Department of Obstetrics and Gynecology, Qilu Hospital of Shandong University, 107 Wenhua Xi Road, Jinan, 250012, People's Republic of China.
Department of Obstetrics and Gynecology, Shanghai General Hospital, School of Medicine, Shanghai Jiao Tong University, 650 XinSongjiang Road, Shanghai, 201620, People's Republic of China.
World J Surg Oncol. 2020 May 2;18(1):85. doi: 10.1186/s12957-020-01857-0.
Peritoneal leiomyomatosis disseminate (LPD) is a rare disease characterized by widespread dissemination of leiomyomas nodules throughout the peritoneal and omental surfaces. Reports of pregnancy with LPD are even rarer. Therefore, there is no clear consensus on the treatment of LPD on pregnancy, and the pathogenesis is still unclear.
We reported a case of LPD patient who developed during pregnancy. The patient underwent a cesarean section at 32 weeks of gestation while removing all visible tumors, and no LPD lesions were seen in the subsequent cesarean section at full term. NGS of LPD lesions detected 4 mutations with focal high-level amplifications of CDK4 (cyclin-dependent kinases 4), NBN (Nibrin), DAXX (death domain associated protein), and MYC (myelocytomatosis oncogene). Immunohistochemistry staining analysis among benign leiomyoma, LPD, and leiomyosarcoma verified that LPD was an unusual intermediate between benign and malignant uterine smooth muscle tumors. Besides, LPD is a hormonal-dependent leiomyoma. After a detailed literature search, we summarized the detailed clinical features and follow-up information of patients with LPD during pregnancy.
This is the first reported LPD case of successful term pregnancy without recurrence, following resection of all visible lesions in a prior pregnancy. LPD is an unusual intermediate between benign and malignant uterine smooth muscle tumors.
腹膜平滑肌瘤病播散(LPD)是一种罕见疾病,其特征为广泛播散的平滑肌瘤结节遍布腹膜和大网膜表面。LPD 合并妊娠的报道更为罕见。因此,对于妊娠合并 LPD 的治疗尚无明确共识,其发病机制仍不清楚。
我们报告了一例妊娠合并 LPD 的患者。患者在妊娠 32 周时行剖宫产术,切除所有可见肿瘤,足月时再次剖宫产未见 LPD 病变。LPD 病变的 NGS 检测到 4 个突变,存在 CDK4(细胞周期蛋白依赖性激酶 4)、NBN(Nibrin)、DAXX(死亡域相关蛋白)和 MYC(髓母细胞瘤癌基因)的局灶性高水平扩增。良性平滑肌瘤、LPD 和平滑肌肉瘤之间的免疫组织化学染色分析证实 LPD 是良性和恶性子宫平滑肌肿瘤之间的一种不常见的中间状态。此外,LPD 是一种激素依赖性平滑肌瘤。经过详细的文献检索,我们总结了妊娠合并 LPD 患者的详细临床特征和随访信息。
这是首例成功足月妊娠且无复发的 LPD 病例,在先前妊娠中切除所有可见病变后。LPD 是良性和恶性子宫平滑肌肿瘤之间的一种不常见的中间状态。