We reviewed the clinical, pathological and immunohistochemical feature
s of six unilocular cystic granulosa cell tumors (UCGs) of the ovary.
The mean age of the patients was 46 years and the presenting features
included abdominal distension, pain, urinary frequency, dyspareunia an
d amenorrhea. The tumors were unilateral, thin walled and consisted of
a single large cavity with a smooth internal lining. The mean tumor-d
iameter was 10 cm and in all instances the UCGs were of adult histolog
ical type. The granulosa cells in six tumors demonstrated immunoreacti
vity for vimentin, five neoplasms showed reactivity for both alpha-inh
ibin and progesterone-receptor protein and four showed reactivity for
smooth muscle actin. All tumors were nonimmunoreactive with antibodies
to epithelial membrane antigen, beta-inhibin, estrogen-receptor prote
in, testosterone and s100 antigen. Only one of the three patients test
ed pre-operatively had an elevated serum inhibin concentration and thi
s returned to normal six weeks post-operatively. We conclude that UCGs
are distinguished by the infrequency of diagnostic serum tumor marker
s and clinically evident endocrine activity. Correct diagnosis is depe
ndent on histological examination and the finding of a monotonous popu
lation of cells, a trabecular growth pattern, nuclear grooves, low mit
otic activity and an immunohistochemical profile compatible with granu
losa cell tumors.