LymphedemaV1, Main, Exploration, bibRecordById, pubmed:7747790

Lymphangiosarcoma in late‐onset hereditary lymphedema: Case report and nosological implications

Identifieur interne : 00C576 ( Main/Exploration ); précédent : 00C575; suivant : 00C577

Lymphangiosarcoma in late‐onset hereditary lymphedema: Case report and nosological implications

Auteurs : Hans C. Andersson [États-Unis] ; Dilys M. Parry [États-Unis] ; John J. Mulvihill [États-Unis]

Source :

American Journal of Medical Genetics [ 0148-7299 ] ; 1995-03-13.

RBID : ISTEX:E37491AAD9ABF5D1C45CEAFC2F19FF76F24E58C6

Descripteurs français

KwdFr :
- Adulte, Adulte d'âge moyen, Femelle, Gènes dominants, Humains, Issue fatale, Lymphangiosarcome (), Lymphangiosarcome (génétique), Lymphoedème (), Lymphoedème (génétique), Mâle, Pedigree, Sujet âgé, Syndromes néoplasiques héréditaires (), Syndromes néoplasiques héréditaires (génétique).
MESH :
- génétique : Lymphangiosarcome, Lymphoedème, Syndromes néoplasiques héréditaires.
- Adulte, Adulte d'âge moyen, Femelle, Gènes dominants, Humains, Issue fatale, Lymphangiosarcome, Lymphoedème, Mâle, Pedigree, Sujet âgé, Syndromes néoplasiques héréditaires.
Pascal (Inist)
- Complication, Etude cas, Etude familiale, Homme, Lymphangiosarcome, Lymphoedème.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Adult, Aged, Case study, Complication, Family study, Fatal Outcome, Female, Genes, Dominant, Human, Humans, Lymphangiosarcoma, Lymphangiosarcoma (complications), Lymphangiosarcoma (genetics), Lymphedema, Lymphedema (complications), Lymphedema (genetics), Male, Middle Aged, Neoplastic Syndromes, Hereditary (complications), Neoplastic Syndromes, Hereditary (genetics), Pedigree.
MESH :
- complications : Lymphangiosarcoma, Lymphedema, Neoplastic Syndromes, Hereditary.
- genetics : Lymphangiosarcoma, Lymphedema, Neoplastic Syndromes, Hereditary.
- Adult, Aged, Fatal Outcome, Female, Genes, Dominant, Humans, Male, Middle Aged, Pedigree.

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 lym‐phedema in their mid‐twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post‐mastectomy lym‐phedema, 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. © 1995 Wiley‐Liss, Inc.

Url:

https://api.istex.fr/document/E37491AAD9ABF5D1C45CEAFC2F19FF76F24E58C6/fulltext/pdf

DOI: 10.1002/ajmg.1320560116

Affiliations:

Le document en format XML

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<front><div type="abstract" xml:lang="en">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 lym‐phedema in their mid‐twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post‐mastectomy lym‐phedema, 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. © 1995 Wiley‐Liss, Inc.</div>
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Serveur d'exploration sur le lymphœdème

Lymphangiosarcoma in late‐onset hereditary lymphedema: Case report and nosological implications

Lymphangiosarcoma in late‐onset hereditary lymphedema: Case report and nosological implications

Source :

Descripteurs français

English descriptors

Abstract

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Pour manipuler ce document sous Unix (Dilib)

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