DOI: 10.1148/rg.253055006
Lymphangioleiomyomatosis: Radiologic-Pathologic Correlation1
Gerald F. Abbott, MD,
Melissa L. Rosado-de-Christenson, MD,
Aletta Ann Frazier, MD,
Teri J. Franks, MD,
Robert D. Pugatch, MD and
Jeffrey R. Galvin, MD
1 From the Dept of Diagnostic Imaging, Brown Medical School, Rhode Island Hospital, 593 Eddy St, Providence, RI 02903 (G.F.A.); Dept of Radiology, Ohio State Univ Medical Center, Columbus, Ohio (M.L.R.); Dept of Radiology and Nuclear Medicine, Uniformed Services Univ of the Health Sciences, Bethesda, Md (M.L.R.); Depts of Radiologic Pathology (A.A.F., J.R.G.) and Pulmonary and Mediastinal Pathology (T.J.F.), Armed Forces Institute of Pathology, Washington, DC; and Dept of Diagnostic Radiology, Univ of Maryland Medical System, Baltimore, Md (A.A.F., J.R.G., R.D.P.). Received January 20, 2005; revision requested January 27 and received February 23; accepted February 25. All authors have no financial relationships to disclose.
View larger version (156K):
[in a new window]
|
Figure 1. LAM in a 32-year-old woman with a 1-month of history of dyspnea. Posteroanterior chest radiograph demonstrates large lung volumes, diffuse bilateral interstitial linear and nodular opacities, and pulmonary artery enlargement suggestive of pulmonary arterial hypertension.
|
|
View larger version (156K):
[in a new window]
|
Figure 2. LAM in a 23-year-old woman with dyspnea, cough, hemoptysis, and weight loss. Posteroanterior chest radiograph demonstrates normal-to-large lung volumes, bilateral increased interstitial opacities, and bilateral pleural effusions (left one larger than the right). The interstitial opacities are more pronounced in the lung bases, and distinct cystic structures are seen in the right lung base (arrow).
|
|
View larger version (148K):
[in a new window]
|
Figure 3a. LAM in a 55-year-old woman with cough and acute onset of dyspnea and chest pain. Posteroanterior (a) and collimated posteroanterior (b) chest radiographs demonstrate a moderate-sized left pneumothorax and the suggestion of cysts in the atelectatic left upper lobe.
|
|
View larger version (114K):
[in a new window]
|
Figure 3b. LAM in a 55-year-old woman with cough and acute onset of dyspnea and chest pain. Posteroanterior (a) and collimated posteroanterior (b) chest radiographs demonstrate a moderate-sized left pneumothorax and the suggestion of cysts in the atelectatic left upper lobe.
|
|
View larger version (79K):
[in a new window]
|
Figure 4a. LAM in a 39-year-old woman with dyspnea. High-resolution CT scans (obtained at descending levels) demonstrate bilateral diffuse small thin-walled cysts with relative sparing of the lung apices (cf a with b and c). Note the moderate left pleural effusion.
|
|
View larger version (91K):
[in a new window]
|
Figure 4b. LAM in a 39-year-old woman with dyspnea. High-resolution CT scans (obtained at descending levels) demonstrate bilateral diffuse small thin-walled cysts with relative sparing of the lung apices (cf a with b and c). Note the moderate left pleural effusion.
|
|
View larger version (92K):
[in a new window]
|
Figure 4c. LAM in a 39-year-old woman with dyspnea. High-resolution CT scans (obtained at descending levels) demonstrate bilateral diffuse small thin-walled cysts with relative sparing of the lung apices (cf a with b and c). Note the moderate left pleural effusion.
|
|
View larger version (118K):
[in a new window]
|
Figure 5a. LAM in a 52-year-old woman with cough and dyspnea on exertion, who recently had undergone laser ablation and pleurectomy for treatment of recurrent spontaneous right pneumothorax. High-resolution CT scans demonstrate mild involvement by thin-walled cysts randomly distributed throughout both lungs with relative sparing of the lung bases (cf b with a).
|
|
View larger version (102K):
[in a new window]
|
Figure 5b. LAM in a 52-year-old woman with cough and dyspnea on exertion, who recently had undergone laser ablation and pleurectomy for treatment of recurrent spontaneous right pneumothorax. High-resolution CT scans demonstrate mild involvement by thin-walled cysts randomly distributed throughout both lungs with relative sparing of the lung bases (cf b with a).
