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From the Archives of the AFIP

Pulmonary Langerhans Cell Histiocytosis¹

¹ From the Department of Diagnostic Imaging, Brown Medical School, Rhode Island Hospital, 593 Eddy St, Providence, RI 02903 (G.F.A.); Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, Md (M.L.R.); Department of Diagnostic Radiology, University of Maryland Medical System, Baltimore (A.A.F., J.R.G.); and Departments of Pulmonary and Mediastinal Pathology (T.J.F.) and Radiologic Pathology (A.A.F., J.R.G.), Armed Forces Institute of Pathology, Washington, DC. Received January 9, 2004; revision requested January 23 and received February 10; accepted February 16. All authors have no financial relationships to disclose. Address correspondence to G.F.A. (e-mail: gabbott@cox.net).

View larger version (124K): [in a new window]   Figure 1.  LC morphology. Oil immersion photomicrograph (original magnification, x1200; hematoxylin-eosin [H-E] stain) shows LCs with irregular, deeply grooved nuclei and indistinct cell borders.

View larger version (94K): [in a new window]   Figure 2a.  Electron microscopic features of LCs. (a) Drawing of an LC shows numerous surface dendritic processes, a convoluted nucleus, and a prominent nucleolus. Rod-shaped Birbeck granules (inset) characteristically have a pentalaminar appearance with a zipperlike central axis. Dilatations at the rod ends give granules their characteristic tennis racquet appearance. (b) High-power electron photomicrograph demonstrates Birbeck granules located near the cell nucleus. Note parallel linear structures separated by finely granular material and a terminal expansion producing the tennis racquet appearance.

View larger version (209K): [in a new window]   Figure 2b.  Electron microscopic features of LCs. (a) Drawing of an LC shows numerous surface dendritic processes, a convoluted nucleus, and a prominent nucleolus. Rod-shaped Birbeck granules (inset) characteristically have a pentalaminar appearance with a zipperlike central axis. Dilatations at the rod ends give granules their characteristic tennis racquet appearance. (b) High-power electron photomicrograph demonstrates Birbeck granules located near the cell nucleus. Note parallel linear structures separated by finely granular material and a terminal expansion producing the tennis racquet appearance.

View larger version (122K): [in a new window]   Figure 3.  Gross features of PLCH. Photograph of a lung biopsy specimen from a patient with PLCH demonstrates multiple grayish-white irregular nodules with intervening relatively normal lung parenchyma.

View larger version (170K): [in a new window]   Figure 4.  Microscopic features of PLCH. Intermediate-power photomicrograph (original magnification, x480; H-E stain) of a nodular lesion shows infiltrates composed of variable numbers of LCH cells (arrow), eosinophils (arrowhead), lymphocytes, fibroblasts, plasma cells, neutrophils, and pigmented cells. The latter are smoker’s macrophages derived from smoker’s bronchiolitis.

View larger version (163K): [in a new window]   Figure 5.  Three-dimensional appearance of a PLCH lesion. Artist’s rendering, based on the reconstructions by Kambouchner et al (40), illustrates the elongated morphology and variable cellular and fibrotic composition of PLCH with correlative histologic sections. As a PLCH lesion evolves, the nodule of densely packed cells (bottom, a) is centripetally replaced by fibrous tissue and ultimately becomes a stellate scar (top, c). This continuum of change may be evident within a single lesion. PLCH lesions are bronchiolocentric and propagate both proximally and distally along the small airways. The involved bronchiolar lumen may become either dilated or obliterated. The histologic sections correspond to the early, middle, and late phases of PLCH. In the early phase (a), there is a densely cellular nodule with delicate stellate extensions along the adjacent alveolar walls (original magnification, x12; H-E stain). As the disease progresses (b), cellularity diminishes as fibroblasts replace the lesion (original magnification, x19.2; H-E stain). Note that the stellate extensions have become more prominent, the central bronchiole (*) is dilated, and adjacent alveolar spaces have coalesced because of focal destruction of alveolar walls (paracicatricial air-space enlargement). In the final phase (c), the characteristic LCH cells are absent and only a fibrous, stellate scar remains (original magnification, x24; H-E stain). This phase is often accompanied by paracicatricial air-space enlargement (**).

