID: 11310207

Recent systematic reviews

Treatment and Diagnose of Spinal Phosphaturic Mesenchymal Tumor: A Case Report and a Systematic Literature Review.
Chen D, Zhang L, Zhang J, Yin M, Gao X, Huang Q, Li L, Yang X
World Neurosurg 2024 Apr;184:65-73. Epub 2024 Jan 12 doi: 10.1016/j.wneu.2024.01.032. PMID: 38218444
Clinical Characteristics of Malignant Phosphaturic Mesenchymal Tumor Causing Tumor-Induced Osteomalacia.
Abate V, Vergatti A, De Filippo G, Damiano V, Menale C, D'Elia L, Rendina D
J Clin Endocrinol Metab 2024 Feb 20;109(3):e1006-e1011. doi: 10.1210/clinem/dgad690. PMID: 38006315

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