Chest
Volume 117, Issue 1, January 2000, Pages 25-30
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Clinical Investigations
PULMONARY VASCULATURE
Frequent Estrogen and Progesterone Receptor Immunoreactivity in Renal Angiomyolipomas From Women With Pulmonary Lymphangioleiomyomatosis

https://doi.org/10.1378/chest.117.1.25Get rights and content

Objective

To determine whether renal angiomyolipomasfrom women with pulmonary lymphangioleiomyomatosis (LAM) expressestrogen receptor (ER) and progesterone receptor (PR).

Design

Retrospective study of archival tissue.

Patients

Twelve women with LAM and angiomyolipomas.

Setting

Fox Chase Cancer Center.

Interventions

ER and PR expression was studied usingimmunohistochemistry. The hormonal status of the patients at the timeof resection of the angiomyolipoma was determined.

Results

Ten of the angiomyolipomas had ER immunoreactivity(83%), and all 12 had PR immunoreactivity (100%). The ER and PRpositivity was in the smooth muscle component of the angiomyolipomasonly. For five women, pulmonary LAM specimens were also available; twowere ER positive (40%), and all five were PR positive (100%). Allfour angiomyolipomas from women receiving progesterone therapy were ERand PR positive. One tumor from a woman receiving tamoxifen was ERnegative and strongly PR positive. One woman was pregnant; her tumorwas ER and PR positive.

Conclusions

ER and PRexpression is frequent in renal angiomyolipoma cells from women withLAM. PR was more consistently present than ER in angiomyolipomas and inLAM. Our data suggest that angiomyolipoma growth could be affected byhormonal factors. If the growth of LAM-associated angiomyolipomas slowsduring hormonal therapy, there are two potential implications for LAMpatients: first, angiomyolipoma size could serve as a measurableindication of response to hormonal therapy; and second, surgicalremoval of angiomyolipomas might be avoided in somecases.

Section snippets

Patients

This study was approved by the Institutional Review Board of Fox Chase Cancer Center.

None of the 12 women in this study had any clinical signs or symptoms of TSC, or a family history of either TSC or LAM. Ten of the patients had brain CT or MRI scans to exclude TSC, all 12 had dermatologic examinations, and 6 had ophthalmologic examinations. The degree to which individual patients were screened for TSC has been previously reported,13 with the exception of patient 482. Patient 482 had

Results

As shown in Table 1, all 12 of the angiomyolipomas were PR positive (100%) and 10 were ER positive (83%). The degree of positivity for ER, relative to the breast cancer specimen used as a positive control, was moderate in four cases (33%), weak in six cases (50%), and absent in two cases (17%). The PR positivity was strong in five cases (42%), moderate in six cases (50%), and weak in one case (8%). The ER positivity was seen in between 0% and 50% of the nuclei. The PR positivity was seen in 20

Discussion

LAM occurs almost exclusively in women of childbearing age. The underlying reasons for this are not known. A better understanding of the hormone receptor status in LAM could contribute toward the rational selection of appropriate hormonal therapy.4 Currently, many women with LAM are treated with high-dose progesterone, oophorectomy, or tamoxifen. The clinical efficacy of these therapies has never been assessed in a controlled study.

In this study, we examined ER and PR expression in

ACKNOWLEDGMENTS

We are grateful to Dr. Alfred Knudson, Dr. Frank McCormack, Ms. Sue Byrnes, and the LAM Foundation for their ongoing support; to William A. Petri and Drs. Ann Petri, Rebecca Raftogianis, and Leena Khare for critical review of the manuscript; and to Ms. Catherine Thompson for expert secretarial assistance.

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    This work was supported by the LAM Foundation (Cincinnati, OH) and the National Institutes of Health (HL60746).

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