肺淋巴管肌瘤病的临床及病理特点

目的 提高对肺淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)的临床及病理诊断和认识水平.方法 回顾分析复旦大学附属中山医院确诊为肺LAM的病例15例,并复习1993-2008年国内文献的73例报道,分析该病的临床及病理特点.其中1例患者死后行尸体解剖,对其全身各脏器进行病理组织学观察.结果 88例患者均为女性,平均发病年龄(37±9)岁,常见临床表现依次为呼吸困难83例(94%)、咯血48例(54%)、气胸41例(47%)、乳糜胸28例(32%).其中35例患者行肺功能检查,32例存在弥散功能障碍,25例表现为阻寨性通气功能障碍,10例表现为混合性通气功能障碍.胸部高分辨率CT检查显示双肺为典型的弥漫性薄壁囊状阴影.病理检查结果显示肺组织旱弥漫性囊状改变,增生的LAM细胞沿细支气管壁、肺泡壁、淋巴管肇和血管壁周围分布,形成结节状.1例尸解病理检查结果显示肺脏、肾脏、淋巴结及软组织等多器官受累.42例行腹部B超及影像学检查,其中23例伴发肺外LAM.结论 LAM常累及全身多个系统,但肺是最主要的受累器官.育龄期妇女出现进行性呼吸困难、气胸、乳糜胸及高分辨CT表现为弥漫性小囊状改变时,应考虑到LAM的可能.确诊需依赖肺组织活检病理检查结果,并应常规进行腹部及盆腔影像学检查,以了解患者是否合并肺外LAM.     

肺淋巴管肌瘤病并肺母细胞瘤1例临床分析并文献复习

目的 加强肺淋巴管肌瘤病（LAM）合并恶性肿瘤的临床、影像、病理特点的认识,提高对该病的早期认识和治疗水平.方法 分析2013年3月收治的1例肺LAM并肺母细胞瘤患者临床资料及诊治经过,并结合相关文献进行复习.截止至2014年2月,以“Lymphangiomyomatosis、cancer”为检索词,在PubMed检索系统进行检索,论著3篇,综述1篇;在万方数据库中以“淋巴管肌瘤病、恶性肿瘤”为检索词进行检索,病例报告1篇.结果 患者,女,37岁,慢性病程,主要症状为咳嗽、咯血、胸痛3个月,既往有“双肾错构瘤”史,曾行右肾错构瘤切除术,术后病理示血管平滑肌脂肪瘤,左肾未予处理.曾有“自发性气胸”病史.胸部高分辨率CT示右中下肺巨大肿块并纵隔淋巴结肿大,考虑恶性病变;肺LAM.血清血管内皮生长因子D为1 092.61 ng/L.免疫组化示CD56灶（＋）,Vim小细胞（＋）,Syn（＋）,CD99（＋/-）,组织改变考虑为肺母细胞瘤.复习国内外文献,尚无LAM合并肺母细胞瘤的病例报道.结论 近十年来,随着LAM分子发病机制的深入研究,LAM目前可定义为低度恶性转移性肿瘤.本例患者为LAM合并高度恶性肿瘤肺母细胞瘤,在国内外尚属首例报道.但究其机制,是LAM细胞癌变为肺母细胞瘤,或是二者同时存在,目前尚无定论.     

肺移植术的麻醉管理

1 术前管理 肺移植受体多是终末期肺疾病患者,病情多已严重恶化,对麻醉耐受力极差.麻醉医师在术前应详细了解肺移植患者的病情,呼吸和循环系统的病理改变,如通气和弥散功能、肺储备能力.     

肺淋巴管平滑肌瘤病肺移植治疗研究进展

肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)是一种罕见的、发病机制尚不清楚的弥漫性肺部疾病,常见于育龄期妇女,与结节性硬化症(tuberous sclerosis,TSC)基因1和2的突变有关[1,3].该病以异常的平滑肌细胞(LAM细胞)在气道、淋巴管及血管等中异常增殖为特点,最终导致肺实质形成薄壁囊状结构,肺功能减退甚至呼吸衰竭,从而危及患者生命.内科治疗效果欠佳,肺移植为目前惟一可能有效的治疗手段.     

