Renal and pulmonary lymphangioleiomyomatosis: a case report

Lymphangioleiomyomatosis (LAM) is a rare disease that most commonly involves the lung. However, extrapulmonary lymphangioleiomyomatosis rarely occurs with or without subsequent involvement of the lung. We report a case of incidentally found renal and pulmonary lymphangioleiomyomatosis in a patient who had no stigmata of tuberous sclerosis.     

淋巴管肌瘤病的影像表现

目的：探讨淋巴管肌瘤病(LAM)的影像学诊断价值。方法 回顾性分析15例经临床和病理证实的LAM胸部X线平片、HRCT,腹部CT和直接淋巴管造影(DLG)及DLG后胸腹部CT扫描等影像资料。结果 15例中,X线胸片上未见异常1例,双肺纹理增多3例,弥漫性小蜂窝状影或网格状影11例,气胸2例,胸腔积液14例。胸部常规CT和HRCT扫描显示15例均具有典型LAM表现,均可见两肺散在囊状影或广泛密布的囊状影。按Avila等肺部疾病程度分级标准：Ⅰ级3例;Ⅱ级5例;Ⅲ级7例。腹部CT显示14例在腹膜后、盆腔可见囊性淋巴管瘤9例,淋巴管肌瘤13例,二者共同存在7例,并发肝脏脂肪瘤和血管平滑肌脂肪瘤、肾脏小错构瘤及子宫肌瘤各1例。DLG检查,除1例淋巴管梗阻部位在腰3水平外,其余14例均可见胸导管不同程度的狭窄、梗阻及颈干和（或）锁骨下干和（或）支气管纵隔干淋巴管反流。DLG术后CT,除3例未显示胸导管出口梗阻外,其余12例显示胸导管出口梗阻情况与DLG基本一致。结论 HRCT对肺淋巴管肌瘤(PLAM)的诊断具有特征性价值,CT可发现腹部LAM,DLG和DLG后MSCT对因LAM引起的胸导管或淋巴管干梗阻部位的显示具有价值,可为手术治疗提供一定的帮助。     

Sporadic lymphangioleiomyomatosis and tuberous sclerosis complex with lymphangioleiomyomatosis: comparison of CT features

PURPOSE: To retrospectively compare the frequencies of computed tomographic (CT) findings in patients with lymphangioleiomyomatosis (LAM) and patients with tuberous sclerosis complex (TSC) and LAM. MATERIALS AND METHODS: Institutional review board approval and informed consent were obtained for the HIPAA-compliant study. In 256 patients with LAM (mean age, 44 years) and 67 patients with TSC/LAM (mean age, 40 years), CT scans of the chest, abdomen, and pelvis were reviewed by a single radiologist. The fraction of lung involvement with cysts was estimated from high-spatial-resolution CT scans. Other findings assessed included noncalcified pulmonary nodules, pleural effusion, thoracic duct dilatation, hepatic and renal angiomyolipomas (AMLs), lymphangioleiomyoma (LALM), ascites, nephrectomy, and renal embolization. Confidence intervals and hypothesis tests of differences in frequencies, comparison of age quartiles, RIDIT analysis, analysis of variance, and correlation coefficients were used in the statistical analysis. RESULTS: Patients with LAM had more extensive lung involvement (RIDIT score, 0.36) and higher frequency of LALM (29% vs 9%, P<.001), thoracic duct dilatation (4% vs 0, P=.3), pleural effusion (12% vs 6%, P=.2), or ascites (10% vs 6%, P=.3). Patients with TSC/LAM had higher frequency of noncalcified pulmonary nodules (12% vs 1%, P<.01), hepatic (33% vs 2%, P<.001) and renal (93% vs 32%, P<.001) AMLs, nephrectomy (25% vs 7%, P<.001), or renal artery embolization (9% vs 2%, P<.05). CONCLUSION: The extent of lung disease is greater in LAM than TSC/LAM. Hepatic and renal AMLs and noncalcified lung nodules are more common in TSC/LAM, while lymphatic involvement-thoracic duct dilatation, chylous pleural effusion, ascites, and LALM-is more common in LAM.     

Pulmonary lymphangioleiomyomatosis: CT findings

Lymphangioleiomyomatosis, a rare disease occurring in women of childbearing age, is characterized by proliferation of smooth muscle in pulmonary lymphatic channels and mediastinal and abdominal lymph nodes. Chest radiographs typically reveal interstitial disease with normal lung volume. Pneumothorax and pleural effusions may be present. CT scans in eight patients with biopsy-proved pulmonary lymphangioleiomyomatosis were reviewed. The prominent feature of the disease was multiple thin-walled cysts throughout the lungs, best visualized on scans made with 1.5-mm collimation. Mediastinal and/or retrocrural lymphadenopathy, often not appreciated on the chest radiograph, was present in four of eight patients. CT can suggest a diagnosis of lymphangioleiomyomatosis when diagnosis by clinical presentation and chest radiographs is uncertain.     