View larger version (155K): [in a new window]   Figure 6.  Scanning magnification appearance. Low-power photomicrograph (original magnification, x12; H-E stain) of a wedge biopsy specimen shows discrete bronchiolocentric stellate lesions separated by uninvolved lung with air-space enlargement.

View larger version (165K): [in a new window]   Figure 7a.  PLCH in a 41-year-old man with fatigue, malaise, weight loss, and a 50-pack year history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle bilateral upper lobe nodules with indistinct borders. (c, d) High-resolution CT scans (lung window) of the right (c) and left (d) lungs show multifocal nodules and cysts. The nodules have irregular borders, and the cyst walls have variable thickness ranging from thin and uniform to nodular and irregular.

View larger version (155K): [in a new window]   Figure 7b.  PLCH in a 41-year-old man with fatigue, malaise, weight loss, and a 50-pack year history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle bilateral upper lobe nodules with indistinct borders. (c, d) High-resolution CT scans (lung window) of the right (c) and left (d) lungs show multifocal nodules and cysts. The nodules have irregular borders, and the cyst walls have variable thickness ranging from thin and uniform to nodular and irregular.

View larger version (144K): [in a new window]   Figure 7c.  PLCH in a 41-year-old man with fatigue, malaise, weight loss, and a 50-pack year history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle bilateral upper lobe nodules with indistinct borders. (c, d) High-resolution CT scans (lung window) of the right (c) and left (d) lungs show multifocal nodules and cysts. The nodules have irregular borders, and the cyst walls have variable thickness ranging from thin and uniform to nodular and irregular.

View larger version (145K): [in a new window]   Figure 7d.  PLCH in a 41-year-old man with fatigue, malaise, weight loss, and a 50-pack year history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle bilateral upper lobe nodules with indistinct borders. (c, d) High-resolution CT scans (lung window) of the right (c) and left (d) lungs show multifocal nodules and cysts. The nodules have irregular borders, and the cyst walls have variable thickness ranging from thin and uniform to nodular and irregular.

View larger version (167K): [in a new window]   Figure 8a.  PLCH in an 18-year-old woman with chronic cough and no known history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle, bilateral reticular and nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (c-d) Targeted unenhanced high-resolution CT scans (lung window) of the upper (c), middle (d), and lower right (e) lung show irregular nodules as well as thin- and thick-walled cysts. The profusion of abnormalities is greatest in the upper lung and progressively decreases in the middle and lower lung zones.

View larger version (150K): [in a new window]   Figure 8b.  PLCH in an 18-year-old woman with chronic cough and no known history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle, bilateral reticular and nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (c-d) Targeted unenhanced high-resolution CT scans (lung window) of the upper (c), middle (d), and lower right (e) lung show irregular nodules as well as thin- and thick-walled cysts. The profusion of abnormalities is greatest in the upper lung and progressively decreases in the middle and lower lung zones.

View larger version (168K): [in a new window]   Figure 8c.  PLCH in an 18-year-old woman with chronic cough and no known history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle, bilateral reticular and nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (c-d) Targeted unenhanced high-resolution CT scans (lung window) of the upper (c), middle (d), and lower right (e) lung show irregular nodules as well as thin- and thick-walled cysts. The profusion of abnormalities is greatest in the upper lung and progressively decreases in the middle and lower lung zones.

View larger version (165K): [in a new window]   Figure 8d.  PLCH in an 18-year-old woman with chronic cough and no known history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle, bilateral reticular and nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (c-d) Targeted unenhanced high-resolution CT scans (lung window) of the upper (c), middle (d), and lower right (e) lung show irregular nodules as well as thin- and thick-walled cysts. The profusion of abnormalities is greatest in the upper lung and progressively decreases in the middle and lower lung zones.