经支气管镜肺活检对肺淋巴管肌瘤病的诊断价值

肺淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)可根据临床、血清学和影像学等标准诊断,仍有部分患者需经病理明确诊断。LAM 需和其他肺部囊性病变鉴别,病理是重要的鉴别诊断依据。经支气管镜肺活检的创伤性小、安全性高,在诊断其他弥漫性肺实质病变中应用广泛,但在 LAM 中的应用尚存争议。根据现有文献,诊断阳性率在33%~87%,气胸发生率为6%,出血发生率为4%。经支气管镜肺活检可以安全地用于 LAM 的有创诊断。     

慢性阻塞性肺病患者治疗依从性对生活质量的影响

目的：探讨慢性阻塞性肺疾病患者治疗依从性对生活质量的影响。方法：选择2006年1月至2006年12月于北京安贞医院呼吸内科门诊就诊的慢性阻塞性肺疾病患者共187例，在门诊就诊期间以诺丁汉健康调查问卷和西雅图阻塞性肺疾病问卷对患者进行调查。结果：有173例患者接受了问卷的调查。2份生活质量问卷的信度分析显示其内部一致性系数分别为0．85、0．77。2份生活质量问卷的结构效度分析显示存在不同程度的统计学相关性。根据患者依从性分成高依从性组（共86例），低依从性患者（共87例），两组患者诺丁汉健康调查问卷总分及其各个亚组和西雅图阻塞性肺疾病问卷总分及其各个亚组得分差异均存在显著差异性（P均〈0．01）。结论：患者的治疗依从性是影响慢性阻塞性肺疾病患者生活质量的因素。     

血清抗脂阿拉伯甘露糖及38kD抗体检测对涂阴肺结核及肺外结核诊断价值

目的　评估血清抗脂阿拉伯甘露糖及38kDa抗体 (LAM38kD IgG)检测对涂阴肺结核和肺外结核的诊断价值。方法 采用斑点免疫金渗滤法 (dotimmunogoldfiltrationassay，DIGFA)检测57例涂阴肺结核，52例肺外结核，32例涂阳肺结核，29例肺癌患者及33例正常人血清中LAM38kD IgG。 结果 涂阴肺结核组LAM38kD IgG阳性率为73.7%，其中痰结核菌涂 (-)培 (+)组为84 .6%，涂 (-)培 (-)组为70 .5%。肺外结核组阳性率为71.2%，涂 (+)肺结核组阳性率93.8%，肺癌组假阳性率31%，健康组假阳性率9.1%。结论 血清LAM38kD IgG检测对涂阴肺结核和肺外结核有一定的辅助诊断价值。     

肺淋巴管平滑肌瘤病的诊治进展

<正>肺淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)是以肺部广泛囊性病变为特征的一种罕见疾病,主要发生在育龄期女性,其发病的基本特征为肺部淋巴管平滑肌细胞(LAM细胞)异常增殖引起肺组织的损害和囊性重建[1,2]。一般为独立疾病,有时可与遗传病结节性硬化症(tuberous sclerosis complex,TSC)合并发生。LAM患者的临床症状主     

联检脑脊液中LAM—IgG和TB—DNA在诊断结核性脑膜炎中的应用

目的 了解脑脊液（CSF）中阿拉伯糖甘露糖脂IgG抗体（LAM－IgG)和TB－DNA指标对结脑的诊断价值。方法 以CSF为标本，用酶联免疫吸附试验（ELISA）检测LAM－IgG，用聚合酶链反应（PCR）检测TB－DNA。结果 102份结脑病人CSF标本，LAM－IgG阳性率51．0％（52／102），TB－DNA阳性率81．4％（83／102），LAM－IgG阳性及／或TB－DNA阳性共93例（91．2％）。40份非结核性的中枢神经系统疾病病例的CSF标本，均未检出LAM－IgG和TB－DNA。结论 LAM－IgG和TB－DNA均是诊断结脑的较好的指标，两者联检可进一步提高检测敏感性。     

肺移植后肺孢子菌肺炎二例

肺孢子菌多见于免疫抑制患者,如获得性免疫缺陷综合征(AIDS)和器官移植患者.实体器官移植患者此病发病率差别较大,与移植器官类型、移植中心及免疫方案相关[1].没有使用预防方案、肺和心肺联合移植患者肺孢子菌肺炎(PCP)发病率为10％～40％[2].我们通过对2例肺移植后并发肺孢子菌肺炎患者的病历资料进行分析,并结合相关文献,提示肺孢子菌可能存在空气传播.     

