Treatment of leiomyomatosis peritonealis disseminata with goserelin acetate: A case report and review of the literature

Abstract

Background: Leiomyomatosis peritonealis disseminata (LPD) is a rare condition characterized by multiple pelvic and abdominal nodules, which are composed of smooth-muscle cells. To date, no more than 200 cases have been reported. The diagnosis of LPD is difficult and there are no guidelines on the treatment of LPD. Currently, surgical excision is the mainstay. However, hormone blockade therapy can be an alternative choice.

Case summary: A 33-year-old female patient with abdominal discomfort and palpable abdominal masses was admitted to our hospital. She had undergone four surgeries related to uterine leiomyoma in the past 8 years. Computed tomography revealed multiple nodules scattered within the abdominal wall and peritoneal cavity. Her symptoms and the result of the core-needle biopsy were consistent with LPD. The patient refused surgery and was then treated with tamoxifen, ulipristal acetate (a selective progesterone receptor modulator), and goserelin acetate (a gonadotropin-releasing hormone agonist). Both tamoxifen and ulipristal acetate were not effective in controlling the disease progression. However, the patient achieved an excellent response when goserelin acetate was attempted with relieved syndromes and obvious shrinkage of nodules. The largest nodule showed a 25% decrease in the sum of the longest diameters from pretreatment to posttreatment. Up to now, 2 years have elapsed and the patient remains asymptomatic and there is no development of further nodules.

Conclusion: Goserelin acetate is effective for the management of LPD. The long-term use of goserelin acetate is thought to be safe and effective. Hormone blockade therapy can replace repeated surgical excision in recurrent patients.

Keywords: Case report; Conservative treatment; Disseminated peritoneal leiomyomatosis; Goserelin acetate; Hormone blockade therapy; Leiomyomatosis peritonealis disseminata.

Figure 1

Pathological examination of leiomyomatosis peritonealis disseminate. A: Hematoxylin-eosin staining of tumor cells showed a spindle-shaped smooth-muscle cell tumor without necrosis and atypia (400-fold); B-F: Immunohistochemical staining of smooth-muscle cells showed that Caldesmon (B), Desmin (C), smooth-muscle actin (D), estrogen receptors (E), and progesterone receptors (F) were positive (400-fold).

Figure 2

Computed tomography and magnetic resonance imaging scans before and after treatment. The red arrows indicate the nodules involving the abdominal wall and pelvic cavity. A: Computed tomography (CT) scan of the pelvis in the transverse plane in January 2018; B: CT scan of the pelvis in the sagittal plane in January 2018; C: Magnetic resonance imaging (MRI) scan of the pelvis in the transverse plane in December 2019; D: MRI scan of the pelvis in the sagittal plane in December 2019.

Figure 3

Comparison before and after treatment. A: Computed tomography scan of the biggest nodule before treatment in January 2018; B: Magnetic resonance imaging (MRI) scan of the biggest nodule after tamoxifen therapy in July 2018; C: MRI scan of the biggest nodule after first goserelin acetate therapy in January 2019; D: MRI scan of the biggest nodule after ulipristal acetate treatment in June 2019; E: MRI scan of the biggest nodule after second goserelin acetate therapy in December 2019; F: MRI scan of the biggest nodule in October 2020.