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Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications

Identifieur interne : 000C65 ( PascalFrancis/Corpus ); précédent : 000C64; suivant : 000C66

Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications

Auteurs : H. C. Andersson ; D. M. Parry ; J. J. Mulvihill

Source :

RBID : Pascal:95-0223766

Descripteurs français

English descriptors

Abstract

Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease). We describe a family in which three individuals in three generations had unusually late onset of lymphedema in their mid-twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post-mastectomy lymphedema, has not been described in late-onset hereditary lymphedema. Because of an unusually high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (approximately 10%) and the proband's own familial cancer background, we speculate that an inherited predisposition to malignancy may underlie the development of lymphedema-associated lymphangiosarcoma

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

pA  
A01 01  1    @0 0148-7299
A02 01      @0 AJMGDA
A03   1    @0 Am. j. med. genet.
A05       @2 56
A06       @2 1
A08 01  1  ENG  @1 Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications
A11 01  1    @1 ANDERSSON (H. C.)
A11 02  1    @1 PARRY (D. M.)
A11 03  1    @1 MULVIHILL (J. J.)
A14 01      @1 NIH, national cancer inst., interinst. medical program @2 Bethesda MD @3 USA
A20       @1 72-75
A21       @1 1995
A23 01      @0 ENG
A43 01      @1 INIST @2 17405 @5 354000055621870150
A44       @0 0000
A45       @0 19 ref.
A47 01  1    @0 95-0223766
A60       @1 P
A61       @0 A
A64 01  1    @0 American journal of medical genetics
A66 01      @0 USA
C01 01    ENG  @0 Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease). We describe a family in which three individuals in three generations had unusually late onset of lymphedema in their mid-twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post-mastectomy lymphedema, has not been described in late-onset hereditary lymphedema. Because of an unusually high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (approximately 10%) and the proband's own familial cancer background, we speculate that an inherited predisposition to malignancy may underlie the development of lymphedema-associated lymphangiosarcoma
C02 01  X    @0 002B12B03
C03 01  X  FRE  @0 Lymphangiosarcome @5 01
C03 01  X  ENG  @0 Lymphangiosarcoma @5 01
C03 01  X  SPA  @0 Linfangiosarcoma @5 01
C03 02  X  FRE  @0 Complication @5 02
C03 02  X  ENG  @0 Complication @5 02
C03 02  X  SPA  @0 Complicación @5 02
C03 03  X  FRE  @0 Homme @5 03
C03 03  X  ENG  @0 Human @5 03
C03 03  X  SPA  @0 Hombre @5 03
C03 04  X  FRE  @0 Lymphoedème @5 04
C03 04  X  ENG  @0 Lymphedema @5 04
C03 04  X  SPA  @0 Linfedema @5 04
C03 05  X  FRE  @0 Etude familiale @5 05
C03 05  X  ENG  @0 Family study @5 05
C03 05  X  SPA  @0 Estudio familiar @5 05
C03 06  X  FRE  @0 Etude cas @5 21
C03 06  X  ENG  @0 Case study @5 21
C03 06  X  SPA  @0 Estudio caso @5 21
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 Tumeur maligne @5 39
C07 03  X  ENG  @0 Malignant tumor @5 39
C07 03  X  SPA  @0 Tumor maligno @5 39
N21       @1 130

Format Inist (serveur)

NO : PASCAL 95-0223766 INIST
ET : Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications
AU : ANDERSSON (H. C.); PARRY (D. M.); MULVIHILL (J. J.)
AF : NIH, national cancer inst., interinst. medical program/Bethesda MD/Etats-Unis
DT : Publication en série; Niveau analytique
SO : American journal of medical genetics; ISSN 0148-7299; Coden AJMGDA; Etats-Unis; Da. 1995; Vol. 56; No. 1; Pp. 72-75; Bibl. 19 ref.
LA : Anglais
EA : Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease). We describe a family in which three individuals in three generations had unusually late onset of lymphedema in their mid-twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post-mastectomy lymphedema, has not been described in late-onset hereditary lymphedema. Because of an unusually high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (approximately 10%) and the proband's own familial cancer background, we speculate that an inherited predisposition to malignancy may underlie the development of lymphedema-associated lymphangiosarcoma
CC : 002B12B03
FD : Lymphangiosarcome; Complication; Homme; Lymphoedème; Etude familiale; Etude cas
FG : Appareil circulatoire pathologie; Lymphatique pathologie; Tumeur maligne
ED : Lymphangiosarcoma; Complication; Human; Lymphedema; Family study; Case study
EG : Cardiovascular disease; Lymphatic vessel disease; Malignant tumor
SD : Linfangiosarcoma; Complicación; Hombre; Linfedema; Estudio familiar; Estudio caso
LO : INIST-17405.354000055621870150
ID : 95-0223766

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Pascal:95-0223766

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