leuprolide and Lymphangioleiomyomatosis

Topics: Antineoplastic Agents, Hormonal; Bronchodilator Agents; Chylothorax; Gonadotropin-Releasing Hormone; Goserelin; Humans; Leuprolide; Lung Neoplasms; Lung Transplantation; Lymphangioleiomyomatosis; Ovariectomy; Pneumothorax; Progesterone; Respiratory Insufficiency

Lymphangiomyomatosis (LAM) is a rare and systemic disease that is characterized by the abnormal proliferation of smooth muscle-like cells in the lungs and along the axial lymphatic system. The authors herein present a rare case of LAM that was treated with long-term use of leuprolide acetate, a gonadotropin-releasing hormone analogue (GnRHa).

Topics: Adult; Female; Follow-Up Studies; Humans; Leuprolide; Lymphangioleiomyomatosis

Benign metastasizing leiomyoma and lymphangioleiomyomatosis (LAM) both are characterized by abnormal proliferation of smooth muscle-like cells in the lung.. A 32-year-old African woman with a diagnosis of LAM underwent myomectomy for uterine leiomyomas. An alternative diagnosis of benign metastasizing leiomyoma was made on repeat lung biopsy. Treatment with leuprolide acetate decreased pulmonary infiltrates and improved lung function and exercise tolerance.. Accurately diagnosing benign metastasizing leiomyoma has important implications for clinical outcome. Because its clinical presentation may be misleading, immunohistochemical techniques may assist in differentiating benign metastasizing leiomyoma from LAM. This is important because, in benign metastasizing leiomyoma, reduced tumor burden and improved pulmonary function may be achieved by suppressing gonadal steroids.

Topics: Adult; Antineoplastic Agents, Hormonal; Diagnosis, Differential; Female; Humans; Leiomyoma; Leuprolide; Lung Neoplasms; Lymphangioleiomyomatosis; Uterine Neoplasms

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown aetiology which mainly affects women of reproductive age. A growing body of evidence suggests a relation between the disease and estrogenic hormones. In particular, pregnancy and use of estrogens favour the progression of the disease, which is in turn slowed down by menopause. These observations suggested already in the past a treatment strategy based on hormonal manipulation: discontinuation of estrogens containing medications, avoiding pregnancy, inhibition of estrogens activity and induction of menopause.. In the present study, conducted on a relatively large population for a rare disease, we sought to show the hormonal manipulation's positive effects for the survival rate.. 36 women suffering from LAM were evaluated between January 1985 and March 2005. All our patients were treated with hormonal therapy, following different schemes. The response to the treatment was evaluated in all patients with arterial blood sampling, lung function tests (total body plethysmography, DLCO), measurement of the hormones in the blood, chest and abdomen CT scan.. The survival rate since the clinical onset was 97% at the 5 year timepoint, 90% at 10 years, and 71% at 25 years. Previous studies, conducted before the introduction of the hormonal treatment showed a survival rate of 20% at 10 years.. Our results highlight the role of the hormonal manipulation as a mainstay in the treatment of LAM, with the capability of reducing mortality and improving the quality of life as well.

Topics: Adult; Estrogen Antagonists; Estrogens; Female; Hormones; Humans; Leuprolide; Luteolytic Agents; Lymphangioleiomyomatosis; Medroxyprogesterone Acetate; Menopause; Middle Aged; Ovariectomy; Respiratory Function Tests; Triptorelin Pamoate

We describe a case of pulmonary lymphangiomyomatosis (LAM) with chylothorax that developed in a 46-year-old Japanese woman. This patient exhibited clinical symptoms of dyspnea and chest X-ray showed right pleural effusion. Thoracocentesis demonstrated chylous effusion. Chest computed tomography (CT) scan revealed multiple cystic lesions. Subsequent thoracoscopy revealed the chylorrhea from swelled vessels on the diaphragm. The clinical diagnosis, based on histological examinations with biopsy specimens obtained by thoracoscopy, was pulmonary LAM. Although the hormone therapy was not effective, chylous effusion was improved by the pleurodesis. Pulmonary LAM developing chylothorax is rare in Japan.

Topics: Chylothorax; Drug Therapy, Combination; Female; Humans; Leuprolide; Lung Diseases; Lymphangioleiomyomatosis; Middle Aged; Pleurodesis; Progesterone; Thoracoscopy; Tomography, X-Ray Computed