Lymphangiomyomatosis: A report of three cases treated with tamoxifen

Abstract

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.

Tuberous Sclerosis with Pulmonary Involvement

“…This is supported by results of radioligand binding tests which revealed estrogen and progester one cytosolic receptors in homogenates of involved pulmonary tissue [1,3,10,12,17,18,25]. Therefore, attempts of hormone-modulating treatment have been undertaken, starting with androgens [4], then with oophorectomy [2,15], followed by antiestrogen therapy with tamoxifen [7,12,17,28], or medroxypro gesterone [10,18,21 ] or both [28]. Besides this, a com bined treatment with oophorectomy and antiestrogen was tried [1,25].…”

“…We suggest that young women in the childbearing age with clinical signs of pTS or LAM should un dergo a histological examination of lung parenchyma [6,19], with regard to the opportunity for an early di agnosis and treatment of this disease [1,7,9,26]. This cannot be provided by lung function tests, X-ray or CT scan alone, which only allows the descriptive di agnosis of an interstitial lung disease.…”

Estradiol and tamoxifen stimulate LAM-associated angiomyolipoma cell growth and activate both genomic and nongenomic signaling pathways

“…Studies of cultured human angiomyolipoma cells could lead to the rational selection of hormonal therapies for patients with symptomatic or enlarging angiomyolipomas. Finally, there are also several reports of women with pulmonary LAM who died of progressive pulmonary disease within months after initiating tamoxifen therapy (12). Whether there is a link between the in vitro stimulation of angiomyolipoma cell growth by tamoxifen and these clinical outcomes is unknown.…”

Lung Transplantation for Lymphangioleiomyomatosis

“…4,5 The predominant occurrence of lymphangioleiomyomatosis in women during their reproductive years suggests hormonal involvement. Treatment is therefore targeted against the production and effects of estrogens [6][7][8][9][10][11] but remains largely ineffective. In recent years, lung transplantation has become a therapeutic approach.…”