Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema
Identifieur interne : 001039 ( PascalFrancis/Curation ); précédent : 001038; suivant : 001040Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema
Auteurs : A. Cerri [Italie] ; C. Gianni [Italie] ; M. Corbellino [Italie] ; M. Pizzuto [Italie] ; L. Moneghini [Italie] ; C. Crosti [Italie]Source :
- EJD. European journal of dermatology [ 1167-1122 ] ; 1998.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
Abstract
Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequently associated with post-mastectomy lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally arise in congenital hereditary lymphedema (Milroy's syndrome and Meige's syndrome) and non-hereditary lymphedema (congenital, praecox or forme tarde lymphedemas). We report a case of lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-hereditary lymphedema of the left genital region and homolateral lower limb. In addition, molecular analysis demonstrated the absence of Kaposi's sarcoma-associated Herpes virus (KSHV) DNA sequences in tumour lesions. To our knowledge, this is the first case of lymphangiosarcoma associated with congenital non-hereditary lymphedema confined to the pubic region. The literature concerning the cases of lymphangiosarcoma arising in congenital hereditary and non-hereditary lymphedema is reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphedema. In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients.
pA |
|
---|
Links toward previous steps (curation, corpus...)
- to stream PascalFrancis, to step Corpus: Pour aller vers cette notice dans l'étape Curation :000A69
Links to Exploration step
Pascal:98-0488381Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema</title>
<author><name sortKey="Cerri, A" sort="Cerri, A" uniqKey="Cerri A" first="A." last="Cerri">A. Cerri</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Gianni, C" sort="Gianni, C" uniqKey="Gianni C" first="C." last="Gianni">C. Gianni</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Corbellino, M" sort="Corbellino, M" uniqKey="Corbellino M" first="M." last="Corbellino">M. Corbellino</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Istituto di Malattie Infettive e Tropicali, Università di Milano</s1>
<s3>ITA</s3>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Pizzuto, M" sort="Pizzuto, M" uniqKey="Pizzuto M" first="M." last="Pizzuto">M. Pizzuto</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Istituto di Malattie Infettive e Tropicali, Università di Milano</s1>
<s3>ITA</s3>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Moneghini, L" sort="Moneghini, L" uniqKey="Moneghini L" first="L." last="Moneghini">L. Moneghini</name>
<affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano</s1>
<s3>ITA</s3>
<sZ>5 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Crosti, C" sort="Crosti, C" uniqKey="Crosti C" first="C." last="Crosti">C. Crosti</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">INIST</idno>
<idno type="inist">98-0488381</idno>
<date when="1998">1998</date>
<idno type="stanalyst">PASCAL 98-0488381 INIST</idno>
<idno type="RBID">Pascal:98-0488381</idno>
<idno type="wicri:Area/PascalFrancis/Corpus">000A69</idno>
<idno type="wicri:Area/PascalFrancis/Curation">001039</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema</title>
<author><name sortKey="Cerri, A" sort="Cerri, A" uniqKey="Cerri A" first="A." last="Cerri">A. Cerri</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Gianni, C" sort="Gianni, C" uniqKey="Gianni C" first="C." last="Gianni">C. Gianni</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Corbellino, M" sort="Corbellino, M" uniqKey="Corbellino M" first="M." last="Corbellino">M. Corbellino</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Istituto di Malattie Infettive e Tropicali, Università di Milano</s1>
<s3>ITA</s3>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Pizzuto, M" sort="Pizzuto, M" uniqKey="Pizzuto M" first="M." last="Pizzuto">M. Pizzuto</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Istituto di Malattie Infettive e Tropicali, Università di Milano</s1>
