Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications.
Identifieur interne : 00B212 ( Ncbi/Checkpoint ); précédent : 00B211; suivant : 00B213Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications.
Auteurs : H C Andersson [États-Unis] ; D M Parry ; J J MulvihillSource :
- American journal of medical genetics [ 0148-7299 ] ; 1995.
Descripteurs français
- KwdFr :
- MESH :
English descriptors
- KwdEn :
- Adult, Aged, Fatal Outcome, Female, Genes, Dominant, Humans, Lymphangiosarcoma (complications), Lymphangiosarcoma (genetics), 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 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.
DOI: 10.1002/ajmg.1320560116
PubMed: 7747790
Affiliations:
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nosological implications.</title><author><name sortKey="Andersson, H C" sort="Andersson, H C" uniqKey="Andersson H" first="H C" last="Andersson">H C Andersson</name><affiliation wicri:level="2"><nlm:affiliation>Interinstitute Medical Genetics Program, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Interinstitute Medical Genetics Program, National Cancer Institute, National Institutes of Health, Bethesda, Maryland</wicri:regionArea><placeName><region type="state">Maryland</region></placeName></affiliation></author><author><name sortKey="Parry, D M" sort="Parry, D M" uniqKey="Parry D" first="D M" last="Parry">D M Parry</name></author><author><name sortKey="Mulvihill, J J" sort="Mulvihill, J J" uniqKey="Mulvihill J" first="J J" last="Mulvihill">J J Mulvihill</name></author></analytic><series><title level="j">American journal of medical genetics</title><idno type="ISSN">0148-7299</idno><imprint><date when="1995" type="published">1995</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Fatal Outcome</term><term>Female</term><term>Genes, Dominant</term><term>Humans</term><term>Lymphangiosarcoma (complications)</term><term>Lymphangiosarcoma (genetics)</term><term>Lymphedema (complications)</term><term>Lymphedema (genetics)</term><term>Male</term><term>Middle Aged</term><term>Neoplastic Syndromes, Hereditary (complications)</term><term>Neoplastic Syndromes, Hereditary (genetics)</term><term>Pedigree</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Adulte d'âge moyen</term><term>Femelle</term><term>Gènes dominants</term><term>Humains</term><term>Issue fatale</term><term>Lymphangiosarcome ()</term><term>Lymphangiosarcome (génétique)</term><term>Lymphoedème ()</term><term>Lymphoedème (génétique)</term><term>Mâle</term><term>Pedigree</term><term>Sujet âgé</term><term>Syndromes néoplasiques héréditaires ()</term><term>Syndromes néoplasiques héréditaires (génétique)</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lymphangiosarcoma</term><term>Lymphedema</term><term>Neoplastic Syndromes, Hereditary</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Lymphangiosarcoma</term><term>Lymphedema</term><term>Neoplastic Syndromes, Hereditary</term></keywords><keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Lymphangiosarcome</term><term>Lymphoedème</term><term>Syndromes néoplasiques héréditaires</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Fatal Outcome</term><term>Female</term><term>Genes, Dominant</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Pedigree</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Adulte d'âge moyen</term><term>Femelle</term><term>Gènes dominants</term><term>Humains</term><term>Issue fatale</term><term>Lymphangiosarcome</term><term>Lymphoedème</term><term>Mâle</term><term>Pedigree</term><term>Sujet âgé</term><term>Syndromes néoplasiques héréditaires</term></keywords></textClass></profileDesc></teiHeader><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 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.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Maryland</li></region></list><tree><noCountry><name sortKey="Mulvihill, J J" sort="Mulvihill, J J" uniqKey="Mulvihill J" first="J J" last="Mulvihill">J J Mulvihill</name><name sortKey="Parry, D M" sort="Parry, D M" uniqKey="Parry D" first="D M" last="Parry">D M Parry</name></noCountry><country name="États-Unis"><region name="Maryland"><name sortKey="Andersson, H C" sort="Andersson, H C" uniqKey="Andersson H" first="H C" last="Andersson">H C Andersson</name></region></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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