|
|
View larger version (173K):
[in a new window]
|
Figure 6a. LAM in a 34-year-old woman with progressive dyspnea. (a) Posteroanterior chest radiograph depicts large lung volumes and somewhat coarse bilateral diffuse reticular and linear opacities. (b–d) High-resolution CT scans (obtained at descending levels) show diffuse severe pulmonary involvement by thin-walled cysts. Although several cysts are small (2–5 mm), the majority are much larger, measuring up to 12 mm. Note variable cyst shapes including polygonal (arrow in b). (e) Photograph of the resected right lung at the time of single lung transplantation demonstrates profuse involvement of every lung lobe by cysts of varying size.
|
|
View larger version (124K):
[in a new window]
|
Figure 6b. LAM in a 34-year-old woman with progressive dyspnea. (a) Posteroanterior chest radiograph depicts large lung volumes and somewhat coarse bilateral diffuse reticular and linear opacities. (b–d) High-resolution CT scans (obtained at descending levels) show diffuse severe pulmonary involvement by thin-walled cysts. Although several cysts are small (2–5 mm), the majority are much larger, measuring up to 12 mm. Note variable cyst shapes including polygonal (arrow in b). (e) Photograph of the resected right lung at the time of single lung transplantation demonstrates profuse involvement of every lung lobe by cysts of varying size.
|
|
View larger version (142K):
[in a new window]
|
Figure 6c. LAM in a 34-year-old woman with progressive dyspnea. (a) Posteroanterior chest radiograph depicts large lung volumes and somewhat coarse bilateral diffuse reticular and linear opacities. (b–d) High-resolution CT scans (obtained at descending levels) show diffuse severe pulmonary involvement by thin-walled cysts. Although several cysts are small (2–5 mm), the majority are much larger, measuring up to 12 mm. Note variable cyst shapes including polygonal (arrow in b). (e) Photograph of the resected right lung at the time of single lung transplantation demonstrates profuse involvement of every lung lobe by cysts of varying size.
|
|
View larger version (139K):
[in a new window]
|
Figure 6d. LAM in a 34-year-old woman with progressive dyspnea. (a) Posteroanterior chest radiograph depicts large lung volumes and somewhat coarse bilateral diffuse reticular and linear opacities. (b–d) High-resolution CT scans (obtained at descending levels) show diffuse severe pulmonary involvement by thin-walled cysts. Although several cysts are small (2–5 mm), the majority are much larger, measuring up to 12 mm. Note variable cyst shapes including polygonal (arrow in b). (e) Photograph of the resected right lung at the time of single lung transplantation demonstrates profuse involvement of every lung lobe by cysts of varying size.
|
|
View larger version (118K):
[in a new window]
|
Figure 6e. LAM in a 34-year-old woman with progressive dyspnea. (a) Posteroanterior chest radiograph depicts large lung volumes and somewhat coarse bilateral diffuse reticular and linear opacities. (b–d) High-resolution CT scans (obtained at descending levels) show diffuse severe pulmonary involvement by thin-walled cysts. Although several cysts are small (2–5 mm), the majority are much larger, measuring up to 12 mm. Note variable cyst shapes including polygonal (arrow in b). (e) Photograph of the resected right lung at the time of single lung transplantation demonstrates profuse involvement of every lung lobe by cysts of varying size.
|
|
View larger version (102K):
[in a new window]
|
Figure 7a. LAM in a 27-year-old woman with dyspnea who presented with a right spontaneous hydropneumothorax. (a–c) High-resolution CT scans (obtained at descending levels) collimated to the left lung demonstrate diffuse but mild (<25% of the lung parenchyma) lung involvement by small (2–5-mm) thin-walled cysts.
|
|
View larger version (109K):
[in a new window]
|
Figure 7b. LAM in a 27-year-old woman with dyspnea who presented with a right spontaneous hydropneumothorax. (a–c) High-resolution CT scans (obtained at descending levels) collimated to the left lung demonstrate diffuse but mild (<25% of the lung parenchyma) lung involvement by small (2–5-mm) thin-walled cysts.
|
|
View larger version (114K):
[in a new window]
|
Figure 7c. LAM in a 27-year-old woman with dyspnea who presented with a right spontaneous hydropneumothorax. (a–c) High-resolution CT scans (obtained at descending levels) collimated to the left lung demonstrate diffuse but mild (<25% of the lung parenchyma) lung involvement by small (2–5-mm) thin-walled cysts.
|
|
View larger version (84K):
[in a new window]
|
Figure 8a. LAM in a 29-year-old woman with dyspnea and chest pain who presented with a left spontaneous pneumothorax. (a–c) High-resolution CT scans (obtained at descending levels) with targeted reconstructions of the right lung demonstrate moderate (between 25% and 80% of the lung parenchyma) lung involvement by numerous thin-walled cysts. Although many cysts are small (2–5 mm), some are larger (10 mm).
