Tuberous sclerosis-associated renal cell carcinoma: a clinicopathologic study of 57 separate carcinomas in 18 patients.
Identifieur interne : 003245 ( PubMed/Curation ); précédent : 003244; suivant : 003246Tuberous sclerosis-associated renal cell carcinoma: a clinicopathologic study of 57 separate carcinomas in 18 patients.
Auteurs : Juan Guo [Canada] ; Maria S. Tretiakova ; Megan L. Troxell ; Adeboye O. Osunkoya ; Oluwole Fadare ; Ankur R. Sangoi ; Steven S. Shen ; Antonio Lopez-Beltran ; Rohit Mehra ; Amer Heider ; John P. Higgins ; Lara R. Harik ; Xavier Leroy ; Anthony J. Gill ; Kiril Trpkov ; Steven C. Campbell ; Christopher Przybycin ; Cristina Magi-Galluzzi ; Jesse K. MckenneySource :
- The American journal of surgical pathology [ 1532-0979 ] ; 2014.
Descripteurs français
- KwdFr :
- Adolescent, Adulte, Adulte d'âge moyen, Biopsie, Enfant, Facteurs temps, Femelle, Humains, Immunohistochimie, Immunophénotypage, Jeune adulte, Marqueurs biologiques tumoraux (analyse), Mâle, Néphrectomie, Néphrocarcinome (), Néphrocarcinome (anatomopathologie), Récidive tumorale locale, Résultat thérapeutique, Sclérose tubéreuse du cerveau (), Sclérose tubéreuse du cerveau (anatomopathologie), Sclérose tubéreuse du cerveau (métabolisme), Seconde tumeur primitive, Sujet âgé, Tumeurs du rein (), Tumeurs du rein (anatomopathologie), Tumeurs primitives multiples.
- MESH :
- analyse : Marqueurs biologiques tumoraux.
- anatomopathologie : Néphrocarcinome, Sclérose tubéreuse du cerveau, Tumeurs du rein.
- métabolisme : Sclérose tubéreuse du cerveau.
- Adolescent, Adulte, Adulte d'âge moyen, Biopsie, Enfant, Facteurs temps, Femelle, Humains, Immunohistochimie, Immunophénotypage, Jeune adulte, Mâle, Néphrectomie, Néphrocarcinome, Récidive tumorale locale, Résultat thérapeutique, Sclérose tubéreuse du cerveau, Seconde tumeur primitive, Sujet âgé, Tumeurs du rein, Tumeurs primitives multiples.
English descriptors
- KwdEn :
- Adolescent, Adult, Aged, Biomarkers, Tumor (analysis), Biopsy, Carcinoma, Renal Cell (chemistry), Carcinoma, Renal Cell (pathology), Carcinoma, Renal Cell (surgery), Child, Female, Humans, Immunohistochemistry, Immunophenotyping, Kidney Neoplasms (chemistry), Kidney Neoplasms (pathology), Kidney Neoplasms (surgery), Male, Middle Aged, Neoplasm Recurrence, Local, Neoplasms, Multiple Primary, Neoplasms, Second Primary, Nephrectomy, Time Factors, Treatment Outcome, Tuberous Sclerosis (metabolism), Tuberous Sclerosis (pathology), Tuberous Sclerosis (surgery), Young Adult.
- MESH :
- chemical , analysis : Biomarkers, Tumor.
- chemistry : Carcinoma, Renal Cell, Kidney Neoplasms.
- metabolism : Tuberous Sclerosis.
- pathology : Carcinoma, Renal Cell, Kidney Neoplasms, Tuberous Sclerosis.
- surgery : Carcinoma, Renal Cell, Kidney Neoplasms, Tuberous Sclerosis.
- Adolescent, Adult, Aged, Biopsy, Child, Female, Humans, Immunohistochemistry, Immunophenotyping, Male, Middle Aged, Neoplasm Recurrence, Local, Neoplasms, Multiple Primary, Neoplasms, Second Primary, Nephrectomy, Time Factors, Treatment Outcome, Young Adult.
Abstract
Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with characteristic tumors involving multiple organ systems. Whereas renal angiomyolipoma (AML) is common in TSC, renal cell carcinoma (RCC) is rarely reported. Fifty-seven RCCs from 13 female and 5 male TSC patients were reviewed. Age at surgery ranged from 7 to 65 years (mean: 42 y). Nine patients (50%) had multiple synchronous and/or metachronous RCCs (range of 2 to 20 RCCs) and 5 had bilateral RCCs (28%). Seventeen patients (94%) had histologically confirmed concurrent renal AMLs, including 15 with multiple AMLs (88%) and 9 (50%) with AMLs with epithelial cysts. None of the 15 patients with available clinical follow-up information had evidence of distant metastatic disease from 6 to 198 months after their initial surgery (mean: 52 mo). The 57 RCCs exhibited 3 major distinct morphologies: (1) 17 RCCs (30%) had features similar to tumors previously described as "renal angiomyoadenomatous tumor" or "RCC with smooth muscle stroma"; (2) 34 RCCs (59%) showed features similar to chromophobe RCC; and (3) 6 RCCs (11%) showed a granular eosinophilic-macrocystic morphology. Distinct histologic changes were also commonly present in the background kidney parenchyma and included cysts or renal tubules lined by epithelial cells with prominent eosinophilic cytoplasm, nucleomegaly, and nucleoli. Immunohistochemically, all RCCs tested showed strong nuclear reactivity for PAX8 and HMB45 negativity. Compared with sporadic RCCs, TSC-associated RCCs have unique clinicopathologic features including female predominance, younger age at diagnosis, multiplicity, association with AMLs, 3 recurring histologic patterns, and an indolent clinical course. Awareness of the morphologic and clinicopathologic spectrum of RCC in this setting will allow surgical pathologists to better recognize clinically unsuspected TSC patients.
DOI: 10.1097/PAS.0000000000000248
PubMed: 25093518
Links toward previous steps (curation, corpus...)
- to stream PubMed, to step Corpus: Pour aller vers cette notice dans l'étape Curation :003356
Links to Exploration step
pubmed:25093518Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Tuberous sclerosis-associated renal cell carcinoma: a clinicopathologic study of 57 separate carcinomas in 18 patients.</title>
<author><name sortKey="Guo, Juan" sort="Guo, Juan" uniqKey="Guo J" first="Juan" last="Guo">Juan Guo</name>
<affiliation wicri:level="1"><nlm:affiliation>*Cleveland Clinic, Robert J. Tomsich Pathology & Laboratory Medicine Institute ***Glickman Urological and Kidney Institute, Cleveland, OH †Department of Pathology, University of Washington, Seattle, WA ‡Department of Pathology, Oregon Health & Science University, Portland, OR §Department of Pathology, Emory University, Atlanta, GA ∥Department of Pathology, Vanderbilt University, Nashville, TN ¶Department of Pathology, El Camino Hospital, Mountain View ‡‡Department of Pathology, Stanford University, Stanford, CA #Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX ††Department of Pathology, University of Michigan, Ann Arbor, MI §§Department of Pathology, Columbia University, New York, NY **Department of Anatomic Pathology, University of Cordoba, Cordoba, Spain ∥∥Department of Pathology, Lille University Hospitals, Lille, France ¶¶Department of Anatomic Pathology, Royal North Shore Hospital, University of Sydney, St Leonards, NSW, Australia ##Department of Pathology and Laboratory Medicine, University of Calgary and Calgary Laboratory Services, Calgary, AB, Canada.</nlm:affiliation>
<country xml:lang="fr">Canada</country>
