Zhu Yashi, Wang Chao, Ding Jianyi, Yang Meiqin, Bo Yin, Ma Mingjun, Hu Haoran, Cheng Jiejun, Han Lingfei, Wang Yu
Department of Gynecology, Shanghai First Maternity and Infant Hospital, School of Medicine, Tongji University, Shanghai, China.
Department of Radiology, Shanghai First Maternity and Infant Hospital, School of Medicine, Tongji University, Shanghai, China.
Front Med (Lausanne). 2023 Dec 22;10:1313503. doi: 10.3389/fmed.2023.1313503. eCollection 2023.
Lymphangioleiomyomatosis (LAM) is a rare, gradually advancing tumor of unknown origin. It is distinguished by the anomalous proliferation of pulmonary smooth muscle cells and predominantly manifests in women of childbearing age. In this study, we aim to present a noteworthy case of LAM accompanied by lymphangioleiomyoma in the retroperitoneal space during pregnancy, a scenario susceptible to misdiagnosis.
A 31-year-old woman, facing an unintended pregnancy, presented during the 13th week with a cystic-solid mass exhibiting abundant blood signals in the pelvic cavity, as revealed by routine obstetrical ultrasound. Concurrently, her chest CT disclosed diffuse thin-walled cavities in both lungs. Despite the absence of clinical symptoms, the patient abandoned pregnancy and underwent a complete curettage. However, 24 days post-operation, she was readmitted for further assessment, revealing an enlargement of the mass encompassing the abdominal aorta and inferior vena cava, along with compression on the middle and lower segments of the ureter. After a multi-disciplinary discussion and patient explanation, an exploratory laparotomy was performed, resulting in the complete removal of the tumor. Intraoperative pathological examination and immunohistochemical staining indicated a retroperitoneal mass devoid of malignant evidence. The comprehensive morphologic and immunophenotypic features substantiated the diagnosis of lymphangioleiomyomatosis. The postoperative course was uneventful, culminating in the patient's discharge.
The consideration of Lymphangioleiomyomatosis (LAM) with a retroperitoneal tumor is crucial in the differential diagnosis of pelvic and abdominal masses. The preoperative diagnosis of this tumor poses a challenge, as ultrasound or CT scans may not yield definitive results. Accurate diagnosis necessitates not only a pathological examination of the retroperitoneal mass but also the correlation with the patient's chest High-Resolution Computed Tomography (HRCT) findings and corresponding clinical manifestations. Optimal management involves radical surgery, with surgeons comprehensively factoring in both fetal and maternal conditions when formulating a treatment plan.
淋巴管平滑肌瘤病（LAM）是一种罕见的、渐进性发展的起源不明的肿瘤。它以肺平滑肌细胞异常增殖为特征，主要发生于育龄女性。在本研究中，我们旨在呈现一例妊娠期间伴有腹膜后淋巴管平滑肌瘤的LAM显著病例，这种情况易被误诊。
一名31岁意外怀孕的女性，在孕13周时经常规产科超声检查发现盆腔内有一个囊实性肿块，伴有丰富血流信号。同时，她的胸部CT显示双肺弥漫性薄壁空洞。尽管没有临床症状，患者还是终止了妊娠并进行了刮宫术。然而，术后24天，她因进一步评估再次入院，发现肿块增大，累及腹主动脉和下腔静脉，并压迫输尿管中下段。经过多学科讨论并向患者解释后，进行了剖腹探查术，成功完整切除肿瘤。术中病理检查及免疫组化染色显示腹膜后肿块无恶性证据。综合形态学和免疫表型特征证实为淋巴管平滑肌瘤病。术后恢复顺利，患者最终出院。
在盆腔和腹部肿块的鉴别诊断中，考虑伴有腹膜后肿瘤的淋巴管平滑肌瘤病（LAM）至关重要。该肿瘤的术前诊断具有挑战性，因为超声或CT扫描可能无法得出明确结果。准确诊断不仅需要对腹膜后肿块进行病理检查，还需要结合患者胸部高分辨率计算机断层扫描（HRCT）结果及相应临床表现。最佳治疗方案是根治性手术，外科医生在制定治疗计划时应综合考虑胎儿和母体情况。
