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An elderly woman with dyspnoea and bronchorrhoea

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Q1: What is your diagnosis?

Histological examination of a transbronchial biopsy specimen revealed bronchioalveolar carcinoma of mucinous type, with extensive vascular invasion.

Q2: What other disorders may show a diffuse alveolar pattern?

Diffuse alveolar radiographic pattern may develop either acutely or as a chronic process. The major causes of both presentations are listed in box 1.

Box 1: Disorders associated with diffuse alveolar pattern

Acute
  • Pulmonary oedema.

  • Pneumonia.

  • Respiratory distress.

  • Aspiration.

  • Pulmonary haemorrhage.

  • Allergic bronchopulmonary aspergillosis.

  • Leukaemic infiltrates.

Chronic
  • Sarcoidosis.

  • Tuberculosis.

  • Fungal infections.

  • Bronchioloalveolar carcinoma.

  • Lymphoma.

  • Alveolar proteinosis.

Q3: What are other causes of bronchorrhoea?

Different volumes of daily sputum have been used to define excessive sputum production.1 Some of its causes are depicted in box 2. Bronchorrhoea is usually accepted as the production of >100 ml/day of sputum.

Box 2: Causes of excessive sputum production

  • Postnasal drip syndrome.

  • Asthma.

  • Gastroesophageal reflux disease.

  • Bronchitis.

  • Bronchiectasis.

  • Left ventricular failure.

  • Diffuse panbronchiolitis.

  • Bronchioloalveolar carcinoma.

  • Cystic fibrosis.

Discussion

Bronchioloalveolar carcinoma is one of the four recognised subtypes of lung adenocarcinoma. The dramatical increase in the incidence of lung adenocarcinoma in the last decade, being in some series the most frequent histological type among all lung malignancies,2 seems to be mostly due to the raising incidence of bronchioloalveolar carcinoma.

Its distinctive pathological feature is growth along alveolar septae without distortion of pulmonary interstitium. Three histopathological subtypes have been described: mucinous, non-mucinous, and sclerotic.

Prior pulmonary lesions, some professional exposures, cigarette smoking, and even a viral agent have been proposed as risk factors for developing bronchioloalveolar carcinoma.3

Males and females are equally affected. Patients may be asymptomatic in up to half of cases. Clinical symptoms include cough, haemoptysis, chest pain, dyspnoea, and weight loss. Two characteristic features, both present in the case under discussion, are large volume bronchorrhoea and refractory hypoxaemia caused by intrapulmonary shunting.

Radiographic patterns include solitary nodules or masses, localised or diffuse consolidation, and diffuse nodules.4In differential diagnosis, benign and malignant neoplasms (including metastatic disease), lobar pneumonia, congestive heart failure, alveolar haemorrhage, and alveolar proteinosis must be considered. Prognosis is usually poor,5 and it has been correlated with the presence or absence of symptoms, tumour extension, and histological type.

The clincal course of this patient was rapidly fatal, and she died on the 11th hospital day after two episodes of massive haemoptysis. Necropsy confirmed the diagnosis and excluded any other primary neoplasm. This was an important finding, as several adenocarcinomas may show pulmonary metastases with histological pictures indistinguishable from primary bronchioloalveolar carcinoma.6

Final diagnosis

Bronchioloalveolar carcinoma.

Learning points

  • The incidence of bronchioloalveolar carcinoma and lung adenocarcinoma have markedly increased in the last decade.

  • A non-resolving consolidative pneumonia, despite correct treatment in an adult with normal immunity, must raise the suspicion of bronchioloalveolar carcinoma.

  • Diagnosis of primary bronchioloalveolar carcinoma can only be made when other extrathoracic primary adenocarcinomas have been excluded.

References

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