Lymphangioleiomyomatosis: Pulmonary and Abdominal Findings with Pathologic Correlation1
Esther Pallisa, MD,
Pilar Sanz, MD,
Antonio Roman, MD,
Joaquim Majó, MD,
Jordi Andreu, MD and
José Cáceres, MD
1 From the Departments of Radiology (E.P., P.S., J.A., J.C.), Respiratory Medicine (A.R.), and Pathology (J.M.), Hospital General Universitari Vall d’Hebron, Passeig Vall d’Hebron 119–129, 08035 Barcelona, Spain. Presented as an education exhibit at the 2001 RSNA scientific assembly. Received February 8, 2002; revision requested March 18; final revision received June 19; accepted July 2. Address correspondence to E.P. (e-mail: 26787epn@comb.es).
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Figure 1. Typical lung cysts in a 52-year-old woman with LAM. High-resolution CT scan shows well-defined thin-walled bilateral lung cysts (arrow), which are randomly distributed throughout the lungs. The lung parenchyma between the cysts is normal. Note the scarring due to open lung biopsy in the lingula.
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Figure 2. Irregular cysts in a 36-year-old woman with LAM. High-resolution CT scan obtained at the level of the aortic arch shows irregular cysts of different sizes. Some cyst walls are barely seen (white arrow). There are also small nodules (black arrow) in the lung parenchyma.
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Figure 3. Atypical lung cysts in a 34-year-old woman with severe dyspnea. High-resolution CT scan obtained at the lung apices shows lung cysts with an anterior distribution in the upper lobes. There is also a left pleural effusion. The diagnosis of LAM was confirmed at analysis of the explanted lungs.
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Figure 4a. Inspiratory and expiratory findings at the lung bases in a 43-year-old woman with biopsy-proved LAM. (a) Inspiratory high-resolution CT scan shows well-defined lung cysts and normal lung tissue between them. Note the surgical biopsy scar in the left lower lobe. (b) Expiratory high-resolution CT scan shows normal-appearing increased attenuation of the normal lung tissue.
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Figure 4b. Inspiratory and expiratory findings at the lung bases in a 43-year-old woman with biopsy-proved LAM. (a) Inspiratory high-resolution CT scan shows well-defined lung cysts and normal lung tissue between them. Note the surgical biopsy scar in the left lower lobe. (b) Expiratory high-resolution CT scan shows normal-appearing increased attenuation of the normal lung tissue.
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Figure 5a. Reticular pattern in a 38-year-old woman with severe dyspnea and a 3-year history of LAM. (a) Chest radiograph shows a bilateral reticular pattern in the lung bases and bilateral pleural effusions. (b) High-resolution CT scan shows that the reticular pattern is due to multiple cysts.
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Figure 5b. Reticular pattern in a 38-year-old woman with severe dyspnea and a 3-year history of LAM. (a) Chest radiograph shows a bilateral reticular pattern in the lung bases and bilateral pleural effusions. (b) High-resolution CT scan shows that the reticular pattern is due to multiple cysts.
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Figure 6. Pulmonary hemorrhage in a 46-year-old woman 10 months after unilateral left lung transplantation for LAM. CT scan shows increased attenuation in the native right lung and a small right pleural effusion. Bronchoalveolar lavage of the right lung showed hemosiderin-laden macrophages in the alveoli. The transplanted left lung was partially collapsed due to hypoventilation and shift of the mediastinum.
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Figure 7a. Increased lung attenuation in a 34-year-old woman with highly cellular LAM. (a) Inspiratory high-resolution CT scan obtained at the level of the lung bases shows bilateral ground-glass attenuation and intralobular and septal lines. The cysts are elongated and have a subpleural (arrow) and peribronchovascular distribution. (b) High-resolution CT scan of the explanted lung (sagittal view) shows the irregular cystic spaces in the left lower lobe along the bronchovascular bundles (arrow). Note the basal distribution of the cysts and the diffuse increased attenuation of the lung.
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Figure 7b. Increased lung attenuation in a 34-year-old woman with highly cellular LAM. (a) Inspiratory high-resolution CT scan obtained at the level of the lung bases shows bilateral ground-glass attenuation and intralobular and septal lines. The cysts are elongated and have a subpleural (arrow) and peribronchovascular distribution. (b) High-resolution CT scan of the explanted lung (sagittal view) shows the irregular cystic spaces in the left lower lobe along the bronchovascular bundles (arrow). Note the basal distribution of the cysts and the diffuse increased attenuation of the lung.
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Figure 8. Chylothorax. Contrast material-enhanced CT scan (7-mm collimation) obtained at the level of the lung bases shows bilateral pleural effusions, which turned out to be chylous.
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Figure 9. Pneumothorax involving the native lung in a 46-year-old woman 3 months after left lung transplantation for LAM. CT scan shows multiple cysts in the native right lung and a large right-sided hydropneumothorax that displaces the mediastinum, compromising the function of the transplanted lung.
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Figure 10a. (a) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows an enlarged air space with a thick wall due to myomatosis (arrow). The surrounding parenchyma has a normal appearance. (b) Photomicrograph (original magnification, x250; Gomori trichrome stain) shows a myomatous bundle in the wall of the lesion. Arrow indicates the myomatous cells.
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Figure 10b. (a) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows an enlarged air space with a thick wall due to myomatosis (arrow). The surrounding parenchyma has a normal appearance. (b) Photomicrograph (original magnification, x250; Gomori trichrome stain) shows a myomatous bundle in the wall of the lesion. Arrow indicates the myomatous cells.