View larger version (146K): [in a new window]   Figure 8e.  PLCH in an 18-year-old woman with chronic cough and no known history of cigarette smoking. (a, b) Posteroanterior (a) and posteroanterior collimated (b) radiographs show subtle, bilateral reticular and nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (c-d) Targeted unenhanced high-resolution CT scans (lung window) of the upper (c), middle (d), and lower right (e) lung show irregular nodules as well as thin- and thick-walled cysts. The profusion of abnormalities is greatest in the upper lung and progressively decreases in the middle and lower lung zones.

View larger version (163K): [in a new window]   Figure 9a.  PLCH in a 35-year-old woman with a history of smoking who presented with a 3-month history of fever, chills, night sweats, weight loss, and nonproductive cough. Posteroanterior (a) and posteroanterior collimated (b) radiographs show bilateral coarse reticular areas of opacity and cystic changes that predominantly involve the upper and middle lung zones and spare the lung bases.

View larger version (138K): [in a new window]   Figure 9b.  PLCH in a 35-year-old woman with a history of smoking who presented with a 3-month history of fever, chills, night sweats, weight loss, and nonproductive cough. Posteroanterior (a) and posteroanterior collimated (b) radiographs show bilateral coarse reticular areas of opacity and cystic changes that predominantly involve the upper and middle lung zones and spare the lung bases.

View larger version (146K): [in a new window]   Figure 10.  PLCH in a 25-year-old woman with a 4-day history of increasing dyspnea. Posteroanterior radiograph shows bilateral pneumothoraces. Note reticular areas of opacity and cystic changes in the partially collapsed lungs.

View larger version (172K): [in a new window]   Figure 11a.  PLCH in a 54-year-old woman with pleuritic chest pain. (a) Posteroanterior radiograph shows bilateral irregular nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (b) Posteroanterior radiograph obtained 1 year later shows spontaneous regression and complete resolution of the radiographic abnormalities.

View larger version (175K): [in a new window]   Figure 11b.  PLCH in a 54-year-old woman with pleuritic chest pain. (a) Posteroanterior radiograph shows bilateral irregular nodular areas of opacity that predominantly affect the middle and upper lung zones and spare the lung bases. (b) Posteroanterior radiograph obtained 1 year later shows spontaneous regression and complete resolution of the radiographic abnormalities.

View larger version (120K): [in a new window]   Figure 12a.  PLCH in a 31-year-old man with cough, anorexia, weight loss, and a 15 pack-year history of cigarette smoking. (a) High-resolution CT scan (lung window) shows nodules and thick- and thin-walled cysts. Some nodules exhibit a centrilobular distribution. (b) Low-power photomicrograph (original magnification, x12; H-E stain) demonstrates a cellular nodule with stellate peripheral extensions into adjacent alveolar walls. (c) Low-power photomicrograph (original magnification x30; H-E stain) shows one of the cystic lesions produced by bronchiolar dilatation. Note the surrounding paracicatricial air-space enlargement (**).

View larger version (178K): [in a new window]   Figure 12b.  PLCH in a 31-year-old man with cough, anorexia, weight loss, and a 15 pack-year history of cigarette smoking. (a) High-resolution CT scan (lung window) shows nodules and thick- and thin-walled cysts. Some nodules exhibit a centrilobular distribution. (b) Low-power photomicrograph (original magnification, x12; H-E stain) demonstrates a cellular nodule with stellate peripheral extensions into adjacent alveolar walls. (c) Low-power photomicrograph (original magnification x30; H-E stain) shows one of the cystic lesions produced by bronchiolar dilatation. Note the surrounding paracicatricial air-space enlargement (**).

