{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T15:11:31Z","timestamp":1779894691481,"version":"3.53.1"},"reference-count":19,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2005,6,7]],"date-time":"2005-06-07T00:00:00Z","timestamp":1118102400000},"content-version":"vor","delay-in-days":3739,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1995,3,13]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hereditary lymphedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non\u2010syndromic 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\u2010phedema in their mid\u2010twenties or thirties. The proband additionally developed a very rare lymphangiosarcoma. This tumor, usually associated with post\u2010mastectomy lym\u2010phedema, has not been described in late\u2010onset 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\u2010associated lymphangiosarcoma. \u00a9 1995 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/ajmg.1320560116","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T17:58:53Z","timestamp":1118512733000},"page":"72-75","source":"Crossref","is-referenced-by-count":26,"title":["Lymphangiosarcoma in late\u2010onset hereditary lymphedema: Case report and nosological implications"],"prefix":"10.1002","volume":"56","author":[{"given":"Hans C.","family":"Andersson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilys M.","family":"Parry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"Mulvihill","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2005,6,7]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"320","article-title":"Lymphangiosarcoma in chronic hereditary oedema (Milroy's disease)","volume":"78","author":"Bostrum L\u2010A","year":"1989","journal-title":"Ann Chir Gynecol"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9610(60)90334-2"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(62)90069-4"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1177\/003591575404700308"},{"key":"e_1_2_1_6_1","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1001\/archinte.1983.00350050071014","article-title":"Hereditary late\u2010onset lymphedema with pleural effusion 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