Nodular Proliferation in Parkes Weber Syndrome.
Identifieur interne : 000341 ( Main/Exploration ); précédent : 000340; suivant : 000342Nodular Proliferation in Parkes Weber Syndrome.
Auteurs : Diego José Rojas Esquivel [Espagne] ; Elena Marín Manzano [Espagne] ; Nieves Aleicel Concepci N [Espagne] ; Juan Carlos Lopéz Gutierréz [Espagne] ; Luis Riera De Cubas [Espagne]Source :
- Annals of vascular surgery [ 1615-5947 ] ; 2017.
Descripteurs français
- KwdFr :
- Adulte, Angiographie par tomodensitométrie, Anticorps monoclonaux d'origine murine, Antigènes CD31 (analyse), Antigènes CD34 (immunologie), Artères (), Artères (anatomopathologie), Artères (imagerie diagnostique), Biopsie, Humains, Immunohistochimie, Malformations artérioveineuses (), Malformations artérioveineuses (anatomopathologie), Malformations artérioveineuses (imagerie diagnostique), Membre inférieur (), Mâle, Procédures de chirurgie vasculaire, Prolifération cellulaire, Résultat thérapeutique, Syndrome de Sturge-Weber (), Syndrome de Sturge-Weber (anatomopathologie), Syndrome de Sturge-Weber (imagerie diagnostique), Veines (), Veines (anatomopathologie), Veines (imagerie diagnostique).
- MESH :
- analyse : Antigènes CD31.
- anatomopathologie : Artères, Malformations artérioveineuses, Syndrome de Sturge-Weber, Veines.
- imagerie diagnostique : Artères, Malformations artérioveineuses, Syndrome de Sturge-Weber, Veines.
- immunologie : Antigènes CD34.
- Adulte, Angiographie par tomodensitométrie, Anticorps monoclonaux d'origine murine, Artères, Biopsie, Humains, Immunohistochimie, Malformations artérioveineuses, Membre inférieur, Mâle, Procédures de chirurgie vasculaire, Prolifération cellulaire, Résultat thérapeutique, Syndrome de Sturge-Weber, Veines.
English descriptors
- KwdEn :
- Adult, Antibodies, Monoclonal, Murine-Derived, Antigens, CD31 (analysis), Antigens, CD34 (immunology), Arteries (chemistry), Arteries (diagnostic imaging), Arteries (pathology), Arteries (surgery), Arteriovenous Malformations (diagnostic imaging), Arteriovenous Malformations (pathology), Arteriovenous Malformations (surgery), Biopsy, Cell Proliferation, Computed Tomography Angiography, Humans, Immunohistochemistry, Lower Extremity (blood supply), Male, Sturge-Weber Syndrome (diagnostic imaging), Sturge-Weber Syndrome (pathology), Sturge-Weber Syndrome (surgery), Treatment Outcome, Vascular Surgical Procedures, Veins (chemistry), Veins (diagnostic imaging), Veins (pathology), Veins (surgery).
- MESH :
- chemical , analysis : Antigens, CD31.
- chemical , immunology : Antigens, CD34.
- chemical : Antibodies, Monoclonal, Murine-Derived.
- blood supply : Lower Extremity.
- chemistry : Arteries, Veins.
- diagnostic imaging : Arteries, Arteriovenous Malformations, Sturge-Weber Syndrome, Veins.
- pathology : Arteries, Arteriovenous Malformations, Sturge-Weber Syndrome, Veins.
- surgery : Arteries, Arteriovenous Malformations, Sturge-Weber Syndrome, Veins.
- Adult, Biopsy, Cell Proliferation, Computed Tomography Angiography, Humans, Immunohistochemistry, Male, Treatment Outcome, Vascular Surgical Procedures.
Abstract
Parkes Weber syndrome (PWS) is characterized by the association of high flow vascular malformation and overgrowth of a part of the body, usually a limb. In a previous review of 10 patients with PWS from our hospital we described a case of congenital short femur and four cases of severe lymphedema. We present a case of PWS associated with a nodular proliferative form not previously described. A 38 year old male with diagnosis of PWS with involvement of the right lower limb (RLL) was derived to our clinic. He complained about the appearance of painful nodular tumors in his RLL and some episodes of bleeding through the tumors. The physical examination revealed increased size of the RLL compared to left lower limb. Two nodular tumors were evident in his RLL. One located proximal in the leg and another one in ankle. The computed tomographic angiography revealed multiple arterio-venous shunts in the RLL. The tumors were not arterio-venous shunts, neither aneurysms. We decided to make surgical resection of the tumors. In the pathology analysis the tumors were positive for CD31, CD34 and negative for D240 markers. Eight months after surgery the patient had no recurrence of the tumors, and he is asymptomatic.The presence of nodular tumors in PWS has not been previously described. This makes us to think that these could be hamartomatous lesions similar to those of the CLOVES syndrome or a PIK3CA mutation.
