Yang Jia-Wen, Hua Yu, Xu Hua, He Liu, Huo Hai-Zhong, Zhu Chen-Fang
Department of General Surgery, Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Discipline Construction Research Center of China Hospital Development Institute, Shanghai Jiao Tong University, Shanghai 200011, China.
World J Clin Cases. 2021 Jul 6;9(19):5217-5225. doi: 10.12998/wjcc.v9.i19.5217.
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.
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.
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.
播散性腹膜平滑肌瘤病(LPD)是一种罕见疾病,其特征为多个盆腔和腹部结节,由平滑肌细胞构成。迄今为止,报告的病例不超过200例。LPD的诊断困难,且尚无关于其治疗的指南。目前,手术切除是主要治疗方法。然而,激素阻断疗法可作为一种替代选择。
一名33岁女性患者因腹部不适及可触及腹部肿块入院。她在过去8年中接受了4次与子宫平滑肌瘤相关的手术。计算机断层扫描显示腹壁和腹腔内散在多个结节。她的症状及粗针活检结果与LPD相符。患者拒绝手术,随后接受他莫昔芬、醋酸乌利司他(一种选择性孕激素受体调节剂)和醋酸戈舍瑞林(一种促性腺激素释放激素激动剂)治疗。他莫昔芬和醋酸乌利司他均未能有效控制疾病进展。然而,尝试使用醋酸戈舍瑞林时患者取得了良好反应,症状缓解且结节明显缩小。最大结节的最长直径总和从治疗前到治疗后减少了25%。至今已过去2年,患者仍无症状,未出现新的结节。
醋酸戈舍瑞林对LPD的治疗有效。长期使用醋酸戈舍瑞林被认为是安全有效的。激素阻断疗法可替代复发患者反复进行的手术切除。