A rare malignant Brenner tumor of the ovary presenting with hyperestro
genism in a 79 year old woman was examined immunohistochemically and b
y light and electron microscopy. High pre-operative serum and urinary
estrogen concentrations, low serum luteinizing hormone (LH) and follic
le-stimulating hormone (FSH) levels, and histologically confirmed atyp
ical endometrial hyperplasia suggested the presence of hyperestrogenis
m. The reduction in serum and urinary estrogen and the increase in ser
um LH and FSH concentrations after tumor removal confirmed that the tu
mor was synthesizing estrogen. Histologically, the malignant element w
as predominantly a squamous cell carcinoma, Transitional cell carcinom
a was partially found on the cyst wall, There was a spectrum of morpho
logic changes between benign and malignant elements with an intermedia
te area with a proliferating Brenner tumor. Immunohistochemically, onl
y the carcinoembryonic antigen was positive exclusively on the maligna
nt element as well as in the microcyst in the benign epithelial cord,
whereas all of the markers for germ cell tumors were negative. The ult
rastructural features of the stromal cells were of two types: fibrobla
sts and steroid-producing cells, The latter type of cells might corres
pond morphologically to estrogen-producing cells. The present case is
the fourth report showing a malignant Brenner tumor combined with appa
rent hyperestrogenism.