<wicri:regionArea>*Cleveland Clinic, Robert J. Tomsich Pathology & Laboratory Medicine Institute ***Glickman Urological and Kidney Institute, Cleveland, OH †Department of Pathology, University of Washington, Seattle, WA ‡Department of Pathology, Oregon Health & Science University, Portland, OR §Department of Pathology, Emory University, Atlanta, GA ∥Department of Pathology, Vanderbilt University, Nashville, TN ¶Department of Pathology, El Camino Hospital, Mountain View ‡‡Department of Pathology, Stanford University, Stanford, CA #Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX ††Department of Pathology, University of Michigan, Ann Arbor, MI §§Department of Pathology, Columbia University, New York, NY **Department of Anatomic Pathology, University of Cordoba, Cordoba, Spain ∥∥Department of Pathology, Lille University Hospitals, Lille, France ¶¶Department of Anatomic Pathology, Royal North Shore Hospital, University of Sydney, St Leonards, NSW, Australia ##Department of Pathology and Laboratory Medicine, University of Calgary and Calgary Laboratory Services, Calgary, AB</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Tretiakova, Maria S" sort="Tretiakova, Maria S" uniqKey="Tretiakova M" first="Maria S" last="Tretiakova">Maria S. Tretiakova</name>
</author>
<author><name sortKey="Troxell, Megan L" sort="Troxell, Megan L" uniqKey="Troxell M" first="Megan L" last="Troxell">Megan L. Troxell</name>
</author>
<author><name sortKey="Osunkoya, Adeboye O" sort="Osunkoya, Adeboye O" uniqKey="Osunkoya A" first="Adeboye O" last="Osunkoya">Adeboye O. Osunkoya</name>
</author>
<author><name sortKey="Fadare, Oluwole" sort="Fadare, Oluwole" uniqKey="Fadare O" first="Oluwole" last="Fadare">Oluwole Fadare</name>
</author>
<author><name sortKey="Sangoi, Ankur R" sort="Sangoi, Ankur R" uniqKey="Sangoi A" first="Ankur R" last="Sangoi">Ankur R. Sangoi</name>
</author>
<author><name sortKey="Shen, Steven S" sort="Shen, Steven S" uniqKey="Shen S" first="Steven S" last="Shen">Steven S. Shen</name>
</author>
<author><name sortKey="Lopez Beltran, Antonio" sort="Lopez Beltran, Antonio" uniqKey="Lopez Beltran A" first="Antonio" last="Lopez-Beltran">Antonio Lopez-Beltran</name>
</author>
<author><name sortKey="Mehra, Rohit" sort="Mehra, Rohit" uniqKey="Mehra R" first="Rohit" last="Mehra">Rohit Mehra</name>
</author>
<author><name sortKey="Heider, Amer" sort="Heider, Amer" uniqKey="Heider A" first="Amer" last="Heider">Amer Heider</name>
</author>
<author><name sortKey="Higgins, John P" sort="Higgins, John P" uniqKey="Higgins J" first="John P" last="Higgins">John P. Higgins</name>
</author>
<author><name sortKey="Harik, Lara R" sort="Harik, Lara R" uniqKey="Harik L" first="Lara R" last="Harik">Lara R. Harik</name>
</author>
<author><name sortKey="Leroy, Xavier" sort="Leroy, Xavier" uniqKey="Leroy X" first="Xavier" last="Leroy">Xavier Leroy</name>
</author>
<author><name sortKey="Gill, Anthony J" sort="Gill, Anthony J" uniqKey="Gill A" first="Anthony J" last="Gill">Anthony J. Gill</name>
</author>
<author><name sortKey="Trpkov, Kiril" sort="Trpkov, Kiril" uniqKey="Trpkov K" first="Kiril" last="Trpkov">Kiril Trpkov</name>
</author>
<author><name sortKey="Campbell, Steven C" sort="Campbell, Steven C" uniqKey="Campbell S" first="Steven C" last="Campbell">Steven C. Campbell</name>
</author>
<author><name sortKey="Przybycin, Christopher" sort="Przybycin, Christopher" uniqKey="Przybycin C" first="Christopher" last="Przybycin">Christopher Przybycin</name>
</author>
<author><name sortKey="Magi Galluzzi, Cristina" sort="Magi Galluzzi, Cristina" uniqKey="Magi Galluzzi C" first="Cristina" last="Magi-Galluzzi">Cristina Magi-Galluzzi</name>
</author>
<author><name sortKey="Mckenney, Jesse K" sort="Mckenney, Jesse K" uniqKey="Mckenney J" first="Jesse K" last="Mckenney">Jesse K. Mckenney</name>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">PubMed</idno>
<date when="2014">2014</date>
<idno type="RBID">pubmed:25093518</idno>
<idno type="pmid">25093518</idno>
<idno type="doi">10.1097/PAS.0000000000000248</idno>
<idno type="wicri:Area/PubMed/Corpus">003356</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">003356</idno>
<idno type="wicri:Area/PubMed/Curation">003245</idno>
<idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">003245</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en">Tuberous sclerosis-associated renal cell carcinoma: a clinicopathologic study of 57 separate carcinomas in 18 patients.</title>
<author><name sortKey="Guo, Juan" sort="Guo, Juan" uniqKey="Guo J" first="Juan" last="Guo">Juan Guo</name>
