Clinical, histopathologic, subtype, and immunohistochemical analysis of jaw phosphaturic mesenchymal tumors

Jaw phosphaturic mesenchymal tumors (PMTs) are a rare neoplasm with uncertain histogenesis. This study aimed to clarify the clinical and pathological features of jaw PMTs.We reviewed the clinical records of 39 patients diagnosed with PMTs in the jaws, and investigated clinical and morphologic charac...

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Veröffentlicht in:Medicine (Baltimore) 2020-02, Vol.99 (7), p.e19090-e19090
Hauptverfasser: Li, Dongmei, Zhu, Ran, Zhou, Lian, Zhong, Dingrong
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Zhu, Ran
Zhou, Lian
Zhong, Dingrong
description Jaw phosphaturic mesenchymal tumors (PMTs) are a rare neoplasm with uncertain histogenesis. This study aimed to clarify the clinical and pathological features of jaw PMTs.We reviewed the clinical records of 39 patients diagnosed with PMTs in the jaws, and investigated clinical and morphologic characteristics, histologic subtypes, and immunophenotypes of all cases.Microscopic analyses revealed 2 major histologic tumor subtypes: "phosphaturic mesenchymal tumors of mixed epithelial and connective tissue" (PMTMECT), and "phosphaturic mesenchymal tumors of mixed connective tissue" (PMTMCT). PMTMECTs and PMTMCTs accounted for 29 and 10 cases of PMTs, respectively. Most PMTMECT diagnoses were made predominantly in males aged
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This study aimed to clarify the clinical and pathological features of jaw PMTs.We reviewed the clinical records of 39 patients diagnosed with PMTs in the jaws, and investigated clinical and morphologic characteristics, histologic subtypes, and immunophenotypes of all cases.Microscopic analyses revealed 2 major histologic tumor subtypes: "phosphaturic mesenchymal tumors of mixed epithelial and connective tissue" (PMTMECT), and "phosphaturic mesenchymal tumors of mixed connective tissue" (PMTMCT). PMTMECTs and PMTMCTs accounted for 29 and 10 cases of PMTs, respectively. Most PMTMECT diagnoses were made predominantly in males aged &lt;45 years, and the incidence was similar in both the mandible and maxilla. In contrast, patients with PMTMCTs are predominantly females aged ≥45 years, and all tumors were in the mandible. Histologically, PMTMECT had lower cellularity and a more elongated and spindled mesenchymal component with less elaborate intrinsic microvasculature than PMTMCT. Immunohistochemically, the epithelia of all PMTMECTs was immunoreactive for AE1/AE3. Other immunohistochemical staining of PMTMECTs revealed positive expression of vimentin, SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34 in either one or both components. Immunohistochemical staining of PMTMCTs was diffusely positive for vimentin and a varied ratio of positivity for SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34, but negative for AE1/AE3. Most patients were cured by complete resection, except 2 patients who had repeated recurrences, one of which also had multiple metastasis.Jaw PMT can be divided into 2 major histological subtypes. PMTMECTs are more common than are PMTMCTs, and can transform into malignant PMTMCTs during the progression. PMTMECTs were more commonly observed in males and the incidence was similar in both the maxilla and mandible. PMTMCTs were almost always observed in the mandible of females. Compared with PMTMCTs, PMTMECTs have an admixture of epithelial components with less prominent vasculature and lower cellularity. There were no statistically significant differences in the expression of immunohistochemical markers except AE1/AE3 between PMTMECTs and PMTMCTs. However, immunohistochemical markers have great significance for differentiating other mesenchymal tumors.</description><identifier>ISSN: 0025-7974</identifier><identifier>EISSN: 1536-5964</identifier><identifier>DOI: 10.1097/MD.0000000000019090</identifier><identifier>PMID: 32049812</identifier><language>eng</language><publisher>United States: the Author(s). Published by Wolters Kluwer Health, Inc</publisher><subject>Biomarkers, Tumor - metabolism ; Female ; Humans ; Jaw Neoplasms - metabolism ; Jaw Neoplasms - pathology ; Jaw Neoplasms - surgery ; Male ; Observational Study ; Sex Factors ; Survival Analysis ; Treatment Outcome</subject><ispartof>Medicine (Baltimore), 2020-02, Vol.99 (7), p.e19090-e19090</ispartof><rights>the Author(s). Published by Wolters Kluwer Health, Inc.