Langerhans Cell Histiocytosis Imaging
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Langerhans cell histiocytosis (LCH) describes a group of syndromes that share the common pathologic feature of infiltration of involved tissues by Langerhans cells. Typically, the skeletal system is involved, with a characteristic lytic bone lesion form that occurs in young children or a more acute disseminated form that occurs in infants.
Pulmonary involvement is not unusual in systemic forms of LCH, but symptoms are rarely a prominent feature. [1, 2] Pulmonary Langerhans cell histiocytosis is considered a reactive proliferation of dendritic Langerhans cells to chronic tobacco-derived plant proteins resulting from incomplete combustion, but it can also occur as a tumor-like systemic disease in children. [3] CT scans of eosinophilic granuloma are depicted in the images below.
Localized pulmonary LCH (also termed pulmonary eosinophilic granuloma) is a rare pulmonary disease that occurs predominantly in young adults. The precise incidence and prevalence of pulmonary LCH are unknown, although studies of lung biopsy specimens from patients with interstitial lung disease identified pulmonary LCH in only 5%.
A confident diagnosis of pulmonary LCH often can be made based on the patient’s age, smoking history, and characteristic high-resolution computed tomography (HRCT) scan findings, especially if patients are followed without treatment.
Definitive diagnosis, if necessary, can be made by identification of Langerhans cell granulomas in lung biopsy samples acquired by video-assisted thoracoscopy. Biopsy sites are selected on the basis of HRCT scan findings. [4] Transbronchial biopsy has a low diagnostic yield (10-40%) because of the patchy nature of the disease and the small amounts of tissue obtained.
Treatment consists of smoking cessation, which stabilizes symptoms in most patients. Corticosteroids are used in progressive or systemic disease. Cytotoxic agents (eg, cyclophosphamide) can be employed for patients who do not respond to smoking cessation and steroids. No treatment has been confirmed to be useful, and no double-blind therapeutic trials have been reported. Lung transplantation also has been performed for treatment of LCH.
In patients with suggested eosinophilic granuloma, obtain a chest radiograph and a chest HRCT scan. [1, 4, 5]
The typical HRCT pattern of pulmonary Langerhans cell histiocytosis includes small poorly limited nodules, cavitated nodules, and thick- and thin-walled cysts. In rare cases, HRCT allows the disease to be diagnosed before the development of cysts. [2, 6, 7, 8] In HRCT scanning, the small star-like scars can still be detected even after complete cessation of tobacco smoking. [3]
Recent research cites the usefulness of positron emission tomography (PET)/CT scanning. [9, 10]
Chest radiographs are normal in fewer than 10% of patients. Usually, diffuse reticulonodularity (3- to 10-mm nodules that may cavitate) is observed in a symmetrical pattern, predominantly in the upper and middle lobes (bases tend to be spared). [11]
Lung volumes are generally preserved, or even increased, in contrast to most other pulmonary infiltrative diseases. [4] Associated pneumothorax is found in 15-25% of patients, and pleural effusion is rare.
In the late stage, diffuse cysts may be found that spare only the costophrenic angles.
On chest HRCT scans, the distribution of the disease is similar to that seen on chest radiography, with an upper lobe predominance (see the images below). [2, 7, 8] Findings include the following:
Centrilobular opacities
Small nodules (1-5 mm, may be up to 1.5 cm)
Cystic cavitation of small nodules; this is not readily visible on plain radiographs [4]
Cysts (initially thick walled, typically progressing to thin walled over time)
Some authors believe that nodules do not cavitate and cysts represent a paracicatricial emphysematous change adjacent to nodules. Cysts usually are smaller than 10 mm, although cysts larger than 10 mm are found in more than 50% of patients. Cyst walls usually are thin (less than 1 mm) but can vary, and the cysts are not necessarily round; they may be bilobed or branching. The intervening lung parenchyma appears normal. The extent of cystic involvement seen on HRCT scans has been correlated with the degree of lung function impairment. [12]
In the early stages, only nodules may be seen. In the late stages, diffuse cysts may be seen, with no nodules evident (approximately 20% of patients). Late-stage disease may be indistinguishable from lymphangiomyomatosis; however, sparing of the costophrenic angles suggests the diagnosis of eosinophilic granuloma. Mediastinal adenopathy has been described in some series but usually is uncommon. Other authors report mediastinal adenopathy in as many as 30% of patients. [13, 14]
A diagnosis of pulmonary eosinophilic granuloma can be established with a high degree of confidence when small nodules and cysts are seen in a young adult patient with a history of smoking. [15, 16]
On CT scans of 40 patients with Langerhans cell histiocytosis, 25 patients were found to have cysts involving upper lung zones with costophrenic sparing, 9 had a micronodular pattern in the middle-upper zone, and 6 had a combination of the 2 radiologic patterns. Pulmonary hypertension was seen in 4 patients. [6]
Gallium-67 scans usually are negative.
