Xiao Shan, Chen Yijia, Tang Qianjue, Xu Lianwei, Zhao Li, Wang Zhenzhen, Yu Erkai
Department of Gynecology, LongHua Hospital Affiliated to Shanghai University of Traditional Chinese Medicine, Shanghai, China.
Front Med (Lausanne). 2022 Jul 15;9:917628. doi: 10.3389/fmed.2022.917628. eCollection 2022.
Lymphangioleiomyomatosis (LAM) is a rare low-grade metastatic tumor with an unknown origin that spreads through lymphatic vessels. It is characterized by the proliferation of smooth muscle-like or epithelioid tumor cells in the lung and axial lymphatic system. Extrapulmonary LAM is a localized disease with a low incidence rate, and the location of the related lesions is atypical. It is difficult to diagnose. The LAM of pelvic lymph nodes is hidden. It is usually found through gynecological oncology surgery.
We report a 57-year-old postmenopausal woman with a pelvic mass and vaginal bleeding as the main symptoms. The patient had no history of pulmonary LAM, tuberous sclerosis complex (TSC), or renal angiomyolipoma and had not used exogenous hormones. We performed a total hysterectomy, bilateral adnexectomy, greater omentum resection, and pelvic lymphadenectomy under laparoscopy. The postoperative pathology confirmed high-grade serous carcinoma of the left fallopian tube, and four lymph nodes were found in the pelvic lymph nodes, suggesting lymphangiomyomatosis. Immunohistochemical results also showed that these cells could express markers of smooth muscle cells and melanoma cells. The patient was treated with chemotherapy after the operation. Chest CT did not suggest lung LAM during the postoperative follow-up, and there was no tumor recurrence.
The diagnosis of this disease is challenging. At the same time, due to insufficient clinical samples, it is still unknown whether there is a potential relationship between pelvic and peritoneal lymph node LAM found in the surgical staging of gynecological tumors and lung LAM and/or TSC. There is no evidence that pelvic and peritoneal lymph node LAM will increase the risk of pulmonary LAM. Therefore, additional clinical data are required to analyze and summarize the relationship between pelvic and peritoneal lymph node LAM, pulmonary LAM, and the source of LAM. We present a case of pelvic lymph node LAM and propose a hypothesis that the pathogenesis of endometriosis can be used for reference in the study of this disease.
淋巴管平滑肌瘤病（LAM）是一种罕见的低级别转移性肿瘤，起源不明，通过淋巴管扩散。其特征是肺和轴位淋巴系统中平滑肌样或上皮样肿瘤细胞的增殖。肺外LAM是一种发病率较低的局限性疾病，相关病变位置不典型，诊断困难。盆腔淋巴结LAM较为隐匿，通常通过妇科肿瘤手术发现。
我们报告一名57岁绝经后女性，以盆腔肿块和阴道出血为主要症状。该患者无肺LAM、结节性硬化症（TSC）或肾血管平滑肌脂肪瘤病史，未使用过外源性激素。我们在腹腔镜下进行了全子宫切除术、双侧附件切除术、大网膜切除术和盆腔淋巴结清扫术。术后病理证实为左侧输卵管高级别浆液性癌，盆腔淋巴结中发现4个淋巴结提示淋巴管平滑肌瘤病。免疫组化结果还显示这些细胞可表达平滑肌细胞和黑色素瘤细胞的标志物。患者术后接受化疗。术后随访期间胸部CT未提示肺LAM，且无肿瘤复发。
本病诊断具有挑战性。同时，由于临床样本不足，妇科肿瘤手术分期中发现的盆腔和腹膜淋巴结LAM与肺LAM和/或TSC之间是否存在潜在关系仍不清楚。没有证据表明盆腔和腹膜淋巴结LAM会增加肺LAM的风险。因此，需要更多临床数据来分析和总结盆腔和腹膜淋巴结LAM、肺LAM以及LAM来源之间的关系。我们报告一例盆腔淋巴结LAM病例，并提出一个假说，即子宫内膜异位症的发病机制可为本病研究提供参考。
