Lymphangitis carcinomatosa

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Lymphangitis carcinomatosis; Pulmonary lymphangitic carcinomatosis

Overview

Lymphangitis carcinomatosa is an inflammation of the lymph vessels secondary to a malignancy. Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829. The pathogenesis of lymphangitis carcinomatosa is characterized by the tumoral spread into the lymphatics, following hematogenous seeding of the lungs. The pathogenesis is also characterized by retrograde spread into lymphatics from the mediastinal and hilar lymph nodes in the lungs. Common causes of lymphangitis carcinomatosa include breast cancer (most common), lung cancer, colon cancer, stomach cancer, prostate cancer, cervical cancer, and thyroid cancer. Lymphangitis carcinomatosa is a rare disease. The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide. Lymphangitis carcinomatosa is more commonly observed among patients aged between 40 to 49 years old. Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa. On CT, characteristic findings of lymphangitis carcinomatosa include subpleural nodules, thickening on the interlobar fissures, pleural effusion, and hilar and mediastinal nodal enlargement (40-50%). The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology).

Historical Perspective

• Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829.

Classification

• There is no classification for lymphangitis carcinomatosa.

Pathophysiology

• The pathogenesis of lymphangitis carcinomatosa is characterized by the tumoral spread into the lymphatics, following hematogenous seeding of the lungs.
• The pathogenesis is also characterized by retrograde spread into lymphatics from the mediastinal and hilar lymph nodes in the lungs.
• There are no specific genetic mutations associated with the development of lymphangitis carcinomatosa.
• On gross pathology, characteristic findings of lymphangitis carcinomatosa include:

• No remarkable findings

• On microscopic histopathological analysis, characteristic findings of lymphangitis carcinomatosa include:

• Carcinoma in multiple the lymphatic channels

Causes

• Common causes of lymphangitis carcinomatosa include:
  • Breast cancer (most common)
  • Lung cancer
  • Colon cancer
  • Stomach cancer
  • Prostate cancer
  • Cervical cancer
  • Thyroid cancer

Differentiating Lymphangitis Carcinomatosa from Other Diseases

• Lymphangitis carcinomatosa must be differentiated from other diseases that cause thickened interlobular septae (on imaging finding), dyspnea, fatigue, and weight-loss, such as:

• Sarcoidosis
• Viral pneumonia
• Pulmonary edema
• Radiation pneumonitis
• Lymphocytic interstitial pneumonitis

Epidemiology and Demographics

• Lymphangitis carcinomatosa is a rare disease.
• The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide.

Age

• Patients of all age groups may develop lymphangitis carcinomatosa.

• Lymphangitis carcinomatosa is more commonly observed among patients aged 40–49 years old.
• Lymphangitis carcinomatosa is more commonly observed among middle aged adults.

Gender

• Females are more commonly affected with lymphangitis carcinomatosa than males.

Race

• There is no racial predilection for lymphangitis carcinomatosa.

Risk Factors

• Common risk factors in the development of lymphangitis carcinomatosa include:

• Personal history of cancer
• Preexistent malignant cancer

Natural History, Complications and Prognosis

• The majority of patients with lymphangitis carcinomatosa are symptomatic at the time of diagnosis.
• Early clinical features include dyspnea, fatigue, and weight-loss.
• If left untreated, patients with lymphangitis carcinomatosa may progress to develop acute respiratory failure.
• Common complications of lymphangitis carcinomatosa include:

• Pulmonary tumor embolism
• Pulmonary hypertension

• Prognosis is generally poor, and the mean survival rate of patients after diagnosis of lymphangitis carcinomatosa is approximately 6 months.

Diagnosis

Symptoms

• Lymphangitis carcinomatosa is usually asymptomatic.
• Symptoms of lymphangitis carcinomatosa may include the following:

• Hemoptysis
• Chronic coughing
• Chest pain
• Loss of appetite
• Fatigue

Physical Examination

• Patients with lymphangitis carcinomatosa usually appear pale and malnourished.
• Physical examination may be remarkable for:

Auscultation

• Present pleural friction rub
• Present egophony
• Crackling or bubbling noises
• Present whispered pectoriloquy
• Decreased/absent breath sounds

Percussion

• Dull percussion
• Reduced chest expansion

Laboratory Findings

• There are no specific laboratory findings associated with lymphangitis carcinomatosa.

Imaging Findings

• Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa.
• The most important feature about CT scan is the detection of peripheral and central changes.
• On CT, characteristic findings of lymphangitis carcinomatosa include:

• Subpleural nodules, and thickening on the interlobar fissures
• Pleural effusion
• Hilar and mediastinal nodal enlargement (40-50%)
• Relatively little destruction of overall lung architecture
• Involvement of the peripheral (interlobular septa) and central lymphatic system
• Distribution of changes is variable, but most are asymmetric and patchy
• Usually bilateral (may be unilateral especially in cases of lung and breast cancer)

Treatment

Medical Therapy

• The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology).

Surgery

• Surgery is not recommended for patients with lymphangitis carcinomatosa.

Prevention

• There are no primary preventive measures available for lymphangitis carcinomatosa.
• Once diagnosed and successfully treated, patients with lymphangitis carcinomatosa are followed-up periodically.
• Follow-up testing may include respiratory function tests and disease progression monitorization.