<affiliation wicri:level="1"><nlm:affiliation>*Cleveland Clinic, Robert J. Tomsich Pathology & Laboratory Medicine Institute ***Glickman Urological and Kidney Institute, Cleveland, OH †Department of Pathology, University of Washington, Seattle, WA ‡Department of Pathology, Oregon Health & Science University, Portland, OR §Department of Pathology, Emory University, Atlanta, GA ∥Department of Pathology, Vanderbilt University, Nashville, TN ¶Department of Pathology, El Camino Hospital, Mountain View ‡‡Department of Pathology, Stanford University, Stanford, CA #Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX ††Department of Pathology, University of Michigan, Ann Arbor, MI §§Department of Pathology, Columbia University, New York, NY **Department of Anatomic Pathology, University of Cordoba, Cordoba, Spain ∥∥Department of Pathology, Lille University Hospitals, Lille, France ¶¶Department of Anatomic Pathology, Royal North Shore Hospital, University of Sydney, St Leonards, NSW, Australia ##Department of Pathology and Laboratory Medicine, University of Calgary and Calgary Laboratory Services, Calgary, AB, Canada.</nlm:affiliation>
<country xml:lang="fr">Canada</country>
<wicri:regionArea>*Cleveland Clinic, Robert J. Tomsich Pathology & Laboratory Medicine Institute ***Glickman Urological and Kidney Institute, Cleveland, OH †Department of Pathology, University of Washington, Seattle, WA ‡Department of Pathology, Oregon Health & Science University, Portland, OR §Department of Pathology, Emory University, Atlanta, GA ∥Department of Pathology, Vanderbilt University, Nashville, TN ¶Department of Pathology, El Camino Hospital, Mountain View ‡‡Department of Pathology, Stanford University, Stanford, CA #Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX ††Department of Pathology, University of Michigan, Ann Arbor, MI §§Department of Pathology, Columbia University, New York, NY **Department of Anatomic Pathology, University of Cordoba, Cordoba, Spain ∥∥Department of Pathology, Lille University Hospitals, Lille, France ¶¶Department of Anatomic Pathology, Royal North Shore Hospital, University of Sydney, St Leonards, NSW, Australia ##Department of Pathology and Laboratory Medicine, University of Calgary and Calgary Laboratory Services, Calgary, AB</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Tretiakova, Maria S" sort="Tretiakova, Maria S" uniqKey="Tretiakova M" first="Maria S" last="Tretiakova">Maria S. Tretiakova</name>
</author>
<author><name sortKey="Troxell, Megan L" sort="Troxell, Megan L" uniqKey="Troxell M" first="Megan L" last="Troxell">Megan L. Troxell</name>
</author>
<author><name sortKey="Osunkoya, Adeboye O" sort="Osunkoya, Adeboye O" uniqKey="Osunkoya A" first="Adeboye O" last="Osunkoya">Adeboye O. Osunkoya</name>
</author>
<author><name sortKey="Fadare, Oluwole" sort="Fadare, Oluwole" uniqKey="Fadare O" first="Oluwole" last="Fadare">Oluwole Fadare</name>
</author>
<author><name sortKey="Sangoi, Ankur R" sort="Sangoi, Ankur R" uniqKey="Sangoi A" first="Ankur R" last="Sangoi">Ankur R. Sangoi</name>
</author>
<author><name sortKey="Shen, Steven S" sort="Shen, Steven S" uniqKey="Shen S" first="Steven S" last="Shen">Steven S. Shen</name>
</author>
<author><name sortKey="Lopez Beltran, Antonio" sort="Lopez Beltran, Antonio" uniqKey="Lopez Beltran A" first="Antonio" last="Lopez-Beltran">Antonio Lopez-Beltran</name>
</author>
<author><name sortKey="Mehra, Rohit" sort="Mehra, Rohit" uniqKey="Mehra R" first="Rohit" last="Mehra">Rohit Mehra</name>
</author>
<author><name sortKey="Heider, Amer" sort="Heider, Amer" uniqKey="Heider A" first="Amer" last="Heider">Amer Heider</name>
</author>
<author><name sortKey="Higgins, John P" sort="Higgins, John P" uniqKey="Higgins J" first="John P" last="Higgins">John P. Higgins</name>
</author>
<author><name sortKey="Harik, Lara R" sort="Harik, Lara R" uniqKey="Harik L" first="Lara R" last="Harik">Lara R. Harik</name>
</author>
<author><name sortKey="Leroy, Xavier" sort="Leroy, Xavier" uniqKey="Leroy X" first="Xavier" last="Leroy">Xavier Leroy</name>
</author>
<author><name sortKey="Gill, Anthony J" sort="Gill, Anthony J" uniqKey="Gill A" first="Anthony J" last="Gill">Anthony J. Gill</name>
</author>
<author><name sortKey="Trpkov, Kiril" sort="Trpkov, Kiril" uniqKey="Trpkov K" first="Kiril" last="Trpkov">Kiril Trpkov</name>
</author>
<author><name sortKey="Campbell, Steven C" sort="Campbell, Steven C" uniqKey="Campbell S" first="Steven C" last="Campbell">Steven C. Campbell</name>
</author>