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Figure 11. Photomicrograph (original magnification, x250; HMB-45) shows myomatous cells that are positive for antimelanoma-associated antigen at cytoplasmic immunostaining.
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Figure 12. Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows enlargement of a small air space (*) between a respiratory bronchiole (solid arrow) and a larger and more distal LAM lesion without myomatosis (open arrow).
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Figure 13. Photomicrograph (Gomori trichrome stain) shows a lung biopsy specimen with typical features of LAM. Note that the enlarged air spaces differ in size and location within the secondary lobule. The thickness of the walls of the enlarged air spaces also differs. There is a small lesion in the centrilobular area (arrow) and a medium-sized lesion at the periphery of the lobule (arrowhead) with a subpleural location; in comparison, there is a medium-sized lesion in the middle lobular area with a thicker myomatous wall (*).
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Figure 14. Photograph (parasagittal section) of a left lung with LAM shows evenly distributed enlarged air spaces.
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Figure 15. Renal angiomyolipomas in a 45-year-old woman with LAM. Unenhanced abdominal CT scan shows multiple bilateral small nodules (arrows). Analysis of CT numbers revealed fat attenuation in the left renal cortex, a finding diagnostic of renal angiomyolipomas.
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Figure 16a. Hyperattenuating angiomyolipoma in a young woman with LAM. Abdominal CT scans obtained before (a) and after (b) administration of contrast material show a slightly hyperattenuating renal tumor (arrows) in the left upper pole. Unenhanced CT could not demonstrate tissue of fat attenuation. CT-guided biopsy was performed to confirm the diagnosis.
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Figure 16b. Hyperattenuating angiomyolipoma in a young woman with LAM. Abdominal CT scans obtained before (a) and after (b) administration of contrast material show a slightly hyperattenuating renal tumor (arrows) in the left upper pole. Unenhanced CT could not demonstrate tissue of fat attenuation. CT-guided biopsy was performed to confirm the diagnosis.
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Figure 17. Lymphangioleiomyomas in a young woman with pulmonary cysts. Contrast-enhanced abdominal CT scan (10-mm collimation) shows low-attenuation retroperitoneal cystic masses (arrows), which are consistent with dilatation of the abdominal lymph vessels.
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Figure 18a. Thick-walled lymphangioleiomyoma in a 39-year-old woman with LAM and multiple retroperitoneal low-attenuation masses. (a) Contrast-enhanced CT scan obtained during the arterial phase shows a hypoattenuating pelvic mass (arrow). (b) Contrast-enhanced CT scan obtained 10 minutes later shows increased attenuation of the mass (right arrow). There is also a small hypoattenuating lesion on the right side (left arrow).
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Figure 18b. Thick-walled lymphangioleiomyoma in a 39-year-old woman with LAM and multiple retroperitoneal low-attenuation masses. (a) Contrast-enhanced CT scan obtained during the arterial phase shows a hypoattenuating pelvic mass (arrow). (b) Contrast-enhanced CT scan obtained 10 minutes later shows increased attenuation of the mass (right arrow). There is also a small hypoattenuating lesion on the right side (left arrow).
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Figure 19. Retroperitoneal lymphadenopathy. Contrast-enhanced abdominal CT scan obtained at the level of the kidneys shows a hyperattenuating enlarged paraaortic lymph node (left arrow). Nodular enhancement within the area of adenopathy (right arrow) occurs in patients with replacement of the normal lymph nodes by smooth muscle cells.
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Figure 20. Chylous ascites in a patient who had undergone bilateral lung transplantation. Contrast-enhanced abdominal CT scan shows abundant abdominal fluid. Paracentesis demonstrated that it was chylous ascites.
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Figure 21a. Tuberous sclerosis in a young woman with seizures. (a) Contrast-enhanced CT scan of the brain shows the typical subependymal calcifications and an astrocytoma (arrow). (b) High-resolution CT scan shows bilateral lung cysts indistinguishable from those seen in LAM.
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Figure 21b. Tuberous sclerosis in a young woman with seizures. (a) Contrast-enhanced CT scan of the brain shows the typical subependymal calcifications and an astrocytoma (arrow). (b) High-resolution CT scan shows bilateral lung cysts indistinguishable from those seen in LAM.
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Figure 22a. Langerhans cell histiocytosis in a young male smoker. (a) High-resolution CT scan shows cystic spaces predominating in both lung apices. The cysts are irregular and have well-defined walls. (b) High-resolution CT scan obtained at the costophrenic angles shows that the lung bases are less affected by small cysts (arrows).
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Figure 22b. Langerhans cell histiocytosis in a young male smoker. (a) High-resolution CT scan shows cystic spaces predominating in both lung apices. The cysts are irregular and have well-defined walls. (b) High-resolution CT scan obtained at the costophrenic angles shows that the lung bases are less affected by small cysts (arrows).
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Figure 23. Pulmonary fibrosis. High-resolution CT scan shows rounded, well-defined cysts in the right lung (black arrow) in a patient with traction bronchiectasis (white arrow) and bilateral basal subpleural honeycombing (arrowheads).
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Figure 24. Emphysema in a 40-year-old smoker. High-resolution CT scan of the right lower and middle lobes obtained at the lung bases shows ill-defined hypoattenuating areas of lung destruction (arrow) that lack recognizable walls.
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Copyright © 2002 by the Radiological Society of North America.
