LymphedemaV1, PascalFrancis, Corpus, bibRecord, 000A69

Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema

Identifieur interne : 000A69 ( PascalFrancis/Corpus ); précédent : 000A68; suivant : 000A70

Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema

Auteurs : A. Cerri ; C. Gianni ; M. Corbellino ; M. Pizzuto ; L. Moneghini ; C. Crosti

Source :

EJD. European journal of dermatology [ 1167-1122 ] ; 1998.

RBID : Pascal:98-0488381

Descripteurs français

Pascal (Inist)
- Lymphoedème, Congénital, Membre inférieur, Complication, Lymphangiosarcome, Etude cas, Homme.

English descriptors

KwdEn :
- Case study, Complication, Congenital, Human, Lower limb, Lymphangiosarcoma, Lymphedema.

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.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

A01	`01`	`1`		`@0 1167-1122`
A03		`1`		`@0 EJD, Eur. j. dermatol.`
A05				`@2 8`
A06				`@2 7`
A08	`01`	`1`	`ENG`	`@1 Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema`
A11	`01`	`1`		`@1 CERRI (A.)`
A11	`02`	`1`		`@1 GIANNI (C.)`
A11	`03`	`1`		`@1 CORBELLINO (M.)`
A11	`04`	`1`		`@1 PIZZUTO (M.)`
A11	`05`	`1`		`@1 MONEGHINI (L.)`
A11	`06`	`1`		`@1 CROSTI (C.)`
A14	`01`			`@1 Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8 @2 20142 Milan @3 ITA @Z 1 aut. @Z 2 aut. @Z 6 aut.`
A14	`02`			`@1 Istituto di Malattie Infettive e Tropicali, Università di Milano @3 ITA @Z 3 aut. @Z 4 aut.`
A14	`03`			`@1 Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano @3 ITA @Z 5 aut.`
A20				`@1 511-514`
A21				`@1 1998`
A23	`01`			`@0 ENG`
A43	`01`			`@1 INIST @2 22499 @5 354000071236160120`
A44				`@0 0000 @1 © 1998 INIST-CNRS. All rights reserved.`
A45				`@0 26 ref.`
A47	`01`	`1`		`@0 98-0488381`
A60				`@1 P`
A61				`@0 A`
A64		`1`		`@0 EJD. European journal of dermatology`
A66	`01`			`@0 FRA`
C01	`01`		`ENG`	@0 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.
C02	`01`	`X`		`@0 002B08A`
C03	`01`	`X`	`FRE`	`@0 Lymphoedème @5 01`
C03	`01`	`X`	`ENG`	`@0 Lymphedema @5 01`
C03	`01`	`X`	`SPA`	`@0 Linfedema @5 01`
C03	`02`	`X`	`FRE`	`@0 Congénital @5 02`
C03	`02`	`X`	`ENG`	`@0 Congenital @5 02`
C03	`02`	`X`	`SPA`	`@0 Congénito @5 02`
C03	`03`	`X`	`FRE`	`@0 Membre inférieur @5 03`
C03	`03`	`X`	`ENG`	`@0 Lower limb @5 03`
C03	`03`	`X`	`SPA`	`@0 Miembro inferior @5 03`
C03	`04`	`X`	`FRE`	`@0 Complication @5 04`
C03	`04`	`X`	`ENG`	`@0 Complication @5 04`
C03	`04`	`X`	`SPA`	`@0 Complicación @5 04`
C03	`05`	`X`	`FRE`	`@0 Lymphangiosarcome @5 07`
C03	`05`	`X`	`ENG`	`@0 Lymphangiosarcoma @5 07`
C03	`05`	`X`	`SPA`	`@0 Linfangiosarcoma @5 07`
C03	`06`	`X`	`FRE`	`@0 Etude cas @5 08`
C03	`06`	`X`	`ENG`	`@0 Case study @5 08`
C03	`06`	`X`	`SPA`	`@0 Estudio caso @5 08`
C03	`07`	`X`	`FRE`	`@0 Homme @5 09`
C03	`07`	`X`	`ENG`	`@0 Human @5 09`
C03	`07`	`X`	`SPA`	`@0 Hombre @5 09`
C07	`01`	`X`	`FRE`	`@0 Appareil circulatoire pathologie @5 37`
C07	`01`	`X`	`ENG`	`@0 Cardiovascular disease @5 37`
C07	`01`	`X`	`SPA`	`@0 Aparato circulatorio patología @5 37`
C07	`02`	`X`	`FRE`	`@0 Lymphatique pathologie @5 38`
C07	`02`	`X`	`ENG`	`@0 Lymphatic vessel disease @5 38`
C07	`02`	`X`	`SPA`	`@0 Linfático patología @5 38`
C07	`03`	`X`	`FRE`	`@0 Peau pathologie @5 40`
C07	`03`	`X`	`ENG`	`@0 Skin disease @5 40`
C07	`03`	`X`	`SPA`	`@0 Piel patología @5 40`
C07	`04`	`X`	`FRE`	`@0 Tumeur maligne @5 55`
C07	`04`	`X`	`ENG`	`@0 Malignant tumor @5 55`
C07	`04`	`X`	`SPA`	`@0 Tumor maligno @5 55`
N21				`@1 320`

