Symptom relief of leiomyomatosis peritonealis disseminata with ulipristal acetate

Leiomyomatosis peritonealis disseminata (LPD) are multiple nodules adherent to and superficially invading the peritoneum, mimicking metastatic ovarian carcinoma. Their origin would be metaplastic mesenchymal cells (MC), which in susceptible women MC could originate from surgical myoma fragments [ 1 ]. Estrogen may stimulate subcoelomic MC to proliferate, differentiating into myoblasts, myofibroblasts, fibroblasts, and even decidua-like cells [ 2 ]. LPD can express sex steroid receptors, hence be promoted by hormonal stimulation, e.g., pregnancy or exogenous intake [ 3 ]. LPD is rare (<200 cases reported), essentially benign, though malignant transformation has been reported [ 4 ]. LPD often is asymptomatic but can cause abdominal distension. We report the first case of successful management of LPD with ulipristal, a selective progesterone receptor modulator (SPRM), registered for preoperative management of uterine myoma [ 5 , 6 ]. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000. Informed consent was obtained from the patient for being included in the study as the use of ulipristal in LPD is off-label.