CT and MR imaging findings of lymphangioleiomyomatosis involving the uterus and pelvic cavity

Abstract

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease and this is characterized by a proliferation of abnormal smooth muscle cells in the lungs and in the lymphatic system of the thorax and retroperitoneum. The female genital tract is rarely affected by LAM. We report here on the CT and MR imaging findings of extensive LAM involving the uterus and pelvic cavity, and this was seen as multiple cystic uterine and parauterine masses with internal hemorrhage in a young female with tuberous sclerosis complex.

Keywords: Computed tomography (CT); Lymphangioleiomyomatosis; Magnetic resonance (MR); Tuberous sclerosis complex; Uterus.

Fig. 1

MR and CT images of 29-year-old woman with clinical history of tuberous sclerosis complex. A. Axial T1-weighted MR image shows small hyperintense intramural lesion (arrow) at right side of uterine body. Uterine cavity is markedly distended and filled with high signal intensity fluid (H), which is suggestive of hematometra. Another hyperintense round lesion is seen at right ovary (open arrow). B. Axial T2-weighted MR image shows ovoid intramural lesion of mild hyperintensity (arrow), which is suggestive of intralesional hemorrhage. Right ovarian lesion shows signal shading, which is suggestive of chronic repetitive bleeding in lesion (open arrow). C. Coronal reformatted contrast-enhanced CT obtained five years after initial MR imaging shows multiple, large, lobulated thick-walled high density cystic masses (arrows) involving uterus and pelvic cavity. Endometrial cavity (asterisk) is dilated and filled with high density fluid. Right kidney (RK) is enlarged by multiple, variable sized masses of fat and soft tissue attenuation, and this is compatible with angiomyolipomas. D, E. Axial fat-suppressed T1-weighted image (D) and T2-weighted image (E) show huge, irregularly shaped masses (arrows) involving uterus and parauterine pelvic cavity; there is high signal intensity on T1-weighted image and mixed low and high signal intensity on T2-weighted image. High signal intensity is seen in dilated uterine cavity (asterisk) on T1-weighted image, which suggests hematometra. Multiloculated fluid collections with fluid-fluid levels are seen in cul-de-sac (open arrows). F. Sagittal T2-weighted image shows large, lobulated mass (arrows) that originated from uterine myometrium. Uterine cavity is dilated (asterisk). Small amount of fluid collection is seen in cul-de-sac (open arrow). LO = left ovary. G. Axial chest CT shows numerous, well-defined, thin-walled cysts (arrows), distributed diffusely throughout lungs. H. Axial FLAIR (fluid attenuated inversion recovery) image of brain shows multiple cortical and subcortical tubers (white arrows), as well as subependymal nodule (open arrow), in left lateral ventricle, and this all represents tuberous sclerosis. I. Tumor is composed of smooth muscle cells that are arranged in short fascicles around dilated lymphatic vessels, and this is consistent with lymphangioleiomyomatosis (Hematoxylin & Eosin, × 200). J. Tumor cells show diffuse cytoplasmic staining for HMB-45 (immunostains, × 400).