Symptom relief of leiomyomatosis peritonealis disseminata with ulipristal acetate
© The Author(s) 2013
Received: 24 July 2013
Accepted: 4 October 2013
Published: 5 November 2013
KeywordsLeiomyomatosis peritonealis disseminata Nodules Myoma Ulipristal
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 . Estrogen may stimulate subcoelomic MC to proliferate, differentiating into myoblasts, myofibroblasts, fibroblasts, and even decidua-like cells . LPD can express sex steroid receptors, hence be promoted by hormonal stimulation, e.g., pregnancy or exogenous intake . LPD is rare (<200 cases reported), essentially benign, though malignant transformation has been reported . 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.
We believe that this first use of ulipristal for LPD was successful because lesions were expressing progesterone receptors. The subjective and objective responses were striking. Its effect is underscored by a proven reduction in size of lesions and their regrowth following abortion of ulipristal. Though asymptomatic, the disappearance of LPD was incomplete. In addition, she wishes to conceive, so debulking surgery was offered, though the patient reports no side effects, which sharply contrasts with her previous use of GnRH agonists. There is little known on prolonged ulipristal administration. Endometrial atypical hyperplasia may be a concern, though never described for ulipristal. Endometrial biopsy after 12-month exposure is however reassuring. In conclusion, ulipristal acetate may be considered as an adjunct in the management of women with LPD.
Conflict of interest
Jasper Verguts has received a speaker honorarium from Gedeon Richter. Guy Orye and Sophie Marquette declare that they have no conflict of interest.
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