Pulmonary lymphangioleiomyomatosis in a 46-year-old female: A case report and review of the literature

Abstract

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease occurring frequently in women. The present study reported a case of PLAM in a 46-year-old female and reviewed the literature regarding PLAM. The female experienced dyspnea on exertion for 1 month. The chest computed tomography (CT) revealed diffuse thin-walled cystic lesions, consistent with radiological features of PLAM. CT-guided lung biopsy showed that characteristic smooth muscle cells were positive for smooth muscle actin, human melanoma black 45 and D2-40 by histology and immunohistochemistry techniques, resulting in a diagnosis of PLAM. The patient had a hysterectomy for treating uterine myoma 12 years previously. Additionally, lung diffusion function was impaired slightly, abdominal CT showed a right renal cyst and pelvic ultrasound exhibited a right oophoritic cyst. Sirolimus was administered, which improved the dyspnea and pulmonary function, and the patient required long-term follow-up. Therefore, this is a rare case of PLAM with renal cyst, oophoritic cyst and uterine myoma in a female. In conclusion, PLAM is a rare proliferative disease in the lung. Chest CT characteristically showed that the cysts were bilateral, diffuse and thin-walled. Lung biopsy may provide evidence for the diagnosis of PLAM, which was treated with sirolimus.

Keywords: Northwestern China; case report; pulmonary lymphangioleiomyomatosis; sirolimus.

Figure 1.

Assessment of a patient with pulmonary lymphangioleiomyomatosis. (A) Abdominal computed tomography (CT) and (B) pelvic ultrasound.

Figure 2.

Assessment of a patient with pulmonary lymphangioleiomyomatosis. (A) Chest computed tomography (CT) and (B) bronchoscopy.

Figure 3.

Assessment of a patient with pulmonary lymphangioleiomyomatosis. (A) Computed tomography (CT)-guided lung biopsy. (B) Hematoxylin and eosin staining (H&E) of the lung biopsy samples and immunohistochemistry demonstrates positive staining for smooth muscle actin (SMA), human melanoma black 45 (HMB45) and D2-40 (magnification, ×10).