The incidence of ovarian atypical endometriosis and its association wi
th malignant epithelial tumours in a consecutive series of cases durin
g the period 1987 to 1995 were studied. Atypical glandular changes wer
e observed in four (1.7%) of 255 ovarian endometriosis cases and one p
atient with ovarian atypical endometriosis developed subsequent endome
trioid carcinoma in the abdominal wall. Fifty-four (24.1%) of the 224
ovarian cancers were associated with ovarian endometriosis; 21 with ty
pical and 33 with atypical endometriosis. Clear cell carcinomas and en
dometrioid carcinomas were most frequently associated with endometrios
is, with 54% (27 of 50 cases) and 41.9% (13 of 31), respectively. Atyp
ical endometriosis was found in 18 clear cell carcinomas, in seven end
ometrioid carcinomas, in four serous carcinomas, in three mucinous bor
derline rumours, and in one serous borderline tumour. In 13 cases, the
atypical endometriosis was in contiguity with malignant epithelial ru
mours. We consider that atypical endometriosis possesses a precancerou
s potential or is most frequently associated with clear cell and endom
etrioid carcinomas. Close screening of cellular atypia or hyperplasia
in ovarian endometriosis and careful long-term follow-up of patients w
ith atypical endometriosis is required.