<s3>ITA</s3>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Moneghini, L" sort="Moneghini, L" uniqKey="Moneghini L" first="L." last="Moneghini">L. Moneghini</name>
<affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano</s1>
<s3>ITA</s3>
<sZ>5 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
<author><name sortKey="Crosti, C" sort="Crosti, C" uniqKey="Crosti C" first="C." last="Crosti">C. Crosti</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</inist:fA14>
<country>Italie</country>
</affiliation>
</author>
</analytic>
<series><title level="j" type="main">EJD. European journal of dermatology</title>
<title level="j" type="abbreviated">EJD, Eur. j. dermatol.</title>
<idno type="ISSN">1167-1122</idno>
<imprint><date when="1998">1998</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt><title level="j" type="main">EJD. European journal of dermatology</title>
<title level="j" type="abbreviated">EJD, Eur. j. dermatol.</title>
<idno type="ISSN">1167-1122</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Case study</term>
<term>Complication</term>
<term>Congenital</term>
<term>Human</term>
<term>Lower limb</term>
<term>Lymphangiosarcoma</term>
<term>Lymphedema</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Lymphoedème</term>
<term>Congénital</term>
<term>Membre inférieur</term>
<term>Complication</term>
<term>Lymphangiosarcome</term>
<term>Etude cas</term>
<term>Homme</term>
</keywords>
<keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequently associated with post-mastectomy lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally arise in congenital hereditary lymphedema (Milroy's syndrome and Meige's syndrome) and non-hereditary lymphedema (congenital, praecox or forme tarde lymphedemas). We report a case of lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-hereditary lymphedema of the left genital region and homolateral lower limb. In addition, molecular analysis demonstrated the absence of Kaposi's sarcoma-associated Herpes virus (KSHV) DNA sequences in tumour lesions. To our knowledge, this is the first case of lymphangiosarcoma associated with congenital non-hereditary lymphedema confined to the pubic region. The literature concerning the cases of lymphangiosarcoma arising in congenital hereditary and non-hereditary lymphedema is reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphedema. In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients.</div>
</front>
</TEI>
<inist><standard h6="B"><pA><fA01 i1="01" i2="1"><s0>1167-1122</s0>
</fA01>
<fA03 i2="1"><s0>EJD, Eur. j. dermatol.</s0>
</fA03>
<fA05><s2>8</s2>
</fA05>
<fA06><s2>7</s2>
</fA06>
<fA08 i1="01" i2="1" l="ENG"><s1>Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema</s1>
</fA08>
<fA11 i1="01" i2="1"><s1>CERRI (A.)</s1>
</fA11>
<fA11 i1="02" i2="1"><s1>GIANNI (C.)</s1>
</fA11>
<fA11 i1="03" i2="1"><s1>CORBELLINO (M.)</s1>
</fA11>
<fA11 i1="04" i2="1"><s1>PIZZUTO (M.)</s1>
</fA11>
<fA11 i1="05" i2="1"><s1>MONEGHINI (L.)</s1>
</fA11>
<fA11 i1="06" i2="1"><s1>CROSTI (C.)</s1>
</fA11>
<fA14 i1="01"><s1>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8</s1>
<s2>20142 Milan</s2>
<s3>ITA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
</fA14>
<fA14 i1="02"><s1>Istituto di Malattie Infettive e Tropicali, Università di Milano</s1>
<s3>ITA</s3>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
</fA14>
<fA14 i1="03"><s1>Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano</s1>
<s3>ITA</s3>
<sZ>5 aut.</sZ>
</fA14>
<fA20><s1>511-514</s1>
</fA20>
<fA21><s1>1998</s1>
</fA21>
<fA23 i1="01"><s0>ENG</s0>
</fA23>
<fA43 i1="01"><s1>INIST</s1>
<s2>22499</s2>
<s5>354000071236160120</s5>
</fA43>
<fA44><s0>0000</s0>
<s1>© 1998 INIST-CNRS. All rights reserved.</s1>
</fA44>
<fA45><s0>26 ref.</s0>
</fA45>
<fA47 i1="01" i2="1"><s0>98-0488381</s0>
</fA47>
<fA60><s1>P</s1>
</fA60>
<fA61><s0>A</s0>
</fA61>
<fA64 i2="1"><s0>EJD. European journal of dermatology</s0>
</fA64>
<fA66 i1="01"><s0>FRA</s0>
</fA66>