<author><name sortKey="Przybycin, Christopher" sort="Przybycin, Christopher" uniqKey="Przybycin C" first="Christopher" last="Przybycin">Christopher Przybycin</name>
</author>
<author><name sortKey="Magi Galluzzi, Cristina" sort="Magi Galluzzi, Cristina" uniqKey="Magi Galluzzi C" first="Cristina" last="Magi-Galluzzi">Cristina Magi-Galluzzi</name>
</author>
<author><name sortKey="Mckenney, Jesse K" sort="Mckenney, Jesse K" uniqKey="Mckenney J" first="Jesse K" last="Mckenney">Jesse K. Mckenney</name>
</author>
</analytic>
<series><title level="j">The American journal of surgical pathology</title>
<idno type="eISSN">1532-0979</idno>
<imprint><date when="2014" type="published">2014</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Aged</term>
<term>Biomarkers, Tumor (analysis)</term>
<term>Biopsy</term>
<term>Carcinoma, Renal Cell (chemistry)</term>
<term>Carcinoma, Renal Cell (pathology)</term>
<term>Carcinoma, Renal Cell (surgery)</term>
<term>Child</term>
<term>Female</term>
<term>Humans</term>
<term>Immunohistochemistry</term>
<term>Immunophenotyping</term>
<term>Kidney Neoplasms (chemistry)</term>
<term>Kidney Neoplasms (pathology)</term>
<term>Kidney Neoplasms (surgery)</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Neoplasm Recurrence, Local</term>
<term>Neoplasms, Multiple Primary</term>
<term>Neoplasms, Second Primary</term>
<term>Nephrectomy</term>
<term>Time Factors</term>
<term>Treatment Outcome</term>
<term>Tuberous Sclerosis (metabolism)</term>
<term>Tuberous Sclerosis (pathology)</term>
<term>Tuberous Sclerosis (surgery)</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adolescent</term>
<term>Adulte</term>
<term>Adulte d'âge moyen</term>
<term>Biopsie</term>
<term>Enfant</term>
<term>Facteurs temps</term>
<term>Femelle</term>
<term>Humains</term>
<term>Immunohistochimie</term>
<term>Immunophénotypage</term>
<term>Jeune adulte</term>
<term>Marqueurs biologiques tumoraux (analyse)</term>
<term>Mâle</term>
<term>Néphrectomie</term>
<term>Néphrocarcinome ()</term>
<term>Néphrocarcinome (anatomopathologie)</term>
<term>Récidive tumorale locale</term>
<term>Résultat thérapeutique</term>
<term>Sclérose tubéreuse du cerveau ()</term>
<term>Sclérose tubéreuse du cerveau (anatomopathologie)</term>
<term>Sclérose tubéreuse du cerveau (métabolisme)</term>
<term>Seconde tumeur primitive</term>
<term>Sujet âgé</term>
<term>Tumeurs du rein ()</term>
<term>Tumeurs du rein (anatomopathologie)</term>
<term>Tumeurs primitives multiples</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Biomarkers, Tumor</term>
</keywords>
<keywords scheme="MESH" qualifier="analyse" xml:lang="fr"><term>Marqueurs biologiques tumoraux</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Néphrocarcinome</term>
<term>Sclérose tubéreuse du cerveau</term>
<term>Tumeurs du rein</term>
</keywords>
<keywords scheme="MESH" qualifier="chemistry" xml:lang="en"><term>Carcinoma, Renal Cell</term>
<term>Kidney Neoplasms</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Tuberous Sclerosis</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Sclérose tubéreuse du cerveau</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Carcinoma, Renal Cell</term>
<term>Kidney Neoplasms</term>
<term>Tuberous Sclerosis</term>
</keywords>
<keywords scheme="MESH" qualifier="surgery" xml:lang="en"><term>Carcinoma, Renal Cell</term>
<term>Kidney Neoplasms</term>
<term>Tuberous Sclerosis</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Aged</term>
<term>Biopsy</term>
<term>Child</term>
<term>Female</term>
<term>Humans</term>
<term>Immunohistochemistry</term>
<term>Immunophenotyping</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Neoplasm Recurrence, Local</term>
<term>Neoplasms, Multiple Primary</term>
<term>Neoplasms, Second Primary</term>
<term>Nephrectomy</term>
<term>Time Factors</term>
<term>Treatment Outcome</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Adolescent</term>
<term>Adulte</term>
<term>Adulte d'âge moyen</term>
<term>Biopsie</term>
<term>Enfant</term>
<term>Facteurs temps</term>
<term>Femelle</term>
<term>Humains</term>
<term>Immunohistochimie</term>
<term>Immunophénotypage</term>
<term>Jeune adulte</term>
<term>Mâle</term>
<term>Néphrectomie</term>
<term>Néphrocarcinome</term>
