Chest
Clinical InvestigationsPULMONARY VASCULATUREFrequent Estrogen and Progesterone Receptor Immunoreactivity in Renal Angiomyolipomas From Women With Pulmonary Lymphangioleiomyomatosis
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).