</rights><rights>Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc. 2020</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4508-cb69c62cd7a7ae16ae0c596bef25182f5207373a5b12f282d9b5bdc10d85d4813</citedby><cites>FETCH-LOGICAL-c4508-cb69c62cd7a7ae16ae0c596bef25182f5207373a5b12f282d9b5bdc10d85d4813</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035060/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035060/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,27901,27902,53766,53768</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/32049812$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Li, Dongmei</creatorcontrib><creatorcontrib>Zhu, Ran</creatorcontrib><creatorcontrib>Zhou, Lian</creatorcontrib><creatorcontrib>Zhong, Dingrong</creatorcontrib><title>Clinical, histopathologic, subtype, and immunohistochemical analysis of jaw phosphaturic mesenchymal tumors</title><title>Medicine (Baltimore)</title><addtitle>Medicine (Baltimore)</addtitle><description>Jaw phosphaturic mesenchymal tumors (PMTs) are a rare neoplasm with uncertain histogenesis. This study aimed to clarify the clinical and pathological features of jaw PMTs.We reviewed the clinical records of 39 patients diagnosed with PMTs in the jaws, and investigated clinical and morphologic characteristics, histologic subtypes, and immunophenotypes of all cases.Microscopic analyses revealed 2 major histologic tumor subtypes: "phosphaturic mesenchymal tumors of mixed epithelial and connective tissue" (PMTMECT), and "phosphaturic mesenchymal tumors of mixed connective tissue" (PMTMCT). PMTMECTs and PMTMCTs accounted for 29 and 10 cases of PMTs, respectively. Most PMTMECT diagnoses were made predominantly in males aged &lt;45 years, and the incidence was similar in both the mandible and maxilla. In contrast, patients with PMTMCTs are predominantly females aged ≥45 years, and all tumors were in the mandible. Histologically, PMTMECT had lower cellularity and a more elongated and spindled mesenchymal component with less elaborate intrinsic microvasculature than PMTMCT. Immunohistochemically, the epithelia of all PMTMECTs was immunoreactive for AE1/AE3. Other immunohistochemical staining of PMTMECTs revealed positive expression of vimentin, SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34 in either one or both components. Immunohistochemical staining of PMTMCTs was diffusely positive for vimentin and a varied ratio of positivity for SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34, but negative for AE1/AE3. Most patients were cured by complete resection, except 2 patients who had repeated recurrences, one of which also had multiple metastasis.Jaw PMT can be divided into 2 major histological subtypes. PMTMECTs are more common than are PMTMCTs, and can transform into malignant PMTMCTs during the progression. PMTMECTs were more commonly observed in males and the incidence was similar in both the maxilla and mandible. PMTMCTs were almost always observed in the mandible of females. Compared with PMTMCTs, PMTMECTs have an admixture of epithelial components with less prominent vasculature and lower cellularity. There were no statistically significant differences in the expression of immunohistochemical markers except AE1/AE3 between PMTMECTs and PMTMCTs. However, immunohistochemical markers have great significance for differentiating other mesenchymal tumors.</description><subject>Biomarkers, Tumor - metabolism</subject><subject>Female</subject><subject>Humans</subject><subject>Jaw Neoplasms - metabolism</subject><subject>Jaw Neoplasms - pathology</subject><subject>Jaw Neoplasms - surgery</subject><subject>Male</subject><subject>Observational Study</subject><subject>Sex Factors</subject><subject>Survival Analysis</subject><subject>Treatment Outcome</subject><issn>0025-7974</issn><issn>1536-5964</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpdkUtv3CAUhVHVqpmm_QWVKi-7GKc8jdlUqiZ9SYm6adcIYxxIwLiAM5p_X5JJ0wcbpMN3zr3oAPAawTMEBX93eX4G_xwkoIBPwAYx0rVMdPQp2ECIWcsFpyfgRc7XFSIc0-fghGBIRY_wBtzsvJudVn7bWJdLXFSx0ccrp7dNXodyWMy2UfPYuBDWOd4z2ppwZ6m68ofschOn5lrtm8XGvFhV1uR0E0w2s7aHUMGyhpjyS_BsUj6bVw_3Kfjx6eP33Zf24tvnr7sPF62mDPatHjqhO6xHrrgyqFMG6vqhwUyYoR5PDENOOFFsQHjCPR7FwIZRIzj2bKQ9Iqfg_TF3WYdgRm3mkpSXS3JBpYOMysl_X2Zn5VW8lRwSBjtYA94-BKT4czW5yOCyNt6r2cQ1S0wY5YTxHleUHFGdYs7JTI9jEJR3NcnLc_l_TdX15u8NHz2_e6kAPQL76ItJ-cave5OkNcoXe5_HuMAthrh2jChsq0J68gsAp6B0</recordid><startdate>20200201</startdate><enddate>20200201</enddate><creator>Li, Dongmei</creator><creator>Zhu, Ran</creator><creator>Zhou, Lian</creator><creator>Zhong, Dingrong</creator><general>the Author(s). Published by Wolters Kluwer Health, Inc</general><general>Wolters Kluwer