A case report of lymphangioleiomyomatosis with retroperitoneal masses in pregnancy.
A case report of lymphangioleiomyomatosis with retroperitoneal masses in pregnancy.
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DOI：
10.3389/fmed.2023.1313503
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发表时间：
2023
影响因子：
3.9
通讯作者：
Wang, Yu
中科院分区：
文献类型：
--
作者：
Zhu, Yashi;Wang, Chao;Ding, Jianyi;Yang, Meiqin;Bo, Yin;Ma, Mingjun;Hu, Haoran;Cheng, Jiejun;Han, Lingfei;Wang, Yu
关键词：
淋巴管平滑肌瘤病是一种罕见的，逐渐进展的肿瘤，原因不明。其特征在于肺平滑肌细胞的异常增殖，主要表现在育龄妇女中。在这项研究中，我们的目的是提出一个值得注意的情况下，LAM伴随淋巴管平滑肌瘤在腹膜后间隙在怀孕期间，一个场景容易误诊。一位31岁的女性，面临意外怀孕，在第13周提出了一个囊实性肿块显示丰富的血液信号在盆腔，如常规产科超声显示。同时，她的胸部CT显示双肺弥漫性薄壁腔。尽管没有临床症状，患者放弃妊娠并接受了完全刮宫术。然而，术后24天，她再次入院进行进一步评估，发现腹主动脉和下腔静脉周围的肿块增大，沿着压迫输尿管的中段和下段。经过多学科讨论和患者解释后，进行了剖腹探查术，结果完全切除了肿瘤。术中病理检查及免疫组化染色提示腹膜后肿块无恶性征象。综合形态学和免疫表型特征证实了淋巴管平滑肌瘤病的诊断。术后过程顺利，最终患者出院。考虑淋巴管平滑肌瘤病（LAM）合并腹膜后肿瘤在盆腔和腹部肿块的鉴别诊断中至关重要。这种肿瘤的术前诊断是一个挑战，因为超声或CT扫描可能不会产生明确的结果。准确的诊断不仅需要对腹膜后肿块进行病理检查，而且还需要与患者的胸部高分辨率计算机断层扫描（HRCT）所见和相应的临床表现相关联。最佳的管理包括根治性手术，外科医生在制定治疗计划时综合考虑胎儿和母体的情况。
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.
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影响因子：
24.3
作者：
Johnson, SR
通讯作者：
Johnson, SR
DOI：
10.1164/rccm.201709-1965st
发表时间：
2017-11-15
影响因子：
24.7
作者：
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通讯作者：
McCormack, Francis X.
DOI：
10.1164/rccm.200409-1298oc
发表时间：
2006-01-01
影响因子：
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作者：
Ryu, JH;Moss, J;Fanburg, BL
通讯作者：
Fanburg, BL
DOI：
10.1183/16000617.0163-2019
发表时间：
2020-09-30
期刊：
European respiratory review : an official journal of the European Respiratory Society
影响因子：
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作者：
通讯作者：
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影响因子：
15.3
作者：
Musella, Angela;De Felice, Francesca;D'Andrea, Vito
通讯作者：
D'Andrea, Vito