Format Inist (serveur)

NO :	PASCAL 98-0488381 INIST
ET :	Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema
AU :	CERRI (A.); GIANNI (C.); CORBELLINO (M.); PIZZUTO (M.); MONEGHINI (L.); CROSTI (C.)
AF :	Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8/20142 Milan/Italie (1 aut., 2 aut., 6 aut.); Istituto di Malattie Infettive e Tropicali, Università di Milano/Italie (3 aut., 4 aut.); Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano/Italie (5 aut.)
DT :	Publication en série; Niveau analytique
SO :	EJD. European journal of dermatology; ISSN 1167-1122; France; Da. 1998; Vol. 8; No. 7; Pp. 511-514; Bibl. 26 ref.
LA :	Anglais
EA :	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.
CC :	002B08A
FD :	Lymphoedème; Congénital; Membre inférieur; Complication; Lymphangiosarcome; Etude cas; Homme
FG :	Appareil circulatoire pathologie; Lymphatique pathologie; Peau pathologie; Tumeur maligne
ED :	Lymphedema; Congenital; Lower limb; Complication; Lymphangiosarcoma; Case study; Human
EG :	Cardiovascular disease; Lymphatic vessel disease; Skin disease; Malignant tumor
SD :	Linfedema; Congénito; Miembro inferior; Complicación; Linfangiosarcoma; Estudio caso; Hombre
LO :	INIST-22499.354000071236160120
ID :	98-0488381

Links to Exploration step

Pascal:98-0488381

Le document en format XML

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<s3>ITA</s3>
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<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>
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<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>6 aut.</sZ>
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<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
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<fA14 i1="03"><s1>Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano</s1>
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<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>
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</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>
<server><NO>PASCAL 98-0488381 INIST</NO>
<ET>Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema</ET>
<AU>CERRI (A.); GIANNI (C.); CORBELLINO (M.); PIZZUTO (M.); MONEGHINI (L.); CROSTI (C.)</AU>
<AF>Dermatologica IV, Istituto di Scienze Biomediche, Ospedale San Paolo, Università degli Studi di Milano, via A. di Rudini, 8/20142 Milan/Italie (1 aut., 2 aut., 6 aut.); Istituto di Malattie Infettive e Tropicali, Università di Milano/Italie (3 aut., 4 aut.); Clinica di Anatomia ed Istologia Patologica II, Ospedale San Paolo, Università di Milano/Italie (5 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>EJD. European journal of dermatology; ISSN 1167-1122; France; Da. 1998; Vol. 8; No. 7; Pp. 511-514; Bibl. 26 ref.</SO>
<LA>Anglais</LA>
<EA>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.</EA>
<CC>002B08A</CC>
<FD>Lymphoedème; Congénital; Membre inférieur; Complication; Lymphangiosarcome; Etude cas; Homme</FD>
<FG>Appareil circulatoire pathologie; Lymphatique pathologie; Peau pathologie; Tumeur maligne</FG>
<ED>Lymphedema; Congenital; Lower limb; Complication; Lymphangiosarcoma; Case study; Human</ED>
<EG>Cardiovascular disease; Lymphatic vessel disease; Skin disease; Malignant tumor</EG>
<SD>Linfedema; Congénito; Miembro inferior; Complicación; Linfangiosarcoma; Estudio caso; Hombre</SD>
<LO>INIST-22499.354000071236160120</LO>
<ID>98-0488381</ID>
</server>
</inist>
</record>

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Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema

Lymphangiosarcoma of the pubic region : a rare complication arising in congenital non-hereditary lymphedema

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