|
|
View larger version (108K):
[in a new window]
|
Figure 8b. LAM in a 29-year-old woman with dyspnea and chest pain who presented with a left spontaneous pneumothorax. (a–c) High-resolution CT scans (obtained at descending levels) with targeted reconstructions of the right lung demonstrate moderate (between 25% and 80% of the lung parenchyma) lung involvement by numerous thin-walled cysts. Although many cysts are small (2–5 mm), some are larger (10 mm).
|
|
View larger version (121K):
[in a new window]
|
Figure 8c. LAM in a 29-year-old woman with dyspnea and chest pain who presented with a left spontaneous pneumothorax. (a–c) High-resolution CT scans (obtained at descending levels) with targeted reconstructions of the right lung demonstrate moderate (between 25% and 80% of the lung parenchyma) lung involvement by numerous thin-walled cysts. Although many cysts are small (2–5 mm), some are larger (10 mm).
|
|
View larger version (132K):
[in a new window]
|
Figure 9a. LAM in a 46-year-old woman who developed progressive dyspnea during pregnancy. (a) High-resolution CT scan shows severe lung involvement by numerous thin-walled cysts of various sizes, with a large cyst in the left upper lobe measuring 30 mm in its largest diameter. (b) Photograph of the resected left lung at the time of lung transplantation demonstrates profuse cysts with several dominant cysts.
|
|
View larger version (169K):
[in a new window]
|
Figure 9b. LAM in a 46-year-old woman who developed progressive dyspnea during pregnancy. (a) High-resolution CT scan shows severe lung involvement by numerous thin-walled cysts of various sizes, with a large cyst in the left upper lobe measuring 30 mm in its largest diameter. (b) Photograph of the resected left lung at the time of lung transplantation demonstrates profuse cysts with several dominant cysts.
|
|
View larger version (132K):
[in a new window]
|
Figure 10. LAM in a 40-year-old woman with progressive dyspnea. High-resolution CT image (targeted reconstruction) of the right lung demonstrates severe cystic lung disease and a pseudo-beaded appearance of the major interlobar fissure.
|
|
View larger version (166K):
[in a new window]
|
Figure 11a. LAM in a 21-year-old woman who presented with a small subtle left spontaneous pneumothorax. (a) Posteroanterior chest radiograph demonstrates increased lung volumes. (b, c) High-resolution CT scans collimated to the left lung show the small left pneumothorax (arrow in b) and mild pulmonary involvement by thin-walled cysts. Some of the cysts have a subpleural distribution. (d) Low-power photomicrograph (original magnification, x200; hematoxylin-eosin [H-E] stain) demonstrates a sub-pleural cyst with marginal round and elongated clusters of LAM cells.
|
|
View larger version (118K):
[in a new window]
|
Figure 11b. LAM in a 21-year-old woman who presented with a small subtle left spontaneous pneumothorax. (a) Posteroanterior chest radiograph demonstrates increased lung volumes. (b, c) High-resolution CT scans collimated to the left lung show the small left pneumothorax (arrow in b) and mild pulmonary involvement by thin-walled cysts. Some of the cysts have a subpleural distribution. (d) Low-power photomicrograph (original magnification, x200; hematoxylin-eosin [H-E] stain) demonstrates a sub-pleural cyst with marginal round and elongated clusters of LAM cells.
|
|
View larger version (122K):
[in a new window]
|
Figure 11c. LAM in a 21-year-old woman who presented with a small subtle left spontaneous pneumothorax. (a) Posteroanterior chest radiograph demonstrates increased lung volumes. (b, c) High-resolution CT scans collimated to the left lung show the small left pneumothorax (arrow in b) and mild pulmonary involvement by thin-walled cysts. Some of the cysts have a subpleural distribution. (d) Low-power photomicrograph (original magnification, x200; hematoxylin-eosin [H-E] stain) demonstrates a sub-pleural cyst with marginal round and elongated clusters of LAM cells.
|
|
View larger version (107K):
[in a new window]
|
Figure 11d. LAM in a 21-year-old woman who presented with a small subtle left spontaneous pneumothorax. (a) Posteroanterior chest radiograph demonstrates increased lung volumes. (b, c) High-resolution CT scans collimated to the left lung show the small left pneumothorax (arrow in b) and mild pulmonary involvement by thin-walled cysts. Some of the cysts have a subpleural distribution. (d) Low-power photomicrograph (original magnification, x200; hematoxylin-eosin [H-E] stain) demonstrates a sub-pleural cyst with marginal round and elongated clusters of LAM cells.