Health</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>20200201</creationdate><title>Clinical, histopathologic, subtype, and immunohistochemical analysis of jaw phosphaturic mesenchymal tumors</title><author>Li, Dongmei ; Zhu, Ran ; Zhou, Lian ; Zhong, Dingrong</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4508-cb69c62cd7a7ae16ae0c596bef25182f5207373a5b12f282d9b5bdc10d85d4813</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Biomarkers, Tumor - metabolism</topic><topic>Female</topic><topic>Humans</topic><topic>Jaw Neoplasms - metabolism</topic><topic>Jaw Neoplasms - pathology</topic><topic>Jaw Neoplasms - surgery</topic><topic>Male</topic><topic>Observational Study</topic><topic>Sex Factors</topic><topic>Survival Analysis</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Li, Dongmei</creatorcontrib><creatorcontrib>Zhu, Ran</creatorcontrib><creatorcontrib>Zhou, Lian</creatorcontrib><creatorcontrib>Zhong, Dingrong</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Medicine (Baltimore)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Li, Dongmei</au><au>Zhu, Ran</au><au>Zhou, Lian</au><au>Zhong, Dingrong</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Clinical, histopathologic, subtype, and immunohistochemical analysis of jaw phosphaturic mesenchymal tumors</atitle><jtitle>Medicine (Baltimore)</jtitle><addtitle>Medicine (Baltimore)</addtitle><date>2020-02-01</date><risdate>2020</risdate><volume>99</volume><issue>7</issue><spage>e19090</spage><epage>e19090</epage><pages>e19090-e19090</pages><issn>0025-7974</issn><eissn>1536-5964</eissn><abstract>Jaw phosphaturic mesenchymal tumors (PMTs) are a rare neoplasm with uncertain histogenesis. This study aimed to clarify the clinical and pathological features of jaw PMTs.We reviewed the clinical records of 39 patients diagnosed with PMTs in the jaws, and investigated clinical and morphologic characteristics, histologic subtypes, and immunophenotypes of all cases.Microscopic analyses revealed 2 major histologic tumor subtypes: "phosphaturic mesenchymal tumors of mixed epithelial and connective tissue" (PMTMECT), and "phosphaturic mesenchymal tumors of mixed connective tissue" (PMTMCT). PMTMECTs and PMTMCTs accounted for 29 and 10 cases of PMTs, respectively. Most PMTMECT diagnoses were made predominantly in males aged &lt;45 years, and the incidence was similar in both the mandible and maxilla. In contrast, patients with PMTMCTs are predominantly females aged ≥45 years, and all tumors were in the mandible. Histologically, PMTMECT had lower cellularity and a more elongated and spindled mesenchymal component with less elaborate intrinsic microvasculature than PMTMCT. Immunohistochemically, the epithelia of all PMTMECTs was immunoreactive for AE1/AE3. Other immunohistochemical staining of PMTMECTs revealed positive expression of vimentin, SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34 in either one or both components. Immunohistochemical staining of PMTMCTs was diffusely positive for vimentin and a varied ratio of positivity for SATB2, ERG, CD99, Bcl-2, CD56, S-100, D2-40, CD68, SMA, and CD34, but negative for AE1/AE3. Most patients were cured by complete resection, except 2 patients who had repeated recurrences, one of which also had multiple metastasis.Jaw PMT can be divided into 2 major histological subtypes. PMTMECTs are more common than are PMTMCTs, and can transform into malignant PMTMCTs during the progression. PMTMECTs were more commonly observed in males and the incidence was similar in both the maxilla and mandible. PMTMCTs were almost always observed in the mandible of females. Compared with PMTMCTs, PMTMECTs have an admixture of epithelial components with less prominent vasculature and lower cellularity. There were no statistically significant differences in the expression of immunohistochemical markers except AE1/AE3 between PMTMECTs and PMTMCTs. However, immunohistochemical markers have great significance for differentiating other mesenchymal tumors.</abstract><cop>United States</cop><pub>the Author(s). Published by Wolters Kluwer Health, Inc</pub><pmid>32049812</pmid><doi>10.1097/MD.0000000000019090</doi><oa>free_for_read</oa></addata></record>
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subjects Biomarkers, Tumor - metabolism
Female
Humans
Jaw Neoplasms - metabolism
Jaw Neoplasms - pathology
Jaw Neoplasms - surgery
Male
Observational Study
Sex Factors
Survival Analysis
Treatment Outcome
title Clinical, histopathologic, subtype, and immunohistochemical analysis of jaw phosphaturic mesenchymal tumors
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