A case of pulmonary lymphangioleiomyomatosis complicated with uterine and retroperitoneal tumors

Abstract

A 39-year-old female experienced dyspnea on exertion for eight months. Chest CT demonstrated findings of Lymphangioleiomyomatosis (LAM), including diffuse thin-walled cystic lesions. A surgical lung biopsy revealed human melanoma black-45-positive cell infiltration and aggregation, resulting in a diagnosis of sporadic LAM without tuberous sclerosis complex. Pelvic MRI showed two large tumors, one of which was in the myometrium and the other was in the retroperitoneal space. Because we were not able to exclude the presence of malignant tumors using MR imaging, the tumors were surgically resected. The histopathology demonstrated the resected tumors to be composed of LAM cells. The patient's symptoms worsened, and sirolimus was administered, which improved the dyspnea and pulmonary function. The adverse effect was mild liver damage. Following the initiation of treatment with sirolimus, transient elevation of the serum KL-6 level was detected without interstitial pneumonia. This LAM case complicated with large uterine and retroperitoneal tumors was successfully treated with surgical resection and sirolimus.

A case of uterine lymphangioleiomyomatosis in a young woman that was identified via peritonitis

“…Although there are no histopathological differences between TSC‐LAM and sporadic LAM, the presence of different lesions in several organs (e.g., renal angiomyolipoma and noncalcified pulmonary nodules) is more frequent in TSC‐LAM than in sporadic LAM . Occurrence of uterine LAM is rare, and to the best our knowledge, only 10 cases have been reported . Although antiestrogen therapy has been performed to treat LAM for many years, and the efficacy of GnRH analog therapy has also been reported, antiestrogen therapy cannot be considered to be an established treatment for LAM.…”

“…Although antiestrogen therapy has been performed to treat LAM for many years, and the efficacy of GnRH analog therapy has also been reported, antiestrogen therapy cannot be considered to be an established treatment for LAM. Therefore, surgery is ultimately required in many cases …”

“…Cases of LAM have been reported to occur in the retroperitoneal and pelvic cavities . However, there have been few reports of uterine LAM and the incidence of such cases is not well known …”

Extrapulmonary uterine lymphangioleiomyomatosis (LAM) and dysfunctional uterine bleeding: the first presentation of LAM in a tuberous sclerosis complex patient

“…Despite the uterus being the second most common location for PEComas after the retroperitoneum,17 18 to the best of our knowledge, only 26 cases of uterine LAM have been reported in the literature 3 13 14 19–23. Its rarity, variable, sometimes vague presentation and morphology can often make diagnosing LAM challenging (please see tables 1 and 2 for differentials) 4 13 17 18 24–27…”

“…Currently, there are no proven treatment strategies for E-LAM 6 19 23. Resection of the extra-pulmonary mass or cyst is usually adequate local treatment 12.…”