Diagnostic Challenges in Lymphangioleiomyomatosis: From Ovarian Mass to Systemic Diagnosis.

Background: Clinical case: A 48-year-old woman presented with abdominal numbness, leading to the discovery of a right ovarian mass. CT and MRI identified non-malignant solid mass in the ovary, as well as thin-walled cysts in the lungs, retroperitoneal pelvis, and upper abdomen, suggesting LAM. The patient was referred for pulmonology evaluation. Genetic testing and lung biopsy were inconclusive. One year later, during laparoscopic hysterectomy for early-stage uterine cancer, a biopsy of a left iliac lesion confirmed LAM. Postoperatively, the patient developed lymphocytic and chylous ascites, requiring further surgical intervention. However, the ascites recurred, and it was managed with diuretic therapy. Following the confirmed diagnosis, Conclusions: Due to its rarity, diverse symptoms, and involvement of multiple organs, diagnosis of LAM is challenging. It requires careful clinical observation and a multidisciplinary approach. Early and accurate diagnosis, combined with timely therapeutic interventions, has the potential to significantly improve the patient outcomes in LAM.