Lymphangioleiomyomatosis initially presenting with abdominal pain: a case report

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease characterized by hamartomatous smooth muscle proliferation of the lymph node, lymphatics, blood vessels as well as airways within the lungs, mediastinum and abdomen. It exclusively affects women during the reproductive period. Though extrapulmonary manifestations have been reported, the initial presentation of LAM as abdominal pain is extremely rare. To our knowledge, there are only three cases with LAM presenting first with abdominal symptoms so far [Chest 106 (1994) 267; Eur J Radiol 14 (1992) 192; Eur J Surg 157 (1991) 36]. We describe a case of LAM suffering from abdominal pain followed by pulmonary symptoms and the diagnosis was not made until pathohistological examination.     

淋巴管肌瘤病的CT诊断(附二例报告及文献复习)

目的 描述淋巴管肌瘤病的CT表现。方法 回顾分析2例经病理证实的淋巴管肌瘤病的胸腹CT表现。结果 2例胸部高分辨率CT表现为两肺均匀或不均匀分布的薄壁囊状影，1例伴乳糜胸。腹部CT于2例检出腹膜后淋巴结肿大。1例伴有肾脏及腹膜后血管平滑肌脂肪瘤，另1例伴腹膜后淋巴管肌瘤。结论 淋巴管肌瘤病的胸腹部CT表现有一定特征性。熟悉其胸腹部CT表现有助于该病的诊断，但最终确诊须依靠组织病理学检出。     

Lymphangioleiomyomatosis: abdominopelvic CT and US findings

PURPOSE: To describe the abdominal computed tomographic (CT) and ultrasonographic (US) findings in patients with thoracic lymphangioleiomyomatosis (LAM) and to relate the prevalence of the findings to the severity of pulmonary disease. MATERIALS AND METHODS: Eighty patients with LAM underwent chest and abdominopelvic CT and abdominopelvic US. The images were reviewed prospectively by one radiologist, and the abdominal findings were recorded and correlated with the severity of pulmonary disease at thin-section CT. RESULTS: Sixty-one (76%) of 80 patients had positive abdominal findings. The most common abdominal findings included renal angiomyolipoma (AML) in 43 patients (54%), enlarged abdominal lymph nodes in 31 (39%), and lymphangiomyoma in 13 (16%). Less common findings included ascites in eight (10%), dilatation of the thoracic duct in seven (9%), and hepatic AML in three (4%). A significant correlation (P =.02) was observed between enlarged abdominal lymph nodes and increased severity of lung disease. CONCLUSION: There are characteristic abdominal findings in patients with LAM that, in conjunction with the classic thin-section CT finding of pulmonary cysts, are useful in establishing this diagnosis.     

Lung transplantation for lymphangioleiomyomatosis: role of imaging in the assessment of complications related to the underlying disease

PURPOSE: To identify the complications and imaging findings related to lymphangioleiomyomatosis (LAM) after lung transplantation. MATERIALS AND METHODS: The authors retrospectively reviewed the clinical histories and imaging studies of 13 patients from five major medical centers who underwent unilateral (n = 8) or bilateral (n = 5) lung transplantation for LAM between 1991 and 1997. Complications related to LAM, both before and after transplantation, were recorded. RESULTS: The following LAM-related complications were found during and after transplantation: excessive pleural adhesions (n = 4), native lung pneumothorax (n = 3), chylous effusion (n = 1), chylous ascites (n = 3), complications from renal angiomyolipomas (n = 4), and recurrent LAM (n = 1). Diagnosis could be made or suggested with computed tomography (CT) in all cases. Four patients (31%) died; one patient died of complications of LAM. CONCLUSIONS: Patients who have undergone lung transplantation for LAM have increased morbidity and mortality due to complications related to their underlying disease. These LAM-related complications can be diagnosed or suggested with CT.     

Broncheoalveolar carcinoma associated with pulmonary lymphangioleiomyomatosis and Tuberous Sclerosis Complex: case report

Lymphangioleiomyomatosis (LAM) is a rare disorder that predominantly affects the lung parenchyma of young women and it's characterized by pulmonary cyst. Tuberous sclerosis complex (TSC) is a rare genetic disorder presenting with hamartomas and neurologic symptoms. The two renal pathologies most commonly seen in TSC are angiomyolipomas and cysts; less commonly, TSC co-exist with polycystic kidney disease. In this report is described an uncommon case of a patient with broncheoalveolar carcinoma, pulmonary LAM and TSC with polycystic kidney disease.     

