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Clear-Cell Adenocarcinoma
D efinition —An uncommon primary adenocarcinoma of the vagina, associated with in utero exposure to synthetic estrogen.
Clinical Features
Epidemiology
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Rare, except in women who were exposed to diethylstilbestrol (DES) in utero.
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The risk in DES-exposed women is estimated to be between 1 : 1000 and 1 : 5000 individuals.
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The peak age at diagnosis for DES-exposed women is 20, and it is rarely seen in women over 40.
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In non–DES-exposed women, the age at presentation is usually in the seventh or eighth decade of life; however, the age distribution seems to be bimodal, with a smaller group presenting around the time of menarche.
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