Do high-resolution CT findings of usual interstitial pneumonitis obviate lung biopsy? Views of pulmonologists

BACKGROUND: High-resolution CT (HRCT) of the lungs has become an essential component to evaluate patients with diffuse lung disease. Little is known, however, about the current practices of pulmonologists caring for patients with these complex conditions, and, in particular, whether HRCT can obviate the need for surgical lung biopsy. OBJECTIVES: To investigate the practices of pulmonologists concerning the acceptability of a HRCT diagnosis in lieu of lung biopsy in diffuse lung disease. METHODS: We asked practicing pulmonologists among membership of the American College of Chest Physicians whether HRCT results could replace lung biopsy in 16 diffuse lung diseases. Responses were examined in light of published evidence, practice guidelines, and certain practice parameters. RESULTS: Two hundred and thirty (52.6%) of 437 eligible physicians responded. Sixty-seven percent (67%) of respondents accepted HRCT diagnosis for idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) despite their awareness of guidelines recommending histological diagnosis. Most would not accept a radiologic diagnosis for lymphangioleiomyomatosis (LAM; 37%) or eosinophilic granuloma (Langerhans' cell histiocytosis, LCH; 19%), even though CT findings are frequently characteristic. Responses were similar by type of clinical practice and recency of fellowship training. Chest physicians who referred patients for HRCT more frequently were more likely to accept HRCT diagnosis (p=0.008) and those who had higher self-ratings of proficiency in reading HRCT (p = 0.004) were more likely to believe HRCT often suggests specific diagnosis. CONCLUSIONS: Most US pulmonologists will accept an HRCT diagnosis of IPF/UIP without lung biopsy, but are reluctant to do so for most other diffuse lung conditions including LAM and LCH.     

Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications

STUDY OBJECTIVES: Pneumothorax is a common complication of lymphangioleiomyomatosis (LAM), and the optimal approach to its treatment and prevention is unknown. Chemical or surgical pleurodesis are often required to prevent recurrence. However, their efficacy in LAM is unclear, and whether they contribute to perioperative complications during lung transplantation is uncertain. SETTING: The LAM Foundation database of registered patients. DESIGN: A questionnaire was sent to all registered patients who had at least one pneumothorax to determine rates and patterns of recurrence and efficacy of interventions. A second questionnaire was sent to registered LAM patients who received a lung transplant.Patients or participants: Of 395 registered patients, 260 patients (66%) reported at least one pneumothorax during their lifetime, 193 of whom (74%) completed the questionnaire. Of the 85 lung transplant patients who were sent a separate questionnaire, 80 patients (94%) responded. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Of the 193 respondents to the pneumothorax questionnaire, data on 676 episodes of pneumothorax were collected. Eighty-two percent (158 of 193 patients) had their first pneumothorax prior to a diagnosis of LAM. One hundred forty patients (73%) had at least one additional pneumothorax, either an ipsilateral recurrence (99 of 140 patients, 71%) or a contralateral pneumothorax (104 of 140 patients, 74%). Recurrence rates were 66% after conservative therapy, 27% after chemical pleurodesis, and 32% after surgery. In patients who had undergone lung transplantation, prior chemical or surgical pleurodesis was performed in 45 of 80 patients (56%). Fourteen of 80 patients (18%) reported pleural-related postoperative bleeding, 13 of whom (93%) had prior pleurodesis. CONCLUSIONS: Chemical pleurodesis or surgery are equally effective and better than conservative therapy in preventing recurrence of pneumothorax in LAM. Due to the high recurrence rate, either procedure should be considered for the initial pneumothorax in these patients. However, both contribute to increased perioperative bleeding following lung transplantation, with no effect on length of hospital stay.     

Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism

Lymphangiomyomatosis (LAM) is characterized by the proliferation of abnormal smooth muscle cells and cystic degeneration of the lung. LAM affects almost exclusively young women. Although lung transplantation provides effective therapy for end-stage LAM, there are reports of LAM recurrence after lung transplantation. Whether these recurrent LAM cells arise from the patient or the lung transplant donor is an area of controversy. We used microsatellite marker fingerprinting and TSC2 gene mutational analysis to study a patient with recurrent LAM after single-lung transplantation. The DNA microsatellite marker pattern indicated the presence of patient-derived LAM cells in the allograft. A somatic one base pair deletion in exon 18 of the TSC2 gene was identified in pulmonary and lymph node LAM cells before transplantation. The same mutation was in the recurrent LAM, demonstrating that the recurrent LAM was derived from the patient. Fluorescence in situ hybridization revealed that cells immunoreactive with the monoclonal antibody HMB-45 did not contain a Y chromosome. These data indicate that histologically benign LAM cells can migrate or metastasize in vivo to the transplanted lung. In addition, the patient had no evidence of a renal angiomyolipoma at autopsy and therefore demonstrated for the first time that somatic TSC2 mutations cause LAM in patients without angiomyolipomas.     

Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a multisystem disorder of women, characterized by cystic degeneration of the lungs, renal angiomyolipomas (AML), and lymphatic abnormalities. LAM lesions result from the proliferation of benign-appearing, smooth muscle-like LAM cells, which are characterized by loss of heterozygosity (LOH) of one of the tuberous sclerosis complex (TSC) genes. LAM cells are believed to migrate among the involved organs. Because of the apparently metastatic behavior of LAM, we tried to isolate LAM cells from body fluids. A cell fraction separated by density gradient centrifugation from blood had TSC2 LOH in 33 of 60 (55%) LAM patients. Cells with TSC2 LOH were also found in urine from 11 of 14 (79%) patients with AML and in chylous fluid from 1 of 3 (33%) patients. Identification of LAM cells with TSC2 LOH in body fluids was not correlated with severity of lung disease or extrapulmonary involvement and was found in one patient after double lung transplantation. These studies are compatible with a multisite origin for LAM cells. They establish the existence of disseminated, potentially metastatic LAM cells through a relatively simple, noninvasive procedure that should be valuable for molecular and genetic studies of somatic mutations in LAM and perhaps other metastatic diseases.     

Unwillingness of rheumatoid arthritis patients to risk adverse effects

OBJECTIVE: To evaluate patient willingness to accept the risk of adverse effects (AEs) commonly associated with arthritis medications. METHODS: Rheumatoid arthritis patients were asked to rate their willingness to take a medication associated with 17 specific AEs using a visual analogue scale. RESULTS: We interviewed 100 patients. Eighty-one were currently using one or more disease-modifying anti-rheumatic drugs (DMARDs) and 29 had previously experienced AEs related to DMARDs. Seventy-five stated that they were doing very well or well with respect to their arthritis compared with other people their age. Thirty-five per cent of those interviewed were unwilling to accept the risk of cosmetic changes, 38% were unwilling to accept the risk of temporary discomfort and 45% were unwilling to accept the risk of major toxicity. Patients who had previously experienced AEs were more willing to accept the risk of cosmetic changes (83 vs. 58%, P=0.02), temporary discomfort (79 vs. 55%, P=0.02) and major toxicity (83 vs. 44%, P=0.001) compared with those who had not previously experienced AEs. CONCLUSIONS: Many rheumatoid arthritis patients are very concerned about potential drug toxicity. However, risk adversity appeared to be attenuated by past experience with AEs. Our results suggest that certain patients, especially those with milder disease activity, might be reluctant to accept commonly used arthritis medications if they are fully informed of their potential toxicity.     

