Giant angiofibromas in tuberous sclerosis complex: A possible role for localized lymphedema in their pathogenesis
Identifieur interne : 004941 ( Main/Exploration ); précédent : 004940; suivant : 004942Giant angiofibromas in tuberous sclerosis complex: A possible role for localized lymphedema in their pathogenesis
Auteurs : Denisa Kacerovska [République tchèque] ; Katrin Kerl [République tchèque] ; Michal Michal [République tchèque] ; Hana Filipova [République tchèque] ; Radek Vrtel [République tchèque] ; Tomas Vanecek [République tchèque] ; Hana Zelenakova [République tchèque] ; Jaroslav Kraus [République tchèque] ; Roman Kodet [République tchèque] ; Dmitry V. Kazakov [République tchèque]Source :
- Journal of the American Academy of Dermatology [ 0190-9622 ] ; 2012.
Descripteurs français
- Pascal (Inist)
English descriptors
Abstract
Background: Giant angiofibromas in patients with tuberous sclerosis complex (TSC) are rare. Objective: We sought to report two patients who had TSC with unusually large and disfiguring facial angiofibromas and to identify underlying histopathologic changes that may possibly explain the clinical features. Methods: We performed a clinicopathologic, immunohistochemical, and molecular biologic study using 42 lesional specimens and peripheral blood from one of the two patients. The immunohistochemical investigations were mainly focused on the vascular moiety of the lesions. TSC1 and TSC2 alterations were studied using multiplex ligation-dependent probe amplification for large deletion/duplication mutations, whereas screening for small mutations was performed using polymerase chain reaction amplification of individual coding exons and exon-intron junctions of both genes followed by an analysis on denaturation gradient gel electrophoresis. Results: Histopathologic examination revealed, in addition to findings typical of angiofibroma, several unusual features including multinucleated giant cells containing multiple intracytoplasmic vacuoles, Touton-like cells, emperipolesis, pagetoid dyskeratosis, vacuolar alteration at the dermoepidermal junction, Civatte bodies, and melanophages in the subjacent dermis. Numerous dilated lymphatic vessels were detected indicating localized lymphostasis, probably caused by secondary lymphedema. The lymphatic nature of the vessels was confirmed by immunohistochemical study. Genetic testing for TSC1 and TSC2 gene mutations revealed a substitution on position c.2251C>T resulting in a nonsense mutation R751X in fragment 20.2. Limitations: Histopathologic specimens and peripheral blood were available from only one patient. Conclusion: Localized lymphedema may contribute to the formation of large disfiguring angiofibromas in patients with TSC.
Affiliations:
Links toward previous steps (curation, corpus...)
- to stream PascalFrancis, to step Corpus: 000048
- to stream PascalFrancis, to step Curation: 000907
- to stream PascalFrancis, to step Checkpoint: 000098
- to stream Main, to step Merge: 004970
- to stream Main, to step Curation: 004941
Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Giant angiofibromas in tuberous sclerosis complex: A possible role for localized lymphedema in their pathogenesis</title><author><name sortKey="Kacerovska, Denisa" sort="Kacerovska, Denisa" uniqKey="Kacerovska D" first="Denisa" last="Kacerovska">Denisa Kacerovska</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author><author><name sortKey="Kerl, Katrin" sort="Kerl, Katrin" uniqKey="Kerl K" first="Katrin" last="Kerl">Katrin Kerl</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Dermatology, University Hospital</s1><s2>Zurich</s2><s3>CZE</s3><sZ>2 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Michal, Michal" sort="Michal, Michal" uniqKey="Michal M" first="Michal" last="Michal">Michal Michal</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author><author><name sortKey="Filipova, Hana" sort="Filipova, Hana" uniqKey="Filipova H" first="Hana" last="Filipova">Hana Filipova</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Clinical Genetics and Fetal Medicine, University Hospital</s1><s2>Olomouc</s2><s3>CZE</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Olomouc</wicri:noRegion></affiliation></author><author><name sortKey="Vrtel, Radek" sort="Vrtel, Radek" uniqKey="Vrtel R" first="Radek" last="Vrtel">Radek Vrtel</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Clinical Genetics and Fetal Medicine, University Hospital</s1><s2>Olomouc</s2><s3>CZE</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Olomouc</wicri:noRegion></affiliation></author><author><name sortKey="Vanecek, Tomas" sort="Vanecek, Tomas" uniqKey="Vanecek T" first="Tomas" last="Vanecek">Tomas Vanecek</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author><author><name sortKey="Zelenakova, Hana" sort="Zelenakova, Hana" uniqKey="Zelenakova H" first="Hana" last="Zelenakova">Hana Zelenakova</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Diagnostic Institution of Social Care</s1><s2>Tloskov</s2><s3>CZE</s3><sZ>7 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Diagnostic Institution of Social Care</wicri:noRegion></affiliation></author><author><name sortKey="Kraus, Jaroslav" sort="Kraus, Jaroslav" uniqKey="Kraus J" first="Jaroslav" last="Kraus">Jaroslav Kraus</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Otorhinolaryngology, Hospital Benesov</s1><s3>CZE</s3><sZ>8 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Department of Otorhinolaryngology, Hospital Benesov</wicri:noRegion></affiliation></author><author><name sortKey="Kodet, Roman" sort="Kodet, Roman" uniqKey="Kodet R" first="Roman" last="Kodet">Roman Kodet</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Institution of Pathology and Molecular Medicine, Second Medical Faculty and Teacher's Hospital</s1><s2>Prague</s2><s3>CZE</s3><sZ>9 aut.</sZ></inist:fA14><country>République tchèque</country><placeName><settlement type="city">Prague</settlement><region type="région" nuts="2">Bohême centrale</region></placeName></affiliation></author><author><name sortKey="Kazakov, Dmitry V" sort="Kazakov, Dmitry V" uniqKey="Kazakov D" first="Dmitry V." last="Kazakov">Dmitry V. Kazakov</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">13-0031362</idno><date when="2012">2012</date><idno type="stanalyst">PASCAL 13-0031362 INIST</idno><idno type="RBID">Pascal:13-0031362</idno><idno type="wicri:Area/PascalFrancis/Corpus">000048</idno><idno type="wicri:Area/PascalFrancis/Curation">000907</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">000098</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">000098</idno><idno type="wicri:doubleKey">0190-9622:2012:Kacerovska D:giant:angiofibromas:in</idno><idno type="wicri:Area/Main/Merge">004970</idno><idno type="wicri:Area/Main/Curation">004941</idno><idno type="wicri:Area/Main/Exploration">004941</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Giant angiofibromas in tuberous sclerosis complex: A possible role for localized lymphedema in their pathogenesis</title><author><name sortKey="Kacerovska, Denisa" sort="Kacerovska, Denisa" uniqKey="Kacerovska D" first="Denisa" last="Kacerovska">Denisa Kacerovska</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author><author><name sortKey="Kerl, Katrin" sort="Kerl, Katrin" uniqKey="Kerl K" first="Katrin" last="Kerl">Katrin Kerl</name><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Department of Dermatology, University Hospital</s1><s2>Zurich</s2><s3>CZE</s3><sZ>2 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Michal, Michal" sort="Michal, Michal" uniqKey="Michal M" first="Michal" last="Michal">Michal Michal</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author><author><name sortKey="Filipova, Hana" sort="Filipova, Hana" uniqKey="Filipova H" first="Hana" last="Filipova">Hana Filipova</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Clinical Genetics and Fetal Medicine, University