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AUTHOR AND EDITOR INFORMATION

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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 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

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

Editors: 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; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; W Richard Webb, MD, Chief of Thoracic Imaging, Professor, Department of Radiology, University of California at San Francisco; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center

Author and Editor Disclosure

Synonyms and related keywords: interstitial carcinoma, LC, pulmonary metastases, pulmonary lymphatics, adenocarcinomas, Kerley A lines, Kerley B lines

INTRODUCTION

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Background

The lungs are one of the most common targets for metastatic disease. Most pulmonary metastases are nodular, but a significant minority is interstitial. Lymphangitic carcinomatosis (LC) refers to the diffuse infiltration and obstruction of pulmonary parenchymal lymphatic channels by tumor. Various neoplasms can cause lymphangitic carcinomatosis, but 80% are adenocarcinomas.1 The most common primary sites are the breasts, lungs, colon, and stomach.2, 3 Other sources include the pancreas, thyroid, cervix, prostate, and larynx.4, 5, 6, 7, 8 LC can also arise from choriocarcinoma, melanoma, or metastatic adenocarcinoma from an unknown primary cancer.

See also the following related topics in eMedicine:
Secondary Lung Tumors
Lung, Metastases

Pathophysiology

LC occurs as a result of the initial hematogenous spread of tumor to the lungs, with subsequent malignant invasion through the vessel wall into the pulmonary interstitium and lymphatics. Tumor then proliferates and easily spreads through these low-resistance channels. Less commonly, direct infiltration occurs as a result of contiguous mediastinal or hilar lymphadenopathy or an adjacent primary bronchogenic carcinoma.

Histopathologic examination reveals interstitial edema, tumor cells, and interstitial fibrosis secondary to a desmoplastic reaction as a result of tumor extension into adjacent pulmonary parenchyma. Metastatic adenocarcinoma accounts for 80% of cases.

Frequency

United States

LC represents 7% of all pulmonary metastases. The prevalence in postmortem studies is significantly higher than the incidence of radiologically detectable disease. Microscopic interstitial tumor invasion is seen in 56% of patients with pulmonary metastases.

Mortality/Morbidity

The prognosis for patients with LC is poor. Most patients survive only weeks or months.

Age

Most patients are middle-aged adults.

Anatomy

The pulmonary lymphatics are located in the interstitial components of the lung. These involve the axial (peribronchovascular and centrilobular) and peripheral (interlobular and subpleural) compartments.

Clinical Details

In a patient with a known malignancy, the usual presenting complaint is breathlessness. Occasionally, patients may have a dry cough or hemoptysis. Symptoms often precede the development of any radiographic abnormality.9, 10, 11

Preferred Examination

Plain chest radiography is the initial investigation of choice in patients presenting with any respiratory symptoms, and this examination is used in staging most cancers. However, the sensitivity of chest radiography in the detection of lymphangitic carcinomatosis is only approximately 25%.12, 13

In the appropriate clinical circumstances or when the chest radiographic findings are equivocal, the next investigation employs high-resolution computed tomography (HRCT) scanning.14, 15 Although the appearance of LC on HRCT scans is nonspecific, the observation of certain features in a symptomatic patient with an appropriate history of malignancy is highly suggestive of LC; in such instances, further investigation is generally not required (see CT Scan).

Fragmented scintigraphic perfusion defects have been reported in LC, as have ventilation-perfusion (V/Q) mismatches.16, 17 However, a diagnosis of LC cannot be reliably based on the presence of these features.

See also the following related topic in Medscape:
CME New Guidelines Issued on Lung Cancer Diagnosis and Management 

Limitations of Techniques

The radiographic features of LC are essentially nonspecific, with a diagnostic accuracy of 23%. Furthermore, chest radiographic findings are normal in as many as 50% of patients with histologic evidence of disease.

In comparison, HRCT scanning has higher sensitivity, although, as with radiography, the findings may not be diagnostic. The differential diagnosis includes other malignant conditions, such as lymphoma and Kaposi sarcoma, as well as benign entities, such as sarcoidosis.14

With LC, perfusion defects on a V/Q scan are nonspecific and are also described in various pulmonary pathologies, the most important of which is pulmonary embolism.


DIFFERENTIALS

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Kaposi Sarcoma, Thoracic

Other Problems to Be Considered

Kaposi sarcoma, pulmonary
Lymphoma, pulmonary
Sarcoidosis
Interstitial lung disease (ILD)
Pulmonary edema
Hypersensitivity pneumonitis

See also the following related topic in Medscape:
CME Interstitial Lung Disease and Pulmonary Hypertension


RADIOGRAPH

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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.


CT SCAN

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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 MEDICINE

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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.


INTERVENTION

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Medical/Legal Pitfalls

  • The mainstay of LC detection is HRCT scanning.
    • Although HRCT scanning is a sensitive technique, the findings may not be diagnostic.
    • LC must not be confused with other, perhaps curable, conditions, such as lymphoma and Kaposi sarcoma, or with benign entities, such as sarcoidosis.31

See also the Medscape Resource Center Medical Malpractice and Legal Issues.


ACKNOWLEDGMENTS

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Ramesh Chandra Raja, MBChB, FRCR, Consulting Staff, Department of Radiology, Rochdale Healthcare NHS Trust, assisted with this article.


MULTIMEDIA

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Media file 1:  Typical radiographic appearance of lymphangitic carcinomatosis in a patient with breathlessness. Diffuse, bilateral reticulonodular opacification with Kerley B lines is noted.
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Media type:  X-RAY

Media file 2:  Endoscopic image of the stomach shows a primary, ulcerating gastric carcinoma (same patient as in Image 1).
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Media type:  Image

Media file 3:  Fine reticular opacification with Kerley B lines and a tiny left pleural effusion are demonstrated in a patient with a lymphangitic spread of cervical carcinoma.
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Media type:  X-RAY

Media file 4:  High-resolution computed tomography scan demonstrates smooth and nodular interlobular septal thickening (arrow).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 5:  High-grade non–Hodgkin lymphoma mimics lymphangitic carcinomatosis. Note the diffuse interstitial pattern and a tiny left pleural effusion.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  X-RAY

Media file 6:  High-resolution computed tomography scan demonstrates irregularly thickened septa that have created polygonal arcades (arrow).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT

Media file 7:  Prominent axial interstitium with thickened bronchovascular bundles (solid arrow). Also note the thickening of the fissure secondary to the involvement of subpleural lymphatics (open arrow).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT


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Lymphangitic Carcinomatosis excerpt

Article Last Updated: Feb 7, 2008