Bano S, Chaudhary V, Narula MK, Anand R, Venkatesan B, Mandal S, et al. Pulmonary Langerhans cell histiocytosis in children: a spectrum of radiologic findings. Eur J Radiol. 2014 Jan. 83 (1):47-56. [Medline].
Kim HJ, Lee KS, Johkoh T, Tomiyama N, Lee HY, Han J, et al. Pulmonary Langerhans cell histiocytosis in adults: high-resolution CT-pathology comparisons and evolutional changes at CT. Eur Radiol. 2011 Jul. 21 (7):1406-15. [Medline].
Popper HH. [Pulmonary Langerhans cell histiocytosis]. Pathologe. 2015 Sep. 36 (5):451-7. [Medline].
Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur Respir J. 2006 Jun. 27(6):1272-85. [Medline]. [Full Text].
Vrielynck S, Mamou-Mani T, Emond S, Scheinmann P, Brunelle F, de Blic J. Diagnostic value of high-resolution CT in the evaluation of chronic infiltrative lung disease in children. AJR Am J Roentgenol. 2008 Sep. 191(3):914-20. [Medline].
Martin I, Ballester M, Ruiz Y, Llatjós R, Alarza F, Molina M. Presentation of pulmonary Langerhans cell histiocytosis before the development of lung cysts. Respirol Case Rep. 2013 Dec. 1 (2):34-5. [Medline].
Tazi A, Marc K, Dominique S, de Bazelaire C, Crestani B, Chinet T, et al. Serial computed tomography and lung function testing in pulmonary Langerhans’ cell histiocytosis. Eur Respir J. 2012 Oct. 40 (4):905-12. [Medline].
Abbritti M, Mazzei MA, Bargagli E, Refini RM, Penza F, Perari MG, et al. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis. Eur J Radiol. 2012 Aug. 81 (8):1907-12. [Medline].
Lee HJ, Ahn BC, Lee SW, Lee J. The usefulness of F-18 fluorodeoxyglucose positron emission tomography/computed tomography in patients with Langerhans cell histiocytosis. Ann Nucl Med. 2012 Nov. 26(9):730-7. [Medline].
Hansen NJ, Hankins JH. Pulmonary langerhans cell histiocytosis: PET/CT for initial workup and treatment response evaluation. Clin Nucl Med. 2015 Feb. 40 (2):153-5. [Medline].
Sundar KM, Gosselin MV, Chung HL, Cahill BC. Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology, radiology, and clinical evolution of disease. Chest. 2003 May. 123(5):1673-83. [Medline]. [Full Text].
Canuet M, Kessler R, Jeung MY, Métivier AC, Chaouat A, Weitzenblum E. Correlation between high-resolution computed tomography findings and lung function in pulmonary Langerhans cell histiocytosis. Respiration. 2007. 74(6):640-6. [Medline].
Brauner MW, Grenier P, Tijani K. Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology. 1997 Aug. 204(2):497-502. [Medline].
Colby TV, Swensen SJ. Anatomic distribution and histopathologic patterns in diffuse lung disease: correlation with HRCT [published erratum appears in J Thorac Imaging 1996 Spring;11(2):163]. J Thorac Imaging. 1996 Winter. 11(1):1-26. [Medline].
Bonelli FS, Hartman TE, Swensen SJ. Accuracy of high-resolution CT in diagnosing lung diseases. AJR Am J Roentgenol. 1998 Jun. 170(6):1507-12. [Medline].
Hartman TE, Tazelaar HD, Swensen SJ. Cigarette smoking: CT and pathologic findings of associated pulmonary diseases. Radiographics. 1997 Mar-Apr. 17(2):377-90. [Medline].
Scott C Williams, MD Section Chief, Nuclear Medicine Associate Attending Radiologist, Advanced Radiology Consultants, Bridgeport Hospital
Scott C Williams, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Roentgen Ray Society, Radiological Society of North America
Disclosure: Nothing to disclose.
Matthew D Gilman, MD Thoracic Radiologist, Department of Radiology, Massachusetts General Hospital
Matthew D Gilman, MD is a member of the following medical societies: American Roentgen Ray Society
Disclosure: Nothing to disclose.
Bernard D Coombs, MB, ChB, PhD Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
Eugene C Lin, MD Attending Radiologist, Teaching Coordinator for Cardiac Imaging, Radiology Residency Program, Virginia Mason Medical Center; Clinical Assistant Professor of Radiology, University of Washington School of Medicine
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, Society of Nuclear Medicine and Molecular Imaging
Disclosure: Nothing to disclose.
Jeffrey A Miller, MD Associate Adjunct Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey-New Jersey Medical School; Faculty, Department of Radiology, Veterans Affairs of New Jersey Health Care System
Jeffrey A Miller, MD is a member of the following medical societies: American Roentgen Ray Society, Radiology Alliance for Health Services Research, Society of Thoracic Radiology
Disclosure: Nothing to disclose.
Langerhans Cell Histiocytosis Imaging
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