Pulmonary Leiomyomatosis: A Case Report [10L]

INTRODUCTION: Benign metastasizing leiomyomatosis (BML) is a rare disorder, first characterized by Steiner in 1939, which manifests with extrauterine leiomyomata affecting areas such as the lung and bone. The clinical course is generally indolent, but patients can present with pulmonary symptoms. METHODS: CD is a 54 yo G4P2022 who presented to the emergency department with a 7 day complaint of moderate to severe chest pain radiating to her shoulder, exacerbated with movements. Surgical history included total hysterectomy at age 34 for uterine leiomyomata. Initial cardiac work up was negative. CT scan revealed fifteen pulmonary nodules and an ovarian mass. Transvaginal ultrasound showed two unilocular ovarian cysts. Laboratory results included CA-125 = 20.4 and FSH/LH = 14.5/11.3. CT guided biopsy of a pulmonary nodule demonstrated features of leiomyoma. Following this biopsy, the patient underwent VATS resection of the largest pleural nodule. The case was reviewed at the multidisciplinary tumor board. The final diagnosis was pulmonary benign metastasizing leiomyomatosis (BML). The left ovarian mass was felt to represent a benign ovarian neoplasm unrelated to BML. The patient was encouraged to undergo bilateral oophorectomy to limit the growth of her pulmonary leiomyomata. She elected for observation of her condition. CONCLUSION: The diagnosis of BML is rare and typically affects patients with a history of uterine leiomyomata s/p hysterectomy. Differentiating BML from malignant metastasis is important to avoid unnecessary procedures or delayed treatment. BML patients generally remain asymptomatic, though some patients may present with pulmonary symptoms. Anti-estrogenic, anti-hormonal therapies and surgical management have been suggested as treatment.