View larger version (162K): [in a new window]   Figure 12c.  PLCH in a 31-year-old man with cough, anorexia, weight loss, and a 15 pack-year history of cigarette smoking. (a) High-resolution CT scan (lung window) shows nodules and thick- and thin-walled cysts. Some nodules exhibit a centrilobular distribution. (b) Low-power photomicrograph (original magnification, x12; H-E stain) demonstrates a cellular nodule with stellate peripheral extensions into adjacent alveolar walls. (c) Low-power photomicrograph (original magnification x30; H-E stain) shows one of the cystic lesions produced by bronchiolar dilatation. Note the surrounding paracicatricial air-space enlargement (**).

View larger version (96K): [in a new window]   Figure 13a.  PLCH in a 54-year-old woman with a history of cigarette smoking, cough, and dyspnea. Unenhanced CT scans (lung window; a obtained at a higher level than b) show numerous bilateral irregular lung nodules, most numerous in the upper lung zones.

View larger version (119K): [in a new window]   Figure 13b.  PLCH in a 54-year-old woman with a history of cigarette smoking, cough, and dyspnea. Unenhanced CT scans (lung window; a obtained at a higher level than b) show numerous bilateral irregular lung nodules, most numerous in the upper lung zones.

View larger version (123K): [in a new window]   Figure 14a.  PLCH in a 49-year-old woman with a history of cigarette smoking, increasing cough, and new onset of dyspnea. High-resolution CT scans (lung window) show bilateral irregular nodules and cysts predominantly distributed in the upper (a) and middle (b) lung zones with relative sparing of the lung bases (c). Note the irregular nodules that follow the course of an upper lobe bronchiole (arrow in a), an imaging feature that correlates with recent demonstrations of three-dimensional morphology of the PLCH lesion (see Fig 5) (40).

View larger version (124K): [in a new window]   Figure 14b.  PLCH in a 49-year-old woman with a history of cigarette smoking, increasing cough, and new onset of dyspnea. High-resolution CT scans (lung window) show bilateral irregular nodules and cysts predominantly distributed in the upper (a) and middle (b) lung zones with relative sparing of the lung bases (c). Note the irregular nodules that follow the course of an upper lobe bronchiole (arrow in a), an imaging feature that correlates with recent demonstrations of three-dimensional morphology of the PLCH lesion (see Fig 5) (40).

View larger version (78K): [in a new window]   Figure 14c.  PLCH in a 49-year-old woman with a history of cigarette smoking, increasing cough, and new onset of dyspnea. High-resolution CT scans (lung window) show bilateral irregular nodules and cysts predominantly distributed in the upper (a) and middle (b) lung zones with relative sparing of the lung bases (c). Note the irregular nodules that follow the course of an upper lobe bronchiole (arrow in a), an imaging feature that correlates with recent demonstrations of three-dimensional morphology of the PLCH lesion (see Fig 5) (40).

View larger version (147K): [in a new window]   Figure 15a.  PLCH in a 30-year-old man with a history of cigarette smoking, recent fatigue, and malaise. The patient worked as a welder and sandblaster and was referred with a clinical and radiographic diagnosis of "miliary tuberculosis." High-resolution CT scans (lung window) show irregular nodules and thick- (a) and thin-walled (b) cysts with relative sparing of the lung bases (c). Note the irregular morphology of some of the cystic lesions.

View larger version (127K): [in a new window]   Figure 15b.  PLCH in a 30-year-old man with a history of cigarette smoking, recent fatigue, and malaise. The patient worked as a welder and sandblaster and was referred with a clinical and radiographic diagnosis of "miliary tuberculosis." High-resolution CT scans (lung window) show irregular nodules and thick- (a) and thin-walled (b) cysts with relative sparing of the lung bases (c). Note the irregular morphology of some of the cystic lesions.

View larger version (121K): [in a new window]   Figure 15c.  PLCH in a 30-year-old man with a history of cigarette smoking, recent fatigue, and malaise. The patient worked as a welder and sandblaster and was referred with a clinical and radiographic diagnosis of "miliary tuberculosis." High-resolution CT scans (lung window) show irregular nodules and thick- (a) and thin-walled (b) cysts with relative sparing of the lung bases (c). Note the irregular morphology of some of the cystic lesions.

