Malignant presentation of uterine lymphangioleiomyomatosis

The main aim of this case report was to present the method of diagnosis, management, and the 12-year-follow-up of a patient diagnosed with primary uterine lymphangioleiomyomatosis (LAM).

A 47-year-old woman was admitted to the Department of Thoracosurgery due to pulmonary lesions suspected to be metastatic. The potential primary site of the neoplasm was not identified by previous imaging studies and specialist counseling. The patient had a history of total abdominal hysterectomy without ovaries due to a uterine tumor recognized as cellular leiomyoma and left salpingo-oophorectomy due to a solid ovarian tumor also recognized as leiomyoma. She had previously undergone the removal of a left kidney angiomyolipoma. After histopathological examination of the pulmonary lesions and repeated evaluation of the ovarian and uterine tumors, the diagnosis of primary uterine LAM with metastases to the ovary and the lungs was established. Although new metastatic lesions occurred, the patient remained in good condition during the 12-year-follow-up.

The history of our patient and review of the literature suggest that although uterine LAM presents malignant features (i.e., metastasis), the disease is long lasting and the patient can be in good condition for a number of years.