Hospital</s1><s2>Olomouc</s2><s3>CZE</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Olomouc</wicri:noRegion></affiliation></author><author><name sortKey="Vrtel, Radek" sort="Vrtel, Radek" uniqKey="Vrtel R" first="Radek" last="Vrtel">Radek Vrtel</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Department of Clinical Genetics and Fetal Medicine, University Hospital</s1><s2>Olomouc</s2><s3>CZE</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Olomouc</wicri:noRegion></affiliation></author><author><name sortKey="Vanecek, Tomas" sort="Vanecek, Tomas" uniqKey="Vanecek T" first="Tomas" last="Vanecek">Tomas Vanecek</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author><author><name sortKey="Zelenakova, Hana" sort="Zelenakova, Hana" uniqKey="Zelenakova H" first="Hana" last="Zelenakova">Hana Zelenakova</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Diagnostic Institution of Social Care</s1><s2>Tloskov</s2><s3>CZE</s3><sZ>7 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Diagnostic Institution of Social Care</wicri:noRegion></affiliation></author><author><name sortKey="Kraus, Jaroslav" sort="Kraus, Jaroslav" uniqKey="Kraus J" first="Jaroslav" last="Kraus">Jaroslav Kraus</name><affiliation wicri:level="1"><inist:fA14 i1="06"><s1>Department of Otorhinolaryngology, Hospital Benesov</s1><s3>CZE</s3><sZ>8 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Department of Otorhinolaryngology, Hospital Benesov</wicri:noRegion></affiliation></author><author><name sortKey="Kodet, Roman" sort="Kodet, Roman" uniqKey="Kodet R" first="Roman" last="Kodet">Roman Kodet</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>Institution of Pathology and Molecular Medicine, Second Medical Faculty and Teacher's Hospital</s1><s2>Prague</s2><s3>CZE</s3><sZ>9 aut.</sZ></inist:fA14><country>République tchèque</country><placeName><settlement type="city">Prague</settlement><region type="région" nuts="2">Bohême centrale</region></placeName></affiliation></author><author><name sortKey="Kazakov, Dmitry V" sort="Kazakov, Dmitry V" uniqKey="Kazakov D" first="Dmitry V." last="Kazakov">Dmitry V. Kazakov</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Sikl's Department of Pathology, Charles University in Prague, Faculty of Medicine</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Pilsen</wicri:noRegion></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Bioptical Laboratory</s1><s2>Pilsen</s2><s3>CZE</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>6 aut.</sZ><sZ>10 aut.</sZ></inist:fA14><country>République tchèque</country><wicri:noRegion>Bioptical Laboratory</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Journal of the American Academy of Dermatology</title><title level="j" type="abbreviated">J. Am. Acad. Dermatol.</title><idno type="ISSN">0190-9622</idno><imprint><date when="2012">2012</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Journal of the American Academy of Dermatology</title><title level="j" type="abbreviated">J. Am. Acad. Dermatol.</title><idno type="ISSN">0190-9622</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Angiofibroma</term><term>Bourneville syndrome</term><term>Dermatology</term><term>Localized</term><term>Lymphedema</term><term>Pathogenesis</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Angiofibrome</term><term>Phacomatose de Bourneville</term><term>Lymphoedème</term><term>Localisé</term><term>Pathogénie</term><term>Dermatologie</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background: Giant angiofibromas in patients with tuberous sclerosis complex (TSC) are rare. Objective: We sought to report two patients who had TSC with unusually large and disfiguring facial angiofibromas and to identify underlying histopathologic changes that may possibly explain the clinical features. Methods: We performed a clinicopathologic, immunohistochemical, and molecular biologic study using 42 lesional specimens and peripheral blood from one of the two patients. The