Need for ambulatory cardiology after-care rehabilitation in the metropolitan area of a large city. Results of the Cologne model of ambulatory cardiologic rehabilitation--phase II

Within the Cologne Model (CM) of outdoor cardiac rehabilitation (OCR), phase II investigations about the demand for this form of cardiac rehabilitation (CR) after acute cardiac diseases were carried out in three general hospitals, the cardiological and cardiosurgical university hospitals of Cologne. The subsequent questions were investigated: total number of coronary or cardiac operated patients, number of patients with indication for CR, and number of patients corresponding to the restricted indications of CM (age below 65, low risk patient, no cardiac operation). For these groups the acceptance or refusal of CR was checked. Subsequently a sample of patients corresponding to the criteria of CM or of operated patients were confronted with the additional offer of an OCR. The motivation for the acceptance of rehabilitation in specialized hospitals (ICR), OCR or refusal of each kind of CR was inquired. The acceptance of CR in the different groups varied widely. Whereas operated patients in Cologne accept CR in nearly 100% of cases, this is the case in patients after acute myocardial infarction (AMI) in only 50% and in patients after PTCA without AMI in only 5-6%. The analysis of predictors for acceptance brings about that younger patients prefer CR, and if they do, OCR. Patients with the more serious form of disease prefer ICR. Women accept CR more rarely than men, and if they do, they prefer the hospital form. However, this is less gender specific but consequence of the generally more serious form and later onset of CAD in females. Higher educational as well as occupational status favors acceptance of CR and specially OCR. The suspicions that unmarried people prefer OCR and foreigners ICR could not be generally confirmed. Crucial reasons for the form of CR which is accepted or refused are individual ones. ICR is favored by the wish for more safety and better recuperation. For OCR, the comfortable conditions at home with high social support and/or antipathy against hospitals after long clinical treatment are named. Analysis of demand for OCR demonstrates that between 40% in low risk patients (corresponding to CM criteria) and 20% in more serious cases (operated patients) prefer the outhospital form. From these data an estimation of demand for OCR in areas with high population was carried out.     

Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis

STUDY OBJECTIVES: The American College of Chest Physicians Delphi Consensus Statement on management of spontaneous pneumothorax recommended pleurodesis after the first secondary spontaneous pneumothorax to prevent recurrence, and evaluation of patients' perspectives regarding pneumothorax treatment was identified as a future research priority. Patients with lymphangioleiomyomatosis (LAM) are an ideal population for performing these studies, since pneumothorax occurs and recurs more commonly in LAM than in any other chronic pulmonary disorder.Study design and participants: A 23-item questionnaire evaluating opinions of pneumothorax treatment was distributed to 615 patients in the LAM Foundation patient database, with a response rate of 52%. RESULTS: Of respondents, 69% (216 of 314 patients) reported a history of radiographically documented pneumothorax, and 181 patients (84%) reported at least one pleurodesis procedure. Neither a history of pneumothorax nor surgical management of pneumothorax affected reported oxygen use or perception of overall lung function, yet 41% thought that their pneumothorax had contributed to a decline in lung function. Few patients (12%) worried frequently about a pneumothorax developing, but one third made lifestyle modifications due to fear of pneumothorax. Extensive pain associated with chest tube placement and inadequate pain management throughout treatment for pneumothorax were frequent concerns. Only 25% of respondents thought that pleurodesis was appropriate for a first pneumothorax, while 60% favored pleurodesis for a second pneumothorax. Despite the apparent reluctance to undergo pleurodesis, most patients agreed that pleurodesis helps prevent pneumothorax recurrence. One third of patients believed that their physicians did not consider their preferences regarding pneumothorax management. CONCLUSIONS: LAM patients and physicians may have different views about the significance of pneumothorax, in that most patients appear to favor a conservative initial approach to pneumothorax management. In conjunction with appropriate pain management, a better understanding of patients' perspectives will facilitate cooperative decision making and may ultimately improve clinical outcomes in LAM related to pneumothorax.     

Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P).