DOI: 10.1016/j.avsg.2016.06.015
PubMed: 27575310
Affiliations:
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sort="Concepci N, Nieves Aleicel" uniqKey="Concepci N N" first="Nieves Aleicel" last="Concepci N">Nieves Aleicel Concepci N</name><affiliation wicri:level="3"><nlm:affiliation>Department of Vascular Surgery, La Paz University Hospital, Madrid, Spain.</nlm:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Department of Vascular Surgery, La Paz University Hospital, Madrid</wicri:regionArea><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Lopez Gutierrez, Juan Carlos" sort="Lopez Gutierrez, Juan Carlos" uniqKey="Lopez Gutierrez J" first="Juan Carlos" last="Lopéz Gutierréz">Juan Carlos Lopéz Gutierréz</name><affiliation wicri:level="3"><nlm:affiliation>Department of Pediatric Surgery, La Paz University Hospital, Madrid, Spain.</nlm:affiliation><country xml:lang="fr">Espagne</country><wicri:regionArea>Department of Pediatric Surgery, La Paz 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type="published">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Antibodies, Monoclonal, Murine-Derived</term><term>Antigens, CD31 (analysis)</term><term>Antigens, CD34 (immunology)</term><term>Arteries (chemistry)</term><term>Arteries (diagnostic imaging)</term><term>Arteries (pathology)</term><term>Arteries (surgery)</term><term>Arteriovenous Malformations (diagnostic imaging)</term><term>Arteriovenous Malformations (pathology)</term><term>Arteriovenous Malformations (surgery)</term><term>Biopsy</term><term>Cell Proliferation</term><term>Computed Tomography Angiography</term><term>Humans</term><term>Immunohistochemistry</term><term>Lower Extremity (blood supply)</term><term>Male</term><term>Sturge-Weber Syndrome (diagnostic imaging)</term><term>Sturge-Weber Syndrome (pathology)</term><term>Sturge-Weber Syndrome (surgery)</term><term>Treatment Outcome</term><term>Vascular Surgical Procedures</term><term>Veins (chemistry)</term><term>Veins (diagnostic imaging)</term><term>Veins (pathology)</term><term>Veins (surgery)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Angiographie par tomodensitométrie</term><term>Anticorps monoclonaux d'origine murine</term><term>Antigènes CD31 (analyse)</term><term>Antigènes CD34 (immunologie)</term><term>Artères ()</term><term>Artères (anatomopathologie)</term><term>Artères (imagerie diagnostique)</term><term>Biopsie</term><term>Humains</term><term>Immunohistochimie</term><term>Malformations artérioveineuses ()</term><term>Malformations artérioveineuses (anatomopathologie)</term><term>Malformations artérioveineuses (imagerie diagnostique)</term><term>Membre inférieur ()</term><term>Mâle</term><term>Procédures de chirurgie vasculaire</term><term>Prolifération cellulaire</term><term>Résultat thérapeutique</term><term>Syndrome de Sturge-Weber ()</term><term>Syndrome de Sturge-Weber (anatomopathologie)</term><term>Syndrome de Sturge-Weber (imagerie diagnostique)</term><term>Veines ()</term><term>Veines (anatomopathologie)</term><term>Veines (imagerie diagnostique)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Antigens, CD31</term></keywords><keywords scheme="MESH" type="chemical" qualifier="immunology" xml:lang="en"><term>Antigens, CD34</term></keywords><keywords scheme="MESH" type="chemical" xml:lang="en"><term>Antibodies, Monoclonal, Murine-Derived</term></keywords><keywords scheme="MESH" qualifier="analyse" xml:lang="fr"><term>Antigènes CD31</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Artères</term><term>Malformations artérioveineuses</term><term>Syndrome de Sturge-Weber</term><term>Veines</term></keywords><keywords scheme="MESH" qualifier="blood supply" xml:lang="en"><term>Lower Extremity</term></keywords><keywords scheme="MESH" qualifier="chemistry" xml:lang="en"><term>Arteries</term><term>Veins</term></keywords><keywords scheme="MESH" qualifier="diagnostic imaging" xml:lang="en"><term>Arteries</term><term>Arteriovenous Malformations</term><term>Sturge-Weber Syndrome</term><term>Veins</term></keywords><keywords