immunohistochemical investigations were mainly focused on the vascular moiety of the lesions. TSC1 and TSC2 alterations were studied using multiplex ligation-dependent probe amplification for large deletion/duplication mutations, whereas screening for small mutations was performed using polymerase chain reaction amplification of individual coding exons and exon-intron junctions of both genes followed by an analysis on denaturation gradient gel electrophoresis. Results: Histopathologic examination revealed, in addition to findings typical of angiofibroma, several unusual features including multinucleated giant cells containing multiple intracytoplasmic vacuoles, Touton-like cells, emperipolesis, pagetoid dyskeratosis, vacuolar alteration at the dermoepidermal junction, Civatte bodies, and melanophages in the subjacent dermis. Numerous dilated lymphatic vessels were detected indicating localized lymphostasis, probably caused by secondary lymphedema. The lymphatic nature of the vessels was confirmed by immunohistochemical study. Genetic testing for TSC1 and TSC2 gene mutations revealed a substitution on position c.2251C>T resulting in a nonsense mutation R751X in fragment 20.2. Limitations: Histopathologic specimens and peripheral blood were available from only one patient. Conclusion: Localized lymphedema may contribute to the formation of large disfiguring angiofibromas in patients with TSC.</div></front></TEI><affiliations><list><country><li>République tchèque</li></country><region><li>Bohême centrale</li></region><settlement><li>Prague</li></settlement></list><tree><country name="République tchèque"><noRegion><name sortKey="Kacerovska, Denisa" sort="Kacerovska, Denisa" uniqKey="Kacerovska D" first="Denisa" last="Kacerovska">Denisa Kacerovska</name></noRegion><name sortKey="Filipova, Hana" sort="Filipova, Hana" uniqKey="Filipova H" first="Hana" last="Filipova">Hana Filipova</name><name sortKey="Kacerovska, Denisa" sort="Kacerovska, Denisa" uniqKey="Kacerovska D" first="Denisa" last="Kacerovska">Denisa Kacerovska</name><name sortKey="Kazakov, Dmitry V" sort="Kazakov, Dmitry V" uniqKey="Kazakov D" first="Dmitry V." last="Kazakov">Dmitry V. Kazakov</name><name sortKey="Kazakov, Dmitry V" sort="Kazakov, Dmitry V" uniqKey="Kazakov D" first="Dmitry V." last="Kazakov">Dmitry V. Kazakov</name><name sortKey="Kerl, Katrin" sort="Kerl, Katrin" uniqKey="Kerl K" first="Katrin" last="Kerl">Katrin Kerl</name><name sortKey="Kodet, Roman" sort="Kodet, Roman" uniqKey="Kodet R" first="Roman" last="Kodet">Roman Kodet</name><name sortKey="Kraus, Jaroslav" sort="Kraus, Jaroslav" uniqKey="Kraus J" first="Jaroslav" last="Kraus">Jaroslav Kraus</name><name sortKey="Michal, Michal" sort="Michal, Michal" uniqKey="Michal M" first="Michal" last="Michal">Michal Michal</name><name sortKey="Michal, Michal" sort="Michal, Michal" uniqKey="Michal M" first="Michal" last="Michal">Michal Michal</name><name sortKey="Vanecek, Tomas" sort="Vanecek, Tomas" uniqKey="Vanecek T" first="Tomas" last="Vanecek">Tomas Vanecek</name><name sortKey="Vanecek, Tomas" sort="Vanecek, Tomas" uniqKey="Vanecek T" first="Tomas" last="Vanecek">Tomas Vanecek</name><name sortKey="Vrtel, Radek" sort="Vrtel, Radek" uniqKey="Vrtel R" first="Radek" last="Vrtel">Radek Vrtel</name><name sortKey="Zelenakova, Hana" sort="Zelenakova, Hana" uniqKey="Zelenakova H" first="Hana" last="Zelenakova">Hana Zelenakova</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/LymphedemaV1/Data/Main/Exploration
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 004941 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Main/Exploration/biblio.hfd -nk 004941 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Sante
|area= LymphedemaV1
|flux= Main
|étape= Exploration
|type= RBID
|clé= Pascal:13-0031362
|texte= Giant angiofibromas in tuberous sclerosis complex: A possible role for localized lymphedema in their pathogenesis
}}
| This area was generated with Dilib version V0.6.31. |  |