Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications
Identifieur interne : 000C65 ( PascalFrancis/Corpus ); précédent : 000C64; suivant : 000C66Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications
Auteurs : H. C. Andersson ; D. M. Parry ; J. J. MulvihillSource :
- American journal of medical genetics [ 0148-7299 ] ; 1995.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
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
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NO : | PASCAL 95-0223766 INIST |
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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:95-0223766Le 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 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>
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<ET>Lymphangiosarcoma in late-onset hereditary lymphedema : case report and nosological implications</ET>
<AU>ANDERSSON (H. C.); PARRY (D. M.); MULVIHILL (J. J.)</AU>
<AF>NIH, national cancer inst., interinst. medical program/Bethesda MD/Etats-Unis</AF>
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