scheme="MESH" qualifier="imagerie diagnostique" xml:lang="fr"><term>Artères</term><term>Malformations artérioveineuses</term><term>Syndrome de Sturge-Weber</term><term>Veines</term></keywords><keywords scheme="MESH" qualifier="immunologie" xml:lang="fr"><term>Antigènes CD34</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Arteries</term><term>Arteriovenous Malformations</term><term>Sturge-Weber Syndrome</term><term>Veins</term></keywords><keywords scheme="MESH" qualifier="surgery" xml:lang="en"><term>Arteries</term><term>Arteriovenous Malformations</term><term>Sturge-Weber Syndrome</term><term>Veins</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Biopsy</term><term>Cell Proliferation</term><term>Computed Tomography Angiography</term><term>Humans</term><term>Immunohistochemistry</term><term>Male</term><term>Treatment Outcome</term><term>Vascular Surgical Procedures</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Angiographie par tomodensitométrie</term><term>Anticorps monoclonaux d'origine murine</term><term>Artères</term><term>Biopsie</term><term>Humains</term><term>Immunohistochimie</term><term>Malformations artérioveineuses</term><term>Membre inférieur</term><term>Mâle</term><term>Procédures de chirurgie vasculaire</term><term>Prolifération cellulaire</term><term>Résultat thérapeutique</term><term>Syndrome de Sturge-Weber</term><term>Veines</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Parkes Weber syndrome (PWS) is characterized by the association of high flow vascular malformation and overgrowth of a part of the body, usually a limb. In a previous review of 10 patients with PWS from our hospital we described a case of congenital short femur and four cases of severe lymphedema. We present a case of PWS associated with a nodular proliferative form not previously described. A 38 year old male with diagnosis of PWS with involvement of the right lower limb (RLL) was derived to our clinic. He complained about the appearance of painful nodular tumors in his RLL and some episodes of bleeding through the tumors. The physical examination revealed increased size of the RLL compared to left lower limb. Two nodular tumors were evident in his RLL. One located proximal in the leg and another one in ankle. The computed tomographic angiography revealed multiple arterio-venous shunts in the RLL. The tumors were not arterio-venous shunts, neither aneurysms. We decided to make surgical resection of the tumors. In the pathology analysis the tumors were positive for CD31, CD34 and negative for D240 markers. Eight months after surgery the patient had no recurrence of the tumors, and he is asymptomatic.The presence of nodular tumors in PWS has not been previously described. This makes us to think that these could be hamartomatous lesions similar to those of the CLOVES syndrome or a PIK3CA mutation.</div></front></TEI><affiliations><list><country><li>Espagne</li></country><region><li>Communauté de Madrid</li></region><settlement><li>Madrid</li></settlement></list><tree><country name="Espagne"><region name="Communauté de Madrid"><name sortKey="Rojas Esquivel, Diego Jose" sort="Rojas Esquivel, Diego Jose" uniqKey="Rojas Esquivel D" first="Diego José" last="Rojas Esquivel">Diego José Rojas Esquivel</name></region><name sortKey="Concepci N, Nieves Aleicel" sort="Concepci N, Nieves Aleicel" uniqKey="Concepci N N" first="Nieves Aleicel" last="Concepci N">Nieves Aleicel Concepci N</name><name sortKey="Lopez Gutierrez, Juan Carlos" sort="Lopez Gutierrez, Juan Carlos" uniqKey="Lopez Gutierrez J" first="Juan Carlos" last="Lopéz Gutierréz">Juan Carlos Lopéz Gutierréz</name><name sortKey="Marin Manzano, Elena" sort="Marin Manzano, Elena" uniqKey="Marin Manzano E" first="Elena" last="Marín Manzano">Elena Marín Manzano</name><name sortKey="Riera De Cubas, Luis" sort="Riera De Cubas, Luis" uniqKey="Riera De Cubas L" first="Luis" last="Riera De Cubas">Luis Riera De Cubas</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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