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Lymphangitic Carcinomatosis: Imaging

Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad National Guard Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia
Coauthor(s): Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute; Carolyn M Allen, MB, BCh, MRCP, FRCR, CCST, Consultant Radiologist, Department of Clinical Radiology, North Manchester General Hospital, UK
Contributor Information and Disclosures

Updated: Feb 7, 2008

Radiography

Findings

On radiographs, LC appears as reticular or reticulonodular opacification, often with associated septal lines (Kerley A and B lines), peribronchial cuffing, pleural effusions, and mediastinal and/or hilar lymphadenopathy (20-50% of cases).13,18

Degree of Confidence

Chest radiographic findings can be entirely normal. The accuracy of chest radiography is only 23%.

False Positives/Negatives

Chest radiographic findings are normal in most patients with LC. A false-positive diagnosis may occur with other interstitial lung diseases.On chest radiographs, the linear pattern of Kerley A and B lines has a variety of causes, the most common of which is hydrostatic interstitial edema. Besides LC, other causes include sarcoidosis and interstitial pneumonias. All these conditions have a similar appearance to congestive heart failure; thus, the differentiation is largely clinically based. LC and interstitial pneumonias are often asymmetrically distributed, a characteristic that may be useful in distinguishing these from pulmonary edema.

Computed Tomography

Findings

HRCT scan findings include the following14,15,19,20,21,22,23,24 :

  • Irregular, nodular, and/or smooth, interlobular septal thickening
  • Thickening of the fissures as a result of the involvement of the lymphatics concentrated in the subpleural interstitium
  • Preservation of normal parenchymal architecture at the level of the secondary pulmonary lobule
  • Peribronchovascular thickening
  • Centrilobular peribronchovascular thickening, which predominates over interlobular septal thickening in a minority of patients
  • Polygonal arcades or polygons with prominence of the centrilobular bronchovascular bundle in association with interlobular septal thickening (50%)
  • Mediastinal and/or hilar lymphadenopathy (30-50%)
  • Pleural effusions (30-50%)

Findings may be unilateral or bilateral, focal or diffuse, and symmetrical or asymmetrical.25 Focal, unilateral disease accounts for 50% of cases. This pattern is associated in particular with underlying bronchogenic carcinoma. All of the changes described above are often associated with nodular opacities.

Degree of Confidence

Although the appearance of LC on HRCT scans is nonspecific, the development of HRCT scan features in a symptomatic patient with an appropriate history of malignancy is highly suggestive of LC, and further investigation is usually not required.

False Positives/Negatives

LC may not be recognized in the presence of normal radiographic chest findings. HRCT scan findings of LC also are nonspecific and may be misinterpreted.

The differential diagnosis of LC includes sarcoidosis and other chronic interstitial lung diseases such as silicosis, coal worker's pneumoconiosis, extrinsic allergic alveolitis (hypersensitivity pneumonitis), and cryptogenic fibrosing alveolitis, as well as other neoplasms, such as lymphoma and Kaposi sarcoma. Most of these diagnoses can be excluded on the basis of the clinical findings alone. The relative absence of polygons and the presence of architectural distortion at the level of the secondary pulmonary lobule strongly suggest fibrosis rather than LC.

Furthermore, parenchymal involvement in sarcoidosis is typically more central and/or perihilar, as well as more bilaterally symmetrical, than it is in LC. LC never appears as honeycombing, and sarcoid granulomas are often subpleural.

Nuclear Imaging

Findings

Fragmented scintigraphic perfusion defects have been reported in LC, as have V/Q mismatches. However, these features are not reliable in the diagnosis of LC.2,26 Fluorodeoxyglucose positron emission tomography (FDG-PET) scanning is used in the diagnosis and characterization of primary or secondary lung neoplasms, but its value in the characterization of LC is not well documented.27,28

A 2006 study compared findings from FDG-PET scans with those from HRCT scans in 5 patients with pulmonary LC.29 The FDG-PET scan activity distribution appeared to be identical to abnormal lung areas (which could be segmental, lobar, or diffuse) observed on HRCT scans. In segmental LC, a linear or a hazy area of FDG uptake extending from the tumor could be seen. An earlier study in 7 patients showed similar results, with the intensity of FDG uptake in the diseased lung being significantly greater than in the normal, contralateral lung.30

See also the following related topic in Medscape:
CME PET Imaging Gaining Popularity as Diagnostic Tool in Lung Cancer

Degree of Confidence

Experience with FDG-PET in the characterization of LC is limited.

More on Lymphangitic Carcinomatosis

Overview: Lymphangitic Carcinomatosis
Imaging: Lymphangitic Carcinomatosis
Follow-up: Lymphangitic Carcinomatosis
Multimedia: Lymphangitic Carcinomatosis
References
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Further Reading

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Keywords

interstitial carcinoma, LC, pulmonary metastases, pulmonary lymphatics, adenocarcinomas, Kerley A lines, Kerley B lines

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Contributor Information and Disclosures

Author

Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP, Chairman of Medical Imaging, Professor of Radiology, NGHA, King Fahad National Guard Hospital, King Abdulaziz Medical City, Riyadh, Saudi Arabia
Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR, LRCP is a member of the following medical societies: American Institute of Ultrasound in Medicine, Radiological Society of North America, Royal College of Physicians, Royal College of Physicians and Surgeons of the United States, Royal College of Radiologists, and Royal College of Surgeons of England
Disclosure: Nothing to disclose.

Coauthor(s)

Sumaira MacDonald, MBChB, PhD, MRCP, FRCR, Lecturer, Sheffield University Medical School; Endovascular Fellow, Sheffield Vascular Institute
Sumaira MacDonald, MBChB, PhD, MRCP, FRCR is a member of the following medical societies: British Medical Association, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.

Carolyn M Allen, MB, BCh, MRCP, FRCR, CCST, Consultant Radiologist, Department of Clinical Radiology, North Manchester General Hospital, UK
Carolyn M Allen, MB, BCh, MRCP, FRCR, CCST is a member of the following medical societies: American Roentgen Ray Society
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey A Miller, MD, Associate Professor of Clinical Radiology, University of Medicine and Dentistry of New Jersey; Associate Chief of Service, Department of Radiology, Veterans Affairs of New Jersey Health Care System
Jeffrey A Miller, MD is a member of the following medical societies: North American Society for Cardiac Imaging, Society for Health Services Research in Radiology, and Society of Thoracic Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

W Richard Webb, MD, Chief of Thoracic Imaging, Professor, Department of Radiology, University of California at San Francisco
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center
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, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.

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