|
|
View larger version (113K):
[in a new window]
|
Figure 12a. LAM in a 40-year-old woman with progressive dyspnea. (a) High-resolution CT scan demonstrates severe pulmonary involvement by cysts of variable sizes, some with thin, others with thick walls. (b) Contrast-enhanced abdominal CT scan shows extensive retroperitoneal lymphadenopathy. The diagnosis of LAM was established from the biopsy specimen from the retroperitoneal lymph nodes.
|
|
View larger version (126K):
[in a new window]
|
Figure 12b. LAM in a 40-year-old woman with progressive dyspnea. (a) High-resolution CT scan demonstrates severe pulmonary involvement by cysts of variable sizes, some with thin, others with thick walls. (b) Contrast-enhanced abdominal CT scan shows extensive retroperitoneal lymphadenopathy. The diagnosis of LAM was established from the biopsy specimen from the retroperitoneal lymph nodes.
|
|
View larger version (88K):
[in a new window]
|
Figure 13a. TSC-LAM in a 29-year-old woman who presented with a right spontaneous hydropneumothorax and spontaneous right retroperitoneal hemorrhage. These findings led to the diagnosis of TSC. (a) Clinical photograph demonstrates the typical appearance of facial angiofibromas. (b) High-resolution CT scan reveals severe pulmonary involvement by thick- and thin-walled cysts of varying sizes and a right hydropneumothorax. (c, d) Abdominal CT scans (c at a higher level than d) show a left renal mass with fat attenuation and a right soft-tissue renal mass surrounded by perinephric hemorrhage.
|
|
View larger version (125K):
[in a new window]
|
Figure 13b. TSC-LAM in a 29-year-old woman who presented with a right spontaneous hydropneumothorax and spontaneous right retroperitoneal hemorrhage. These findings led to the diagnosis of TSC. (a) Clinical photograph demonstrates the typical appearance of facial angiofibromas. (b) High-resolution CT scan reveals severe pulmonary involvement by thick- and thin-walled cysts of varying sizes and a right hydropneumothorax. (c, d) Abdominal CT scans (c at a higher level than d) show a left renal mass with fat attenuation and a right soft-tissue renal mass surrounded by perinephric hemorrhage.
|
|
View larger version (101K):
[in a new window]
|
Figure 13c. TSC-LAM in a 29-year-old woman who presented with a right spontaneous hydropneumothorax and spontaneous right retroperitoneal hemorrhage. These findings led to the diagnosis of TSC. (a) Clinical photograph demonstrates the typical appearance of facial angiofibromas. (b) High-resolution CT scan reveals severe pulmonary involvement by thick- and thin-walled cysts of varying sizes and a right hydropneumothorax. (c, d) Abdominal CT scans (c at a higher level than d) show a left renal mass with fat attenuation and a right soft-tissue renal mass surrounded by perinephric hemorrhage.
|
|
View larger version (85K):
[in a new window]
|
Figure 13d. TSC-LAM in a 29-year-old woman who presented with a right spontaneous hydropneumothorax and spontaneous right retroperitoneal hemorrhage. These findings led to the diagnosis of TSC. (a) Clinical photograph demonstrates the typical appearance of facial angiofibromas. (b) High-resolution CT scan reveals severe pulmonary involvement by thick- and thin-walled cysts of varying sizes and a right hydropneumothorax. (c, d) Abdominal CT scans (c at a higher level than d) show a left renal mass with fat attenuation and a right soft-tissue renal mass surrounded by perinephric hemorrhage.
|
|
View larger version (130K):
[in a new window]
|
Figure 14a. TSC-LAM in a 37-year-old woman with TSC that manifested with mental retardation and seizures who was evaluated for bilateral renal angiomyolipomas. (a) Contrast-enhanced brain CT scan shows numerous calcified periventricular nodules consistent with multifocal tubers. (b) Contrast-enhanced abdominal CT scan demonstrates bilateral renal enlargement by numerous cysts and large bilateral masses of mixed attenuation containing large vascular structures. Note fat-attenuation lesion in the mid pole of the left kidney. (c) High-resolution CT scan shows mild pulmonary involvement by scattered small thin-walled pulmonary cysts. The cysts were incidentally found at abdominal CT, and the patient had no pulmonary symptoms.
|
|
View larger version (128K):
[in a new window]
|
Figure 14b. TSC-LAM in a 37-year-old woman with TSC that manifested with mental retardation and seizures who was evaluated for bilateral renal angiomyolipomas. (a) Contrast-enhanced brain CT scan shows numerous calcified periventricular nodules consistent with multifocal tubers. (b) Contrast-enhanced abdominal CT scan demonstrates bilateral renal enlargement by numerous cysts and large bilateral masses of mixed attenuation containing large vascular structures. Note fat-attenuation lesion in the mid pole of the left kidney. (c) High-resolution CT scan shows mild pulmonary involvement by scattered small thin-walled pulmonary cysts. The cysts were incidentally found at abdominal CT, and the patient had no pulmonary symptoms.
