Pulmonary pathology

The lung is a forgotten organ in pathology, 'cause radiologists can diagnose much with high resolution CT (HRCT) and a bit of history. This article introduces the lung and discusses an approach to the lung.

Medical lung disease is dealt with in the medical lung disease article. Tumours of the lung are dealt with in lung tumours article. Lung cytopathology is dealt with in the pulmonary cytopathology article.

Lung specimens

• Ultrasound-guided needle core biopsy - for peripheral lesions.
• Transbronchial biopsy - for central lesions.
• "Open lung biopsy" - typically a video-assisted thoracic surgery (VATS) - done for diffuse lung diseases.
  • These specimens should be sectioned to create pieces with a large surface area; ideally, it should be one large piece per block, so one can appreciate the architecture.
• Lobectomy - usually for cancer.
• Pneumonectomy - usually for cancer.
• Explantation - in the context of lung transplantation - done for cystic fibrosis Idiopathic pulmonary fibrosis and other causes.

Basic approach

All lung pathology can be grouped into one of six categories (as per Leslie). The radiology directly correlates to the pathologic grouping, except that air space disease encompasses three pathologic categories (ALI, CCI, AFD).

Pathologic groups:

Lung pathology

Acute lung injury

Fibrosis

Chronic cellular infiltrates (ALI)

Alveolar filling defect (AFD)

Nodules

Near normal histology

Identification of the groups:

• Acute lung injury: hyaline membranes (very pink on H&E).
• Fibrosis = thick walls - pink on H&E.
• Chronic cellular infiltrates = inflammation (blue on H&E).
• Nodules = look at the history/radiology - should say mass or nodule.
• Alveolar filling defect = crap in the alveoli.
• Near normal = looks almost normal.

Radiologic groups:

Lung radiology

Air space disease

Interstitial disease

Mass/nodules

Near normal

Radiologic-pathologic correlation:

• Air space disease (radiologic).
  • Acute lung injury.
  • Chronic cellular infiltrates.
  • Alveolar filling defects.
• Interstitial disease (radiologic).
  • Fibrosis.
• Mass/nodules (radiologic).
  • Nodules.
• Near normal (radiologic).
  • Near normal histology.

Most of the things that come to pathology are in the mass/nodules category and lung tumours (discussed below). The other categories are dealt with in the medical lung disease article.

Anatomical approach:

• Pleura - thickening?
• Airspace - filling?
• Alveolar walls - thickening?
• Airways - inflammation?
• Vessels - thickening?

Normal histology

Cells

Common:

• Type I pneumocyte - cover most of the alveolar surface.
• Type II pneumocyte - stem cell, produce surfactant.
  • Typical location: "angle of alveolus".
• Macrophages.

Rare:

• Pulmonary neuroendocrine cells:
  • Histomorphology:
    • Single cells.
    • Small clusters ~ 6 cells ("neuroepithelial bodies").
  • Identified with immunostains:
    • Serotonin.
    • Bombesin.
    • Chromogranin A.
    • Synaptic vesicle 2.

Lung lobule

Lung lobule:

• Arterial vessels travels with the bronchus.
• Venules travel in the septae.

• Arterial vessels in the lung should be approximately the same size as its accompanying airway.

Memory device: arteries (which were once thought to contain air) are with the airway.

Small airways

The trip to the alveolus:

1. Membranous bronchiole.
2. Terminal bronchiole - dilation distal to this = emphysema.
3. Respiratory bronchiole.
4. Alveolar duct - dilated in ARDS.
5. Alveolus.

Anatomy - terms

• Bronchus = has cartilage.
• Bronchiole = non-cartilaginous airway.

Pathology terminology

• Siderophages = mononuclear phagocyte with hemosiderin.

• Hyaline membrane = glassy layering of an alveolus/small airways with material that is eosinophilic on H&E.
  • Entomology of Hyaline: "... of glass" (Greek).
  • Image: Hyaline membrane (path.upmc.edu).

• Bronchiolization = ciliated (respiratory) epithelium or goblet cells in (distal) air space.
  • Image: Bronchiolization (ucsf.edu).

• Smoker's macrophages = brown-pigmented macrophages - assoc. with smoking.