<term>Récidive tumorale locale</term>
<term>Résultat thérapeutique</term>
<term>Sclérose tubéreuse du cerveau</term>
<term>Seconde tumeur primitive</term>
<term>Sujet âgé</term>
<term>Tumeurs du rein</term>
<term>Tumeurs primitives multiples</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with characteristic tumors involving multiple organ systems. Whereas renal angiomyolipoma (AML) is common in TSC, renal cell carcinoma (RCC) is rarely reported. Fifty-seven RCCs from 13 female and 5 male TSC patients were reviewed. Age at surgery ranged from 7 to 65 years (mean: 42 y). Nine patients (50%) had multiple synchronous and/or metachronous RCCs (range of 2 to 20 RCCs) and 5 had bilateral RCCs (28%). Seventeen patients (94%) had histologically confirmed concurrent renal AMLs, including 15 with multiple AMLs (88%) and 9 (50%) with AMLs with epithelial cysts. None of the 15 patients with available clinical follow-up information had evidence of distant metastatic disease from 6 to 198 months after their initial surgery (mean: 52 mo). The 57 RCCs exhibited 3 major distinct morphologies: (1) 17 RCCs (30%) had features similar to tumors previously described as "renal angiomyoadenomatous tumor" or "RCC with smooth muscle stroma"; (2) 34 RCCs (59%) showed features similar to chromophobe RCC; and (3) 6 RCCs (11%) showed a granular eosinophilic-macrocystic morphology. Distinct histologic changes were also commonly present in the background kidney parenchyma and included cysts or renal tubules lined by epithelial cells with prominent eosinophilic cytoplasm, nucleomegaly, and nucleoli. Immunohistochemically, all RCCs tested showed strong nuclear reactivity for PAX8 and HMB45 negativity. Compared with sporadic RCCs, TSC-associated RCCs have unique clinicopathologic features including female predominance, younger age at diagnosis, multiplicity, association with AMLs, 3 recurring histologic patterns, and an indolent clinical course. Awareness of the morphologic and clinicopathologic spectrum of RCC in this setting will allow surgical pathologists to better recognize clinically unsuspected TSC patients.</div>
</front>
</TEI>
<pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">25093518</PMID>
<DateCreated><Year>2014</Year>
<Month>10</Month>
<Day>11</Day>
</DateCreated>
<DateCompleted><Year>2014</Year>
<Month>12</Month>
<Day>12</Day>
</DateCompleted>
<DateRevised><Year>2015</Year>
<Month>11</Month>
<Day>19</Day>
</DateRevised>
<Article PubModel="Print"><Journal><ISSN IssnType="Electronic">1532-0979</ISSN>
<JournalIssue CitedMedium="Internet"><Volume>38</Volume>
<Issue>11</Issue>
<PubDate><Year>2014</Year>
<Month>Nov</Month>
</PubDate>
</JournalIssue>
<Title>The American journal of surgical pathology</Title>
<ISOAbbreviation>Am. J. Surg. Pathol.</ISOAbbreviation>
</Journal>
<ArticleTitle>Tuberous sclerosis-associated renal cell carcinoma: a clinicopathologic study of 57 separate carcinomas in 18 patients.</ArticleTitle>
<Pagination><MedlinePgn>1457-67</MedlinePgn>
</Pagination>
<ELocationID EIdType="doi" ValidYN="Y">10.1097/PAS.0000000000000248</ELocationID>
<Abstract><AbstractText>Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with characteristic tumors involving multiple organ systems. Whereas renal angiomyolipoma (AML) is common in TSC, renal cell carcinoma (RCC) is rarely reported. Fifty-seven RCCs from 13 female and 5 male TSC patients were reviewed. Age at surgery ranged from 7 to 65 years (mean: 42 y). Nine patients (50%) had multiple synchronous and/or metachronous RCCs (range of 2 to 20 RCCs) and 5 had bilateral RCCs (28%). Seventeen patients (94%) had histologically confirmed concurrent renal AMLs, including 15 with multiple AMLs (88%) and 9 (50%) with AMLs with epithelial cysts. None of the 15 patients with available clinical follow-up information had evidence of distant metastatic disease from 6 to 198 months after their initial surgery (mean: 52 mo). The 57 RCCs exhibited 3 major distinct morphologies: (1) 17 RCCs (30%) had features similar to tumors previously described as "renal angiomyoadenomatous tumor" or "RCC with smooth muscle stroma"; (2) 34 RCCs (59%) showed features similar to chromophobe RCC; and (3) 6 RCCs (11%) showed a granular