Successful Treatment of Postmenopausal Exacerbation of Abdominal Lymphangioleiomyomatosis With Sirolimus: A Report of a Rare Case.

Lymphangioleiomyomatosis (LAM) is a rare disease that primarily affects women of childbearing age and often stabilizes after menopause. We report an unusual case of LAM progression in a 70-year-old postmenopausal woman. Diagnosed with LAM at age 53, the patient experienced progression of abdominal lesions 17 years post-diagnosis, despite stable pulmonary function. Notably, she was receiving aromatase inhibitors for breast cancer. Abdominal CT and MRI revealed enlarging nodules, while serum vascular endothelial growth factor-D (VEGF-D) levels were elevated. Treatment with sirolimus (2 mg/day, later adjusted) led to significant improvement in symptoms and a reduction in abdominal nodules. This case highlights the potential for LAM progression in postmenopausal women, primarily manifesting as abdominal lesions. It underscores the importance of long-term follow-up in LAM patients, regardless of menopausal status, and demonstrates the efficacy of sirolimus in managing postmenopausal LAM progression. Further research is needed to understand the mechanisms driving LAM activity in the absence of high estrogen levels and to optimize treatment strategies for this patient population.