|
|
View larger version (148K):
[in a new window]
|
Figure 14c. TSC-LAM in a 37-year-old woman with TSC that manifested with mental retardation and seizures who was evaluated for bilateral renal angiomyolipomas. (a) Contrast-enhanced brain CT scan shows numerous calcified periventricular nodules consistent with multifocal tubers. (b) Contrast-enhanced abdominal CT scan demonstrates bilateral renal enlargement by numerous cysts and large bilateral masses of mixed attenuation containing large vascular structures. Note fat-attenuation lesion in the mid pole of the left kidney. (c) High-resolution CT scan shows mild pulmonary involvement by scattered small thin-walled pulmonary cysts. The cysts were incidentally found at abdominal CT, and the patient had no pulmonary symptoms.
|
|
View larger version (172K):
[in a new window]
|
Figure 15. LAM, gross features. Photograph of the cut surface of a lung specimen demonstrates numerous cysts evenly distributed throughout the parenchyma with little intervening normal lung tissue.
|
|
View larger version (112K):
[in a new window]
|
Figure 16. LAM, microscopic features. Low-power photomicrograph (original magnification, x40; H-E stain) demonstrates a cystic pattern similar to that seen at gross examination.
|
|
View larger version (81K):
[in a new window]
|
Figure 17. Low-power photomicrograph (original magnification, x40; H-E stain) shows a cyst with only a single, inconspicuous focus of LAM cells (arrowhead).
|
|
View larger version (183K):
[in a new window]
|
Figure 18. Low-power photomicrograph (original magnification, x40; H-E stain) demonstrates abundant LAM cell proliferation with loss of normal architectural features.
|
|
View larger version (185K):
[in a new window]
|
Figure 19. LAM, microscopic features. High-power photomicrograph (original magnification, x400; H-E stain) demonstrates the variable morphology of LAM cells.
|
|
View larger version (143K):
[in a new window]
|
Figure 20. LAM, microscopic features. High-power photomicrograph (original magnification, x400; HMB-45 immunostain) demonstrates the characteristic granular cytoplasmic staining with HMB-45 in a small percentage of cells.
|
|
View larger version (110K):
[in a new window]
|
Figure 21. LAM, microscopic features. High-power photomicrograph (original magnification, x200; H-E stain) of a transbronchial biopsy specimen demonstrates the presence of characteristic LAM cells, which were confirmed with HMB-45 staining.
|
|
View larger version (140K):
[in a new window]
|
Figure 22. LAM. Close-up photograph of a cut section of right lung parenchyma demonstrates extensive cystic changes that extend to apposing pleural surfaces along the interlobar fissures.
|
|
View larger version (142K):
[in a new window]
|
Figure 23a. Pulmonary Langerhans cell histiocytosis in a 32-year-old man. (a, b) High-resolution CT scans demonstrate a small right pneumothorax and irregular, bizarre-shaped cysts that predominantly involve the upper lung zones (a) with relative sparing of the lung bases (b). Nodules are demonstrated in the intervening lung parenchyma.
|
|
View larger version (121K):
[in a new window]
|
Figure 23b. Pulmonary Langerhans cell histiocytosis in a 32-year-old man. (a, b) High-resolution CT scans demonstrate a small right pneumothorax and irregular, bizarre-shaped cysts that predominantly involve the upper lung zones (a) with relative sparing of the lung bases (b). Nodules are demonstrated in the intervening lung parenchyma.
|
|
View larger version (140K):
[in a new window]
|
Figure 24. Centrilobular emphysema in a 59-year-old man. High-resolution CT scan demonstrates multifocal areas of low attenuation with imperceptible walls. The findings predominantly involved the upper lung zones.
|
|
View larger version (112K):
[in a new window]
|
Figure 25. Paraseptal emphysema in a 21-year-old woman with an 11-pack-year history of cigarette smoking. High-resolution CT scan demonstrates areas of low attenuation that have evident walls and are arrayed in a single subpleural tier that extends along interlobar fissures.
|
|
View larger version (120K):
[in a new window]
|
Figure 26. IPF in a 64-year-old man. High-resolution CT scan demonstrates subpleural and basal predominance of honeycomb cystic changes with distortion of adjacent lung parenchyma.
|
|
Copyright © 2005 by the Radiological Society of North America.
