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Lymphangiomyomatosis: A report of three cases treated with tamoxifen

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Summary

Three cases are reported of lymphangiomyomatosis with pulmonary and abdominal manifestations. Two had a chylous pleura effusion, while the third presented a retroperitoneal manifestation, which was completely resectable. Antiestrogen therapy with tamoxifen was administered in all three cases. Two patients died of pulmonary progression after 4 months of therapy. The third is still alive, with stable disease for more than 6 years, and has been receiving tamoxifen for 66 months. These observations indicate that antiestrogen treatment may be as effective as oophorectomy when started at an early stage of the disease.

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Clemm, C., Jehn, U., Wolf-Hornung, B. et al. Lymphangiomyomatosis: A report of three cases treated with tamoxifen. Klin Wochenschr 65, 391–393 (1987). https://doi.org/10.1007/BF01745582

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  • DOI: https://doi.org/10.1007/BF01745582

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