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Medline ® Abstract for Reference 103

of 'Clinical staging and conservative management of peripheral lymphedema'

Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications.
Andersson HC, Parry DM, Mulvihill JJ
Am J Med Genet. 1995;56(1):72.
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.
Interinstitute Medical Genetics Program, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA.