Pulmonary benign metastasizing leiomyoma in patients aged 45 years and younger: clinical features and novelty in treatment

Abstract

Background: Pulmonary benign metastasizing leiomyoma (PBML) is the most common extrauterine spread of uterine leiomyoma and its biological behavior is traditionally thought to be hormone-dependent. Studies about the older PBML patients has been previously reported, but limited literature has been regarding to the clinical features and treatment of PBML in young women. Methods: A total of 65 cases of PBML in women aged 45 years and younger were reviewed, including 56 cases selected from PubMed and 9 cases from our hospital. The clinical characteristics and management of these patients were analyzed. Results: The median age of all the patients at diagnosis was 39.0 years. PBML most commonly presented as bilateral solid lesions (60.9%), with other rare imaging manifestations. Median interval time from a pertinent gynecologic procedure to diagnosis was 6.0 years. 16.7% of patients received careful observation and all achieved stable status in a median follow-up time of 18.0 months. 71.4% of patients were administrated anti-estrogen therapies, including surgical castration (33.3%), gonadotropin-releasing hormone analogue (23.8%) and anti-estrogen drugs (14.3%). Eight of 42 patients underwent surgical resection of metastatic lesions. Patients receiving curative surgery for the removal of pulmonary lesions, combined with adjuvant anti-estrogen therapies, had favorable outcomes compared with the ones who only received surgical resection. The disease control rates of surgical castration, gonadotropin-releasing hormone analogue, anti-estrogen drugs were 85.7%, 90.0%, 50.0%, respectively. Rapamycin for two patients achieved successful relief of symptoms and control of pulmonary lesions without lowering hormone levels and causing estrogen deficiency symptoms. Conclusions: In the absence of standard treatment guidelines for PBML, maintaining a low-estrogen environment by different kinds of anti-estrogen therapies has been the mainstream strategy currently and has satisfying curative effects. A wait-and-see strategy might be an option, but therapeutic approaches must be contemplated when complications or symptoms progressed. For PBML in young women, the negative effect on ovarian function of anti-estrogen treatment especially surgical castration should be considered. Rapamycin might be a new treatment option for young PBML patients, especially for those ones who want to preserve ovarian function.

Leiomioma Pulmonar Benigno Metastatizante, una Causa Infrecuente de Nódulos Pulmonares: Reporte de Caso y Revisión de Literatura

“…Además, al igual que la leiomiomatosis uterina, cuando los leiomiomas se presentan en sitios distantes al útero presentan respuesta positiva a estrógenos y la progesterona inhibe el crecimiento tumoral lo que sugiere su origen ginecológico (Nguyen T, 2017), (Su H, 2023). Dentro de los diagnósticos diferenciales se encuentran otras enfermedades como metástasis maligna de pulmón, cáncer de pulmón primario, tuberculosis pulmonar, sarcoidosis así como enfermedades proliferativas del músculo liso siendo estas de origen primario como hamartomas, linfangioleiomiomatosis, leiomioma pulmonar primario, leiomiosarcoma o metastásicas como leiomiosarcoma metastásico y leiomiomatosis benigna metastácica (Khan M, 2018); Caracterizando al LBM por su localización periférica dentro del parénquima pulmonar y por la falta de desarrollo linfovascular y de las vías respiratorias asociadas así como la nula actividad metabólica evidenciada al realizar tomografía por emisión de positrones (Su H, 2023), (L.B, 2020). Sin embargo, estos hallazgos son inespecíficos por lo que continúa siendo necesaria la evaluación histopatológica.…”

Pulmonary benign metastasizing leiomyoma presented as acute hypoxemic respiratory failure: A case report

“…Pulmonary benign metastasizing leiomyoma (PBML) is a rare lung disease and it often occurs in women of childbearing age with a history of uterine leiomyoma and undergo uterine broids surgery [1]. The onset of PBML is very insidious, and patients may experience coughing, sputum, shortness of breath, or no symptoms.…”