eosinophilic-macrocystic morphology. Distinct histologic changes were also commonly present in the background kidney parenchyma and included cysts or renal tubules lined by epithelial cells with prominent eosinophilic cytoplasm, nucleomegaly, and nucleoli. Immunohistochemically, all RCCs tested showed strong nuclear reactivity for PAX8 and HMB45 negativity. Compared with sporadic RCCs, TSC-associated RCCs have unique clinicopathologic features including female predominance, younger age at diagnosis, multiplicity, association with AMLs, 3 recurring histologic patterns, and an indolent clinical course. Awareness of the morphologic and clinicopathologic spectrum of RCC in this setting will allow surgical pathologists to better recognize clinically unsuspected TSC patients.</AbstractText>
</Abstract>
<AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Guo</LastName>
<ForeName>Juan</ForeName>
<Initials>J</Initials>
<AffiliationInfo><Affiliation>*Cleveland Clinic, Robert J. Tomsich Pathology & Laboratory Medicine Institute ***Glickman Urological and Kidney Institute, Cleveland, OH †Department of Pathology, University of Washington, Seattle, WA ‡Department of Pathology, Oregon Health & Science University, Portland, OR §Department of Pathology, Emory University, Atlanta, GA ∥Department of Pathology, Vanderbilt University, Nashville, TN ¶Department of Pathology, El Camino Hospital, Mountain View ‡‡Department of Pathology, Stanford University, Stanford, CA #Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX ††Department of Pathology, University of Michigan, Ann Arbor, MI §§Department of Pathology, Columbia University, New York, NY **Department of Anatomic Pathology, University of Cordoba, Cordoba, Spain ∥∥Department of Pathology, Lille University Hospitals, Lille, France ¶¶Department of Anatomic Pathology, Royal North Shore Hospital, University of Sydney, St Leonards, NSW, Australia ##Department of Pathology and Laboratory Medicine, University of Calgary and Calgary Laboratory Services, Calgary, AB, Canada.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Tretiakova</LastName>
<ForeName>Maria S</ForeName>
<Initials>MS</Initials>
</Author>
<Author ValidYN="Y"><LastName>Troxell</LastName>
<ForeName>Megan L</ForeName>
<Initials>ML</Initials>
</Author>
<Author ValidYN="Y"><LastName>Osunkoya</LastName>
<ForeName>Adeboye O</ForeName>
<Initials>AO</Initials>
</Author>
<Author ValidYN="Y"><LastName>Fadare</LastName>
<ForeName>Oluwole</ForeName>
<Initials>O</Initials>
</Author>
<Author ValidYN="Y"><LastName>Sangoi</LastName>
<ForeName>Ankur R</ForeName>
<Initials>AR</Initials>
</Author>
<Author ValidYN="Y"><LastName>Shen</LastName>
<ForeName>Steven S</ForeName>
<Initials>SS</Initials>
</Author>
<Author ValidYN="Y"><LastName>Lopez-Beltran</LastName>
<ForeName>Antonio</ForeName>
<Initials>A</Initials>
</Author>
<Author ValidYN="Y"><LastName>Mehra</LastName>
<ForeName>Rohit</ForeName>
<Initials>R</Initials>
</Author>
<Author ValidYN="Y"><LastName>Heider</LastName>
<ForeName>Amer</ForeName>
<Initials>A</Initials>
</Author>
<Author ValidYN="Y"><LastName>Higgins</LastName>
<ForeName>John P</ForeName>
<Initials>JP</Initials>
</Author>
<Author ValidYN="Y"><LastName>Harik</LastName>
<ForeName>Lara R</ForeName>
<Initials>LR</Initials>
</Author>
<Author ValidYN="Y"><LastName>Leroy</LastName>
<ForeName>Xavier</ForeName>
<Initials>X</Initials>
</Author>
<Author ValidYN="Y"><LastName>Gill</LastName>
<ForeName>Anthony J</ForeName>
<Initials>AJ</Initials>
</Author>
<Author ValidYN="Y"><LastName>Trpkov</LastName>
<ForeName>Kiril</ForeName>
<Initials>K</Initials>
</Author>
<Author ValidYN="Y"><LastName>Campbell</LastName>
<ForeName>Steven C</ForeName>
<Initials>SC</Initials>
</Author>
<Author ValidYN="Y"><LastName>Przybycin</LastName>
<ForeName>Christopher</ForeName>
<Initials>C</Initials>
</Author>
<Author ValidYN="Y"><LastName>Magi-Galluzzi</LastName>
<ForeName>Cristina</ForeName>
<Initials>C</Initials>
</Author>
<Author ValidYN="Y"><LastName>McKenney</LastName>
<ForeName>Jesse K</ForeName>
<Initials>JK</Initials>
</Author>
</AuthorList>
<Language>eng</Language>
<PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType>
<PublicationType UI="D016448">Multicenter Study</PublicationType>
