eMedicine Specialties > Radiology > Chest

Lymphangitic Carcinomatosis

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

Introduction

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.

Presentation

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.

Differential Diagnoses

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

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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