Ep. Henske et al., FREQUENT PROGESTERONE-RECEPTOR IMMUNOREACTIVITY IN TUBEROUS SCLEROSIS-ASSOCIATED RENAL ANGIOMYOLIPOMAS, Modern pathology, 11(7), 1998, pp. 665-668
Angiomyolipomas can occur sporadically or in association with tuberous
sclerosis complex (TSC). TSC is an autosomal dominant disorder charac
terized by seizures, mental retardation, and benign tumors of the brai
n, heart, kidney, and skin. Angiomyolipomas are more common in women t
han in men, suggesting a possible hormonal influence on tumor growth.
In this study, 35 angiomyolipomas from 23 patients were immunostained
with antibodies to estrogen receptor (ER) and progesterone receptor (P
R). Eleven angiomyolipomas (31%) contained clusters of PR-immunoreacti
ve smooth muscle cells. None contained ER-immunoreactive cells. Of the
21 tumors from patients with TSC, 11 (48%) were PR immunoreactive. Al
l of the PR-immunoreactive angiomyolipomas were from women younger tha
n 50 years of age, and all except one of these women had TSC. This stu
dy suggests that hormonal factors play a role in the pathogenesis of T
SC-associated angiomyolipomas.