<fC01 i1="01" l="ENG"><s0>Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequently associated with post-mastectomy lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally arise in congenital hereditary lymphedema (Milroy's syndrome and Meige's syndrome) and non-hereditary lymphedema (congenital, praecox or forme tarde lymphedemas). We report a case of lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-hereditary lymphedema of the left genital region and homolateral lower limb. In addition, molecular analysis demonstrated the absence of Kaposi's sarcoma-associated Herpes virus (KSHV) DNA sequences in tumour lesions. To our knowledge, this is the first case of lymphangiosarcoma associated with congenital non-hereditary lymphedema confined to the pubic region. The literature concerning the cases of lymphangiosarcoma arising in congenital hereditary and non-hereditary lymphedema is reviewed. Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphedema. In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients.</s0>
</fC01>
<fC02 i1="01" i2="X"><s0>002B08A</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE"><s0>Lymphoedème</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="ENG"><s0>Lymphedema</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="SPA"><s0>Linfedema</s0>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE"><s0>Congénital</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG"><s0>Congenital</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA"><s0>Congénito</s0>
<s5>02</s5>
</fC03>
<fC03 i1="03" i2="X" l="FRE"><s0>Membre inférieur</s0>
<s5>03</s5>
</fC03>
<fC03 i1="03" i2="X" l="ENG"><s0>Lower limb</s0>
<s5>03</s5>
</fC03>
<fC03 i1="03" i2="X" l="SPA"><s0>Miembro inferior</s0>
<s5>03</s5>
</fC03>
<fC03 i1="04" i2="X" l="FRE"><s0>Complication</s0>
<s5>04</s5>
</fC03>
<fC03 i1="04" i2="X" l="ENG"><s0>Complication</s0>
<s5>04</s5>
</fC03>
<fC03 i1="04" i2="X" l="SPA"><s0>Complicación</s0>
<s5>04</s5>
</fC03>
<fC03 i1="05" i2="X" l="FRE"><s0>Lymphangiosarcome</s0>
<s5>07</s5>
</fC03>
<fC03 i1="05" i2="X" l="ENG"><s0>Lymphangiosarcoma</s0>
<s5>07</s5>
</fC03>
<fC03 i1="05" i2="X" l="SPA"><s0>Linfangiosarcoma</s0>
<s5>07</s5>
</fC03>
<fC03 i1="06" i2="X" l="FRE"><s0>Etude cas</s0>
<s5>08</s5>
</fC03>
<fC03 i1="06" i2="X" l="ENG"><s0>Case study</s0>
<s5>08</s5>
</fC03>
<fC03 i1="06" i2="X" l="SPA"><s0>Estudio caso</s0>
<s5>08</s5>
</fC03>
<fC03 i1="07" i2="X" l="FRE"><s0>Homme</s0>
<s5>09</s5>
</fC03>
<fC03 i1="07" i2="X" l="ENG"><s0>Human</s0>
<s5>09</s5>
</fC03>
<fC03 i1="07" i2="X" l="SPA"><s0>Hombre</s0>
<s5>09</s5>
</fC03>
<fC07 i1="01" i2="X" l="FRE"><s0>Appareil circulatoire pathologie</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="ENG"><s0>Cardiovascular disease</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="SPA"><s0>Aparato circulatorio patología</s0>
<s5>37</s5>
</fC07>
<fC07 i1="02" i2="X" l="FRE"><s0>Lymphatique pathologie</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="ENG"><s0>Lymphatic vessel disease</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA"><s0>Linfático patología</s0>
<s5>38</s5>
</fC07>
<fC07 i1="03" i2="X" l="FRE"><s0>Peau pathologie</s0>
<s5>40</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG"><s0>Skin disease</s0>
<s5>40</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA"><s0>Piel patología</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE"><s0>Tumeur maligne</s0>
<s5>55</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG"><s0>Malignant tumor</s0>
<s5>55</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA"><s0>Tumor maligno</s0>
<s5>55</s5>
</fC07>
<fN21><s1>320</s1>
</fN21>
</pA>
</standard>
</inist>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Sante/explor/LymphedemaV1/Data/PascalFrancis/Curation
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 001039 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/PascalFrancis/Curation/biblio.hfd -nk 001039 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Sante |area= LymphedemaV1 |flux= PascalFrancis |étape= Curation |type= RBID |clé= Pascal:98-0488381 |texte= Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema }}
![]() | This area was generated with Dilib version V0.6.31. | ![]() |