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Lymphangitic Carcinomatosis

Progressive hypoxaemic respiratory failure due to inflammation and obstruction of pulmonary lymphatics secondary to infiltration of malignant cells.

Lymphangitic carcinomatosis is distinct from (but related to) pulmonary tumour embolism, which occurs due to haematogenous embolism of tumour cells into pulmonary vasculature and leads to greater pulmonary hypertension and right heart failure.

Epidemiology and Risk Factors

Pathophysiology

Aetiology

Assessment

Days to weeks history of:

  • Progressive dyspnoea
  • Pleuritic chest pain
  • Cough
  • Haemoptysis
  • Weight loss
  • Fever
  • Tachypnoea

History

Common primary malignancies include:

Patients will usually have a diagnosed malignancy, although on rare occasions lymphangitic carcinomatosis may be the primary presentation.

  • Breast
  • Lung
  • Gastric
  • Prostate
  • Pancreatic
  • Colon
  • Cervical
  • Uterine

Examination

Investigations

Bedside:

Laboratory:

Imaging:

  • CXR
    Normal in 30-50% in early stages, however may demonstrate:
    • Interstitial oedema
    • Pleural effusions
  • CT chest
    • Interstitial oedema
    • Thickening of interlobular septa
    • Preservation of lung architecture
  • PET C Highly sensitive.

Other:

Diagnostic Approach and DDx

Management

  • Treat underlying malignancy
  • Oxygen
  • Consider mechanical ventilation
    Depending on prognosis.
  • Consider steroids

Resuscitation:

Specific therapy:

  • Pharmacological
  • Procedural
  • Physical

Supportive care:

Disposition:

Preventative:

Marginal and Ineffective Therapies

Anaesthetic Considerations

Complications

Prognosis

  • Typically indicates end-stage malignancy, with survival estimates ~6 months

Key Studies


References

  1. Ak AK, Mantri SN. Lymphangitic Carcinomatosis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 Dec 29].