Malignant presentation of uterine lymphangioleiomyomatosis

Abstract

The history of our patient and review of the literature suggest that although uterine LAM presents malignant features (i.e., metastasis), the disease is long lasting and the patient can be in good condition for a number of years.

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.…”

“…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…”

“…There is clinical and genetic evidence that LAM cells can recur in lung transplants. LAM cells have also been isolated from the lymphatics, blood and urine of LAM patients 1 13 19. Regional lymph node metastases are common in uterine LAM cases 19.…”

“…LAM cells have also been isolated from the lymphatics, blood and urine of LAM patients 1 13 19. Regional lymph node metastases are common in uterine LAM cases 19. The origin of LAM is debated with some authors suggesting metastasis from renal AMLs, whereas others hypothesise a possible gynaecological origin for the cells 1 13 30…”