</PublicationTypeList>
</Article>
<MedlineJournalInfo><Country>United States</Country>
<MedlineTA>Am J Surg Pathol</MedlineTA>
<NlmUniqueID>7707904</NlmUniqueID>
<ISSNLinking>0147-5185</ISSNLinking>
</MedlineJournalInfo>
<ChemicalList><Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D014408">Biomarkers, Tumor</NameOfSubstance>
</Chemical>
</ChemicalList>
<CitationSubset>IM</CitationSubset>
<MeshHeadingList><MeshHeading><DescriptorName UI="D000293" MajorTopicYN="N">Adolescent</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D000368" MajorTopicYN="N">Aged</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D014408" MajorTopicYN="N">Biomarkers, Tumor</DescriptorName>
<QualifierName UI="Q000032" MajorTopicYN="N">analysis</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D001706" MajorTopicYN="N">Biopsy</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D002292" MajorTopicYN="N">Carcinoma, Renal Cell</DescriptorName>
<QualifierName UI="Q000737" MajorTopicYN="N">chemistry</QualifierName>
<QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName>
<QualifierName UI="Q000601" MajorTopicYN="N">surgery</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D002648" MajorTopicYN="N">Child</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D007150" MajorTopicYN="N">Immunohistochemistry</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D016130" MajorTopicYN="N">Immunophenotyping</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D007680" MajorTopicYN="N">Kidney Neoplasms</DescriptorName>
<QualifierName UI="Q000737" MajorTopicYN="N">chemistry</QualifierName>
<QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName>
<QualifierName UI="Q000601" MajorTopicYN="N">surgery</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D009364" MajorTopicYN="N">Neoplasm Recurrence, Local</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D009378" MajorTopicYN="Y">Neoplasms, Multiple Primary</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D016609" MajorTopicYN="Y">Neoplasms, Second Primary</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D009392" MajorTopicYN="N">Nephrectomy</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D013997" MajorTopicYN="N">Time Factors</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D016896" MajorTopicYN="N">Treatment Outcome</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D014402" MajorTopicYN="N">Tuberous Sclerosis</DescriptorName>
<QualifierName UI="Q000378" MajorTopicYN="N">metabolism</QualifierName>
<QualifierName UI="Q000473" MajorTopicYN="Y">pathology</QualifierName>
<QualifierName UI="Q000601" MajorTopicYN="N">surgery</QualifierName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D055815" MajorTopicYN="N">Young Adult</DescriptorName>
</MeshHeading>
</MeshHeadingList>
</MedlineCitation>
<PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2014</Year>
<Month>8</Month>
<Day>6</Day>
<Hour>6</Hour>
<Minute>0</Minute>
</PubMedPubDate>
<PubMedPubDate PubStatus="pubmed"><Year>2014</Year>
<Month>8</Month>
<Day>6</Day>
<Hour>6</Hour>
<Minute>0</Minute>
</PubMedPubDate>
<PubMedPubDate PubStatus="medline"><Year>2014</Year>
<Month>12</Month>
<Day>17</Day>
<Hour>6</Hour>
<Minute>0</Minute>
</PubMedPubDate>
</History>
<PublicationStatus>ppublish</PublicationStatus>
<ArticleIdList><ArticleId IdType="pubmed">25093518</ArticleId>
<ArticleId IdType="doi">10.1097/PAS.0000000000000248</ArticleId>
</ArticleIdList>
</PubmedData>
</pubmed>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Asie/explor/AustralieFrV1/Data/PubMed/Curation
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 003245 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/PubMed/Curation/biblio.hfd -nk 003245 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Asie |area= AustralieFrV1 |flux= PubMed |étape= Curation |type= RBID |clé= pubmed:25093518 |texte= Tuberous sclerosis-associated renal cell carcinoma: a clinicopathologic study of 57 separate carcinomas in 18 patients. }}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/PubMed/Curation/RBID.i -Sk "pubmed:25093518" \ | HfdSelect -Kh $EXPLOR_AREA/Data/PubMed/Curation/biblio.hfd \ | NlmPubMed2Wicri -a AustralieFrV1
![]() | This area was generated with Dilib version V0.6.33. | ![]() |