Epidemiology

• Presence of Lymphangitic Pattern in Patient with Known Malignancy: 70-80% probability of metastases being the cause

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Etiology

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Physiology

Lymphangitic Carcinomatosis is the Diffuse Infiltration and Obstruction of Pulmonary parenchymal Lymphatic Channels by Tumor

• Spread of Malignant Cells to Lung Lymphatics from Any of the Following
  • Obstructed Lymph Nodes
  • Hematogenous Spread
  • Previously Seeded Pleural Fluid

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Diagnosis

Chest X-Ray (CXR) (see Chest X-Ray)

• Findings
  • Diffuse Reticulonodular Infiltrates: often with linear markings radiating out from enlarged hilar or mediastinal nodes
  • Kerley B Lines: thickened interlobular septa (seen at periphery of lung) due to pulmonary lymphatic obstruction
  • Coexistent Pleural Effusion
    • Present in 30-50% of Cases
    • Effusion Occurs more commonly in breast cancer cases with lymphangitic lung mets than those with non-lymphangitic lung mets

High-Resolution Chest Computed Tomography (HRCT) (see Chest Computed Tomography)

• Findings
  • “Beaded chain” or “string of pearls” thickening of interlobular septa -> producing polygonal shapes (outlining the lobule)
    • Interlobular septa is usually irregular or nodular, but may be smoothly thickened
      • In contrast, in interstitial pulmonary edema, interlobular septa is usually smoothly thickened
    • Intervening parenchyma between the interlobular septa is typically normal (unlike in IPF, where it is usually distorted or honeycombed)
  • Centrilobular peribronchovascular thickening
  • Preservation of normal parenchymal architecture at the level of the secondary pulmonary lobule
  • Thickening of the fissures as a result of the involvement of the lymphatics concentrated in the subpleural interstitium
  • Peribronchovascular thickening
  • Lung Nodules
  • Pleural effusion (30-50% of cases)
    • Effusion occurs more commonly in breast cancer cases with lymphangitic lung mets than those with non-lymphangitic lung mets
  • Mediastinal and/or hilar lymphadenopathy (30-50% of cases)
  • Above findings may be unilateral or bilateral, focal or diffuse, and symmetrical or asymmetrical
    • Focal, unilateral lymphangitic disease occurs in 50% of cases
    • Focal, unilateral pattern is seen commonly in cases due to lung cancer

Sputum Cytology

• May be used to diagnose lymphangitic mets

Bronchoscopy (see Bronchoscopy)

–BAL is more sensitive than sputum cytology in diagnosis of lymphangitic mets – BAL may be diagnostic in lymphangitic carcinomatosis cases due to lymphoma – However, lymphocytosis >35% may also be seen in sarcoidosis, berylliosis, hypersensitivity pneumonitis, drug-induced ILD, LIP, breast cancer-related lymphangitic carcinmatosis, and infrequently, in lung cancer-related lymphangitic carcinmatosis cases) – Normal BAL lymphocyte percentage: <15% – BAL lymphocytosis is seen in about 50% of breast cancer cases with lymphangitic lung mets (Chest, 1992) – TBB: may be diagnostic – TBB use in this setting represents one of the few high yield uses of TBB in the diagnosis of interstitial lung disease – Cytology: immunohistochemical stains may be useful in identifying germ cell and breast cancers

PET Scan (see xxxx)

• 2005 Study: intensity of FDG uptake in the diseased lung is significantly greater than in the normal, contralateral lung [J Comput Assist Tomogr. May-Jun 2005;29(3):346-9]
• 2006 Study: comparing PET with HRCT scans in 5 patients with lymphangitic carcinomatosis [Clin Nucl Med. Nov 2006;31(11):673-8]
  • FDG-PET scan activity distribution was identical to segmental, lobar, or diffuse areas of lymphangitic disease seen on HRCT
  • In segmental lymphangitic carcinmatosis, linear or hazy FDG uptake extending from the tumor may be seen

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Clinical Manifestations

General Comments

• Asymptomatic: often

Pulmonary Manifestations

• Dyspnea (see Dyspnea)
  • Common
• Orthopnea (see Orthopnea)
  • Common
• Cough (see Cough)
  • Common: due to mucosal edema associated with airway lymphatic infiltration
• Chest Tightness (see Chest Pain)
  • Chest pain (late in course, with pleural involvement)
• Pleural Effusion (see Pleural Effusion-Exudative)
  • Occurs late in course

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Prognosis

• Presence of Bronchoalveolar Lavage (BAL) Lymphocytosis (range: 15-45% of total cells): portends better prognosis in breast cancer cases with lymphangitic mets (Chest, 1992)

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Treatment

• Treat Underlying Malignancy
• Steroids: may provide some symptomatic relief
  • May decrease inflammatory response to tumor cells in lung
• Opiates (see xxxx): may relieve dyspnea
  • Nebulized Morphine Sulfate: may be useful
• Oxygen: may relieve dyspnea
• Antitussives: as required

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