View larger version (119K): [in a new window]   Figure 16.  PLCH in a 34-year-old woman with cough, fatigue, and dyspnea. High-resolution CT scan (lung window) demonstrates a small right pneumothorax and bilateral irregular cysts, which vary in size and configuration. Some cysts appear to coalesce into larger, more irregular structures with bizarre shapes.

View larger version (118K): [in a new window]   Figure 17.  PLCH in a 47-year-old man with a history of smoking and worsening dyspnea. High-resolution CT scan (lung window) demonstrates predominantly thin-walled cysts of variable size and shape and a left pneumothorax.

View larger version (118K): [in a new window]   Figure 18a.  PLCH in a 52-year-old physician with a long history of cigarette smoking and worsening cough. (a-c) High-resolution CT scans (lung window; obtained at cranial to caudal levels) at the time of diagnosis show nodules and cysts that predominantly affect the upper and middle lung zones. (d-f) High-resolution CT scans (lung window; obtained at cranial to caudal levels) taken 3 years following cessation of smoking show marked improvement with near resolution of previously seen nodular and cystic lesions and evidence of centrilobular emphysema.

View larger version (121K): [in a new window]   Figure 18b.  PLCH in a 52-year-old physician with a long history of cigarette smoking and worsening cough. (a-c) High-resolution CT scans (lung window; obtained at cranial to caudal levels) at the time of diagnosis show nodules and cysts that predominantly affect the upper and middle lung zones. (d-f) High-resolution CT scans (lung window; obtained at cranial to caudal levels) taken 3 years following cessation of smoking show marked improvement with near resolution of previously seen nodular and cystic lesions and evidence of centrilobular emphysema.

View larger version (113K): [in a new window]   Figure 18c.  PLCH in a 52-year-old physician with a long history of cigarette smoking and worsening cough. (a-c) High-resolution CT scans (lung window; obtained at cranial to caudal levels) at the time of diagnosis show nodules and cysts that predominantly affect the upper and middle lung zones. (d-f) High-resolution CT scans (lung window; obtained at cranial to caudal levels) taken 3 years following cessation of smoking show marked improvement with near resolution of previously seen nodular and cystic lesions and evidence of centrilobular emphysema.

View larger version (115K): [in a new window]   Figure 18d.  PLCH in a 52-year-old physician with a long history of cigarette smoking and worsening cough. (a-c) High-resolution CT scans (lung window; obtained at cranial to caudal levels) at the time of diagnosis show nodules and cysts that predominantly affect the upper and middle lung zones. (d-f) High-resolution CT scans (lung window; obtained at cranial to caudal levels) taken 3 years following cessation of smoking show marked improvement with near resolution of previously seen nodular and cystic lesions and evidence of centrilobular emphysema.

View larger version (122K): [in a new window]   Figure 18e.  PLCH in a 52-year-old physician with a long history of cigarette smoking and worsening cough. (a-c) High-resolution CT scans (lung window; obtained at cranial to caudal levels) at the time of diagnosis show nodules and cysts that predominantly affect the upper and middle lung zones. (d-f) High-resolution CT scans (lung window; obtained at cranial to caudal levels) taken 3 years following cessation of smoking show marked improvement with near resolution of previously seen nodular and cystic lesions and evidence of centrilobular emphysema.

View larger version (123K): [in a new window]   Figure 18f.  PLCH in a 52-year-old physician with a long history of cigarette smoking and worsening cough. (a-c) High-resolution CT scans (lung window; obtained at cranial to caudal levels) at the time of diagnosis show nodules and cysts that predominantly affect the upper and middle lung zones. (d-f) High-resolution CT scans (lung window; obtained at cranial to caudal levels) taken 3 years following cessation of smoking show marked improvement with near resolution of previously seen nodular and cystic lesions and evidence of centrilobular emphysema.