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disorder of unknown cause characterized by peribronchial, perivascular, and perilymphatic proliferation of abnormal smooth muscle cells leading to cystic lesions. The hypothesis of hormonal dependence and the effectiveness of hormonal therapy have not yet been demonstrated conclusively, and the prevalence of extrathoracic manifestations and the survival of patients with LAM are somewhat contradictory. A multicentric retrospective study was conducted in an attempt to describe better the initial features, the diagnostic procedures, the associated lesions, and, above all, the management and course of LAM in a large homogeneous series of 69 stringently selected patients, with a majority of cases diagnosed since 1990. The aim of the study, based on a review of the literature, also was to provide a comprehensive view of this uncommon disease. The clinical features were in keeping with previous studies, but we found that exertional dyspnea and pneumothorax were the most common features, and chylous involvement was less frequent. LAM was diagnosed after menopause in about 10% of cases. The onset of LAM occurred during pregnancy in 20% of cases, and a clear exacerbation of LAM was observed in 14% of cases during pregnancy. Pulmonary LAM was diagnosed on lung histopathology in 83% of cases, but renal angiomyolipoma, observed in 32% of our patients, may be a useful diagnostic criterion when associated with typical multiple cysts on chest CT scan or with chylous effusion. Chest CT scan was more informative than chest X-ray (normal in 9% of cases), and may be indicated in spontaneous pneumothorax or renal angiomyolipoma in women of childbearing age. About 40% of the patients had a normal initial spirometry, while an obstructive ventilatory defect (44%), a restrictive ventilatory defect (23%), was observed in other patients. Initial diffusing capacity for carbon monoxide was frequently decreased (82%). Hormonal therapy was administered in 57 patients, but a clear > or = 15% improvement of FEV1 was observed in only 4 evaluable patients, treated with tamoxifen and progestogens (n = 2), progestogen (n = 1), and oophorectomy (n = 1). Probably 1 of the most urgent needs for clinical research in LAM is to test the currently available hormonal treatments in the context of international multicenter prospective controlled studies. Pleurodesis was performed in 40 patients. Lung transplantation was performed in 13 patients, 7.8 +/- 5.2 years after onset of LAM, in whom the mean FEV1 was 0.57 +/- 0.15 L. After a follow-up of 2.3 +/- 2.2 years, 9 patients were alive. Mean follow-up from onset of disease to either death or closing date was 8.2 +/- 6.3 years. Overall survival was better than usually reported in LAM, and Kaplan-Meier plot showed survival probabilities of 91% after 5 years, 79% after 10 years, and 71% after 15 years of disease duration.     

A case of bronchostenosis in the allograft after lung transplantation in pulmonary lymphangioleiomyomatosis]

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease characterized by hamartomatous proliferation of abnormal smooth muscle cells in the lungs. Recently, severe LAM has been listed as an indicated disease for lung transplantation. A 34-yr-old woman with severe pulmonary cystic changes in a chest CT scan was diagnosed as having an isolated form of pulmonary LAM without genetic disorders. Despite intensive progesterone treatment, her pulmonary functions deteriorated rapidly. In January 2001, a left single-lung transplantation was performed from a cadaveric donor. The total operating time was 8 hours and 47 minutes. Total ischemic time was 5 hours and 59 minutes, which was within the permitted time limit. Except for right pneumothorax, the postoperative course was fairly good without any sign of rejection or infection in the allograft. For about two months after transplantation, bronchostenosis occurred in the left lower lobe bronchus, and necessitated a stent placement. During the following three months, stenosis of the bronchi in the anastomotic and peripheral sites occurred repeatedly, which also necessitated stent placement or balloon dilations on each occasion. Despite all the intensive treatment, the bronchostenosis of the peripheral sites still remains and improvement of her pulmonary functions has been poor. Moreover, a recent chest CT scan revealed a progression of the disease in the native lung. Consequently, we registered her as a candidate for transplantation of the right lung. Bronchostenosis should be kept in mind as a complication of lung transplantation.     

Living-donor lobar lung transplantation for pulmonary and abdominopelvic lymphangioleiomyomatosis

We performed emergency living-donor lobar lung transplantation (LLTx) successfully in a 24-year-old woman with end-stage pulmonary and massive abdominopelvic lymphangioleiomyomatosis (LAM). Preoperatively, her respiratory condition was critical, but abdominopelvic lesions had been well controlled with medication. No LAM patients with massive abdominopelvic lesions as in the present case have been reportedly treated by LLTx previously. The present case demonstrates that LLTx can be a therapeutic option for end-stage pulmonary LAM with massive abdominopelvic involvement.