Detail articles

Malignancy - lung cancer

Main article: Lung cancer

This pretty much always comes to the pathologist.

Medical lung disease

Main article: Medical lung disease

There are separate articles for:

• Pneumonia.
• Diffuse lung diseases, e.g. usual interstitial pneumonia.
• Pulmonary hypertension.

Lung transplant pathology

Main article: Lung transplant pathology

Specific diagnoses

Bronchogenic cyst

General

• Benign.
• Usually in mediastinum or hilum.
• Fit into the bigger category of foregut cyst.

Microscopic

Features:

• Cyst lined by respiratory epithelium.

Images:

• Bronchogenic cyst (WC).
• Bronchogenic cyst (ctsnetjournals.org).

Pulmonary hamartoma

General

• Benign.

See also: Hamartoma.

Gross

• Well circumscribed lesion.

Microscopic

Features:

• Cartilage - key feature.
  • Single cells in lacunae surrounded by abundant matrix.
    • Paucicellular vis-a-vis malignant lesions.
• Fat (adipocytes) - key feature.
• Respiratory epithelium (columnar epithelium with cilia).

Notes:

• No nuclear atypia.

Images:

• Pulmonary hamartoma (studentdoctor.net).
• Lung hamartoma (path.utah.edu).
• Pulmonary hamartoma - low mag. (WC).
• Pulmonary hamartoma - high mag. (WC).

IHC

• S100 +ve - highlights the fat.

Malformations

Come in three flavours:

1. Congenital pulmonary airway malformation (CPAM).
2. Extralobar sequestration (ELS).
3. Intralobar sequestrations (ILS).

Congenital pulmonary airway malformation

• Previously known as congenital cystic adenomatoid malformation (CCAM).

General:

• Classified according to density:
  • Type I = mostly large cysts.
  • Type II = mostly small cysts.
  • Type III = solid mass.

Gross

• Cystic or solid mass.

Image:

• CPAM (humpath.com).

Microscopic

Features:

• Irregular cystic spaces with bronchial epithelium.
  • Bronchial epithelium = cilia, pseudostratified.

Extralobar sequestration

General:

• Typically not connected to airway tree/trachea.
• Blood supply arises from aorta, not the pulmonary artery.
• Mass lesion.
• Associated with other congenital anomalies. The most common are diaphragmatic abnormalities - including congenital diaphragmatic hernia.

Microscopic

Features:

• Abnormal airways:
  • Dilated irregularly shaped bronchi.
    • Wavy luminal contour/undulating contour (normal ~ round/ovoid).
  • Distal airways with ciliated epithelium.
• +/-Infection.
  • +/-Interstitial fibrosis due to inflammation.

Image:

• Extralobular sequestration - several images (sonoworld.com).

Intralobar sequestration

General:

• Classically identified due to recurrent infections or bronchiectasis.
• Intralobular sequestration can be considered a variant of ELS; it is like an ELS but surrounded by normal lung.

Bronchopulmonary dysplasia

• Abbreviated BPD.

General

• Developmental.
• Assoc. with prematurity.

Microscopic

Features:

• Large alveoli.
• Bronchiolar fibrosis.
• Interstitial fibrosis.

Images:

• BPD (pathologyoutlines.com).

Pulmonary infarct

• AKA lung infarct, lung infarction, pulmonary infarction.

General

• Uncommon because of the dual blood supply (systemic via the bronchial arteries, pulmonary via the pulmonary arteries).

Common causes:

• Pulmonary embolism.
• Sickle cell disease.

Gross

• Lung periphery, classically described as wedge-shaped.

Note:

• In a histologic section, the classic wedge-shaped infarct is triangular:
  • Base of triangle on the pleural aspect.
  • Point furthest from the pleura close to the compromised artery that lead to infarction.

Image:

• Pulmonary infarct (sciencephoto.com).

Microscopic

Features:

• Necrosis of alveolar walls - loss of nuclei.
• Alveolar hemorrhage.

Image:

• Pulmonary infarct - low mag. (WC).

See also

• Medical lung disease.
• Thyroid.
• Basics.
• Heart.

External links

• Pulmonary pathology (flickr.com).