Pulmonary lymphangioleiomyomatosis in a male patient with tuberous sclerosis complex

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology that occurs almost exclusively in women of reproductive age. High-resolution computed tomography (HRCT) reveals the striking feature of diffuse cystic changes throughout the lung parenchyma. The correct diagnosis may be delayed by several years after the onset of symptoms because of the rarity of the disease and the need for chest CT scans to identify the lung involvement. We describe a case of pulmonary LAM in a male patient associated with tuberous sclerosis complex (TSC), in whom the early stage of disease could be depicted by chest HRCT scans.     

Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in four cases

Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. The patients present with progressive shortness of breath, pneumothorax, chylous effusion, and hemoptysis. Four patients with biopsy-proved lymphangioleiomyomatosis of the lung were evaluated using high-resolution CT. In all patients, the scan showed well-defined cystic air spaces, surrounded by uniformly thin walls, distributed diffusely throughout both lungs. The cystic air spaces ranged in size from a few millimeters to 5 cm. Pathologically, these cysts were predominantly bounded by normal-looking parenchymal components, with occasional patchy involvement by a smooth-muscle proliferative process. The CT appearance of lymphangioleiomyomatosis differs quite distinctly from that of other diseases that can cause cystic air spaces, such as fibrosing alveolitis, neurofibromatosis, and bronchiectasis, and less distinctly from pulmonary emphysema and eosinophilic granuloma. Our experience in these few cases suggests that the high-resolution CT findings in lymphangioleiomyomatosis are characteristic of the disease.     

Thoracic Computed Tomographic Manifestations of Tuberous Sclerosis in Adults

The purpose of this article is to illustrate the various manifestations that can be encountered on thoracic computed tomography of tuberous sclerosis in adults. The pulmonary findings include lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia. The extrapulmonary manifestations are divided into cardiac, vascular, mediastinal, osseous, and upper abdominal findings.     

Lymphangioleiomyomatosis: CT of diurnal variation of lymphangioleiomyomas.

PURPOSE: To evaluate the imaging and clinical features of lymphangioleiomyomas and to describe the phenomenon of diurnal variation in the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis. MATERIALS AND METHODS: One hundred twenty-eight patients with lymphangioleiomyomatosis underwent chest and abdominopelvic computed tomography (CT). Thirteen patients underwent CT in the morning and afternoon of the same day to assess diurnal variation in lymphangioleiomyoma size. RESULTS: Twenty-seven of 128 patients (21%) had 54 lymphangioleiomyomas. The vast majority (96%) of these masses contained material of low attenuation at CT. Associated CT findings included enlarged abdominal lymph nodes, pleural effusions, ascites, and dilatation of the thoracic duct. The prevalence of lymphangioleiomyomas was 15% in patients who had mild pulmonary disease, 19% in patients who had moderate disease, and 26% in patients who had severe disease. Diurnal variation in size of masses was demonstrated in 12 of 13 patients. Seven of the 27 patients who had masses underwent biopsy; all seven were confirmed to have lymphangioleiomyomas. The most common symptoms associated with lymphangioleiomyomas were bloating, abdominal pain, and edema of the lower extremities. The majority of the patients reported worsening of symptoms as the day progressed. CONCLUSION: Lymphangioleiomyomas are common in patients with lymphangioleiomyomatosis. Diurnal variation in size may explain worsening of symptoms during the day.     

Pulmonary lymphangioleiomyomatosis: high-resolution CT findings

Lymphangioleiomyomatosis (LAM) of the lung is a very rare disease. There are obvious discrepancies in the literature concerning the appearance of LAM on CT scans of the lung. This study adds the imaging findings of 11 patients and demonstrates how the imaging findings changed over time in four patients. Twenty-two CT examinations, and radiographs that had been obtained close to the CT examinations, of 11 patients with LAM confirmed by open lung biopsy were retrospectively evaluated with particular attention to the size of cystic lesions and wall thickness. Furthermore the CT scans were analysed for the type of pulmonary infiltration process and its distribution, presence or absence of pleural effusion, pneumothorax and lymph node enlargement. Clinical and CT follow-up studies were available in four patients. The CT scans revealed an increase in the interstitial pattern in all patients. Architectural distortion was seen in two patients and cystic lesions were present in all. The size of the cysts varied from small lesions to bullous emphysema. The cystic lesions revealed a wall thickness up to 2 mm but a wall was not perceptible in all. Pneumothorax was seen in only two patients; pleural effusion was seen in two patients. CT examination of patients with LAM reveals neither a uniform nor a pathognomonic appearance. In the early stages of LAM or in cases with interstitial changes the differential diagnosis of centrilobular emphysema or idiopathic pulmonary fibrosis seems to be more difficult than most authors believe. Received: 28 July 1997; Revision received: 20 October 1997; Accepted: 25 March 1998     

