Cheng Dan, Zhang Fangcheng, Hu Ke
Department of Respiratory and Critical Care Medicine, Renmin Hospital of Wuhan University, Wuhan, China.
Lung Biology Center, Department of Medicine, University of California, San Francisco, CA.
Medicine (Baltimore). 2019 Jul;98(30):e16496. doi: 10.1097/MD.0000000000016496.
Multiple pulmonary leiomyomatous hamartoma (MPLH) is an extremely rare benign disease that mostly occurs in women of reproductive age.
A 32-year-old female patient recently diagnosed with multiple bilateral pulmonary nodules. She has the symptoms of dry cough, chest tightness, dyspnea on exertion. Chest X-ray identified multiple bilateral pulmonary nodules in the lung, and the diameter of the largest nodule was about 3.1 cm.
Pathology confirmed the diagnosis of MPLH based on morphology and immunohistochemical staining.
The patient presented with multiple well-defined nodular shadows in chest computed tomography (CT), atypical image and symptoms were detected. Positron emission tomography/CT scan showed mild fluorine-18 fluorodeoxyglucose uptake in the lesions and no abnormal foci in any other parts of her body. She subsequently underwent a video-assisted thoracoscopic surgery with wedge resection of the biggest one of the nodules. Then the patient given symptomatic treatment, without hormone, no further treatment was prescribed.
The patient is in the good general condition and without obvious pulmonary symptoms after the follow-up of 1 year, chest CT scan showed no significant changes in the sizes and locations of her bilateral pulmonary nodules.
Due to its rare presentation, the primary MPLH may be undiagnosed. Awareness of main morphologic and immunohistochemical features of MPLH is critical for the recognition of this uncommon disease.
多发性肺平滑肌瘤性错构瘤（MPLH）是一种极为罕见的良性疾病，主要发生于育龄女性。
一名32岁女性患者近期被诊断出双侧多发性肺结节。她有干咳、胸闷、活动后呼吸困难等症状。胸部X线检查发现肺部双侧有多个肺结节，最大结节直径约3.1厘米。
病理检查根据形态学和免疫组化染色确诊为MPLH。
患者胸部计算机断层扫描（CT）显示多个边界清晰的结节状阴影，检测到非典型图像和症状。正电子发射断层扫描/CT扫描显示病变部位有轻度氟-18氟脱氧葡萄糖摄取，身体其他部位未发现异常病灶。随后她接受了电视辅助胸腔镜手术，对最大的一个结节进行楔形切除。然后给予患者对症治疗，未使用激素，未开其他进一步治疗的医嘱。
随访1年后，患者一般情况良好，无明显肺部症状，胸部CT扫描显示双侧肺结节大小和位置无明显变化。
由于其表现罕见，原发性MPLH可能无法得到诊断。了解MPLH的主要形态学和免疫组化特征对于识别这种罕见疾病至关重要。
