We report the case of a 56-year-old woman, non-smoker, recently diagnosed with breast cancer, who underwent a chest and abdomen computed tomography (CT) as part of her staging study. Multiple pulmonary cysts with a fine, well-defined wall were unexpectedly detected on the CT (Fig. 1A and B), along with a mass in the right kidney containing foci of fatty attenuation (Fig. 1C), consistent with angiomyolipoma (AML). The pulmonary parenchyma interposed between the pulmonary cysts was rigorously normal. In view of these findings, the patient was given a diagnosis of pulmonary lymphangioleiomyomatosis (PLAM).
(A) Axial image of chest CT (pulmonary parenchymal window) showing multiple cysts in both lungs (arrows). Note the normal radiological appearance of the pulmonary parenchyma interposed between the cysts. (B) Minimum intensity projection (minIP) axial reconstruction of chest CT (pulmonary parenchymal window) showing multiple cysts in both lungs (arrows). (C) Axial CT image of the abdomen, showing a mass (black asterisk) in the right kidney (RD) with an area of fatty density (white asterisk), consistent with angiomyolipoma.
PLAM is a rare systemic disease that occurs predominantly in women of childbearing age. It is characterized by interstitial proliferation of abnormal smooth muscle that can obstruct venules, lymph vessels or bronchioles, particularly in the lung, kidney and spleen, and can lead to the development of lymphadenopathies, lymphangioleiomyomas, or chylous collections in the abdominal cavity. PLAM can be sporadic or appear in association with tuberous sclerosis complex (TSC), and many authors consider it a frustrated form of the latter. The existence of mild or subclinical forms of PLAM has recently been demonstrated in some women with AML, and the incidence of breast cancer is higher in women with PLAM.1,2
Please cite this article as: Gorospe Sarasúa L, Ayala-Carbonero AM, Fernández-Méndez MÁ. Linfangioleiomiomatosis pulmonar incidental en una paciente con cáncer de mama. Arch Bronconeumol. 2017;53:161–162.