肺淋巴管肌瘤病的临床及多层螺旋CT特点分析

目的:探讨肺淋巴管肌瘤病(PLAM)的多层螺旋CT(MSCT)表现,结合临床特点进行分析,以期提高对该病的诊断水平。方法:回顾性分析13例经病理证实的肺淋巴管肌瘤病患者的临床及影像资料。结果:13例均以进行性加重的呼吸困难为主要临床表现,4例伴有反复自发性气胸,3例伴乳糜胸,2例伴咯血,1例合并腹部淋巴管瘤。肺功能检查表现以阻塞性通气功能障碍为主合并弥散功能降低;MSCT表现为双肺弥漫分布大小不等的类圆形囊腔影,薄层重组和HRCT图像可清晰显示囊腔薄壁及常规CT不能显示的小的囊腔,囊壁外可见贴壁的血管影。结论:肺淋巴管肌瘤病的MSCT表现具有一定的特征性,结合临床可对多数病例做出正确的诊断。     

Various radiological appearances of angiomyolipoinas in the same kidney

A 21-year-old woman with tuberous sclerosis presented with abdominal distension and flank pain. Imaging studies, including CT and MR imaging, revealed bilateral renal mass lesions, containing fat and suggesting the diagnosis of tuberous sclerosis. However the imaging characteristics of one of these lesions differed from the others with no radiologically detectable fat tissue in this solid lesion suggesting renal cell carcinoma. Histopathological examination of this lesion in the left kidney revealed an angiomyolipoma with minimal fat tissue. The radiological diagnosis of angiomyolipomas with minimal fat tissue remains difficult and the differential diagnosis is discussed. Received: 5 October 1998; Revised: 22 December 1998; Accepted: 7 June 1999     

CT and MR Imaging Findings of Lymphangioleiomyomatosis Involving the Uterus and Pelvic Cavity

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease and this is characterized by a proliferation of abnormal smooth muscle cells in the lungs and in the lymphatic system of the thorax and retroperitoneum. The female genital tract is rarely affected by LAM. We report here on the CT and MR imaging findings of extensive LAM involving the uterus and pelvic cavity, and this was seen as multiple cystic uterine and parauterine masses with internal hemorrhage in a young female with tuberous sclerosis complex.     

肺淋巴管平滑肌瘤病临床报道并文献分析

目的 提高对罕见病肺淋巴管平滑肌瘤病（PLAM）的认识。方法 对2002年收治的3例PLAM患者的临床资料进行分析，并结合文献复习。结果 PLAM是一种罕见的弥漫性肺部痰病，临床表现为反复发作性自发性气胸、活动后呼吸困难和血痰等。肺功能呈阻塞性或混合性通气功能障碍，动脉血气分析示低氧血症。胸部X线表现为肺弥漫分布的网状影，高分辨CT（HRCT）示两肺弥漫性分布囊状改变。病理学检查示肺组织淋巴管增生和扩张，管外平滑肌细胞明显增生。结论 育龄期妇女，如反复出现自发性气胸、血痰、活动后呼吸困难，胸部HRCT示两肺弥漫囊状改变，临床上应想到PLAM可能。PLAM预后差，目前无有效治疗方法。     

A case of lymphangioleiomyomatosis affecting the supraclavicular lymph nodes

The case of a 46-year-old woman with lymphangioleiomyomatosis (LAM) involving the supraclavicular, mediastinal, and pelvic lymph nodes in addition to the lungs is reported. Computed tomography incidentally revealed multiple thin-walled pulmonary cysts and low-attenuating masses in the supraclavicular, mediastinal, and retroperitoneal lymph nodes. A biopsy of the supraclavicular mass was performed and diagnosed as LAM histopathologically. The common sites of extrapulmonary LAM include retroperitoneal and mediastinal lymph nodes; however, supraclavicular lymph node involvement is extremely rare.     

Lymphangioleiomyomatosis: solitary abdominal manifestation (2009: 9b)

Lymphangioleiomyomatosis (LAM) is a rare disease that usually affects premenopausal woman and is characterized by cystic lung lesions and lymphatic disorders. We report a case of a 23-year-old woman who presented with diffuse, but increasing abdominal pain. Transabdominal ultrasound showed multiple cystic formations. Due to the patient’s uncharacteristic symptoms, an exploratory laparotomy with tissue sampling was performed, and the diagnosis of LAM was confirmed by two independent pathologists. With computed tomography a broad abdominal, but no pulmonary, manifestation could be established. During sirolimus therapy the patient showed clinical benefit, but only slight progress in computed tomography.