Lymphangioleiomyomatosis (LAM) is a rare disease that typically affects women of childbearing age. It most commonly affects the lungs (P-LAM) but can occasionally occur in extra-pulmonary sites (E-LAM). There is a strong association between LAM and the tuberous sclerosis complex (TSC). We report a case of a 42-year-old female TSC sufferer who presented with dysfunctional uterine bleeding. She was not known to have LAM. An endometrial biopsy revealed a spindled-cell lesion suspicious of leiomyosarcoma, which correlated with cross-sectional imaging. She underwent a hysterectomy that showed a bizarre (symplastic) leiomyomatous endometrial polyp with background uterine LAM. We discuss the clinical and pathological implications of this unusual case of E-LAM and the importance of clinicopathological correlation in TSC sufferers. The association of uterine LAM with TSC is important and LAM should be considered as a differential of dysfunctional uterine bleeding and a benign mimic to uterine leiomyosarcoma in patients with TSC.

SummaryThe authors present a case of a 51-year-old woman with endometriosis who developed immune thrombocytopenic purpura. Her platelets dropped to 2×10 9 /l, and she had haemorrhage into her right endometrioma causing a large pelvic haematoma. She also had renal failure secondary to endometriosis compressing the right ureter. She was treated with immunoglobulin and steroids with subsequent recovery of platelet count and underwent ureteric stenting to prevent worsening renal function.