Lymphangiosarcoma in late-onset hereditary lymphedema: Case report and nosological implications |
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Authors: | Hans C. Andersson Dilys M. Parry John J. Mulvihill |
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Affiliation: | Department of Psychiatry, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania |
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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. |
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Keywords: | hereditary lymphedema lymphangiosarcoma Meige disease |
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