—  SPECIALTY CONFERENCE HANDOUT  —

Pulmonary Pathology
Wednesday, February 15, 2006 - 7:30 PM
Centennial I




Moderator:

Andrew Churg
University of British Columbia Health Science Center
Vancouver, BC, Canada


Disclosure: The speakers have indicated they have nothing to disclose.


Clinical histories are printed below.
Click on the case numbers for text and references of each case.
Click on each slide thumbnail image for an enlarged view

Case 1

Submitted by:
Kevin O. Leslie
Mayo Clinic
Scottsdale, AZ

Clinical Summary:

A 36 year old married Caucasian woman, with systemic lupus erythematosus for 20 years, presented with symptomatic depression and suicidal thoughts. The patient's clinical course had been marked with episodes of hypoxia and a variably severe skin rash. Her pulmonary disease and skin manifestations responded inconsistently to systemic corticosteroid therapy during flare-ups, in part due to patient compliance issues. During the final hospitalization the patient's pulmonary status declined progressively and she expired from cardiopulmonary failure. An autopsy was performed. (Autopsy material courtesy of Thomas V. Colby, MD)



Case 1 - Figure 1 - Scanning magnification photomicrograph of the H&E stained section of lung showing thick-walled pulmonary arteries and a bone marrow embolus (likely an agonal event).

Case 1 - Figure 2 - Closer inspection of the arteries shows medial thickening.

Case 1 - Figure 3 - Concentric luminal compromise



Case 1 - Figure 4 - Nicely accentuated by an elastic tissue stain (EVG)

Case 1 - Figure 5 - Eccentric subendothelial lesions are also highlighted by the EVG stain

Case 1 - Figure 6 - Two plexiform lesions (PLX), each present adjacent to a pulmonary artery branch, are present in this photomicrograph. Dilated blood-filled thin-walled vascular spaces--so called angiomatoid lesions (dark arrows), can be seen surrounding the plexiform lesion at the top of the photograph.



Case 1 - Figure 7 - A transverse section through a plexiform lesion nicely illustrates the relationship of these complex endovascular lesions to the parent artery.

Case 1 - Figure 8 - Note the thin muscle layer (sm) present above and below the lesion, and the apparent focal nature of the process. The EVG stain outlines an attenuated elastica.



Case 1 - Figure 9 - The EVG stain highlights the relationship of the angiomatoid and plexiform lesions to the parent artery. Note the relative absence of a well-defined elastic lamina in these lesions.

Case 1 - Figure 10 - The triumvirate of pulmonary artery (PA) branch, plexiform lesion (PLX), and angiomatoid (dilatation) lesion (arrows) is well-illustrated in this photomicrograph.




Case 2

Submitted by:
Julia Flint
University of British Columbia
Vancouver, BC, Canada

Clinical Summary:

This 62 year old woman was admitted to hospital with increasing shortness of breath. She had complaints of exertional dyspnea and fatigue that had begun about a year ago. She had questionable Raynaud's phenomenon. She had been a one pack per day smoker from age 18 to 54 and she had suffered from hypertension. The chest CT scan showed no evidence of pulmonary embolus and it showed prominent bronchial circulation. A mild/moderate pericardial effusion was detected as well as small bilateral pleural effusions. Mild diffuse septal thickening was noted throughout both lungs. The right side of the heart was markedly enlarged. The pulmonary arteries were also enlarged with the main PA measuring 3.2 cm.

She had a right sided cardiac catheterization done which showed the pulmonary artery pressure to be 78/31mmHg with a mean of 47 mmHg. A nitric oxide challenge was performed together with coronary artery angiography. The conclusion was reached that she was suffering from pulmonary hypertension with an unclear response to nitric oxide challenge. She suffered a sudden cardiac arrest and she died in hospital.



Case 2 - Figure 1 - Pulmonary vein showing almost completely obstructive intimal fibrosis.

Case 2 - Figure 2 - Elastic stain highlights almost complete venous occlusion and interstitial thickening around the vein.

Case 2 - Figure 3 - Elastic stain with an arterialized pulmonary vein on the right side showing obstructive intimal fibrosis and on the left hand side fibrous thickening of the interlobular septum.



Case 2 - Figure 4 - Elastic stain of pulmonary vein showing arterialization with the medial coat of smooth muscle bounded by internal and external elastic laminae.

Case 2 - Figure 5 - Area of peripheral interstitial fibrosis.




Case 3

Submitted by:
Joseph F. Tomashefski
Case Western Reserve University
Cleveland, Ohio

Clinical Summary:

A 40-year old African-American woman with sickle cell disease and frequent sickle cell crises died 2 weeks after hospitalization for Enterococcus cloacae sepsis. Her hospital course included respiratory failure requiring supplemental oxygen and mechanical ventilation. Echocardiogram revealed an elevated pulmonary artery pressure of 69 mm Hg. The patient was taking hydromorphone and methadone, as well as multiple other medications for pain control, and was on a patient-controlled anesthesia pump.



Case 3 - Figure 1 - H&E, low magnification

Case 3 - Figure 2 - H&E, intermediate magnification

Case 3 - Figure 3 - H&E, polarized light



Case 3 - Figure 4 - Mucicarmine stain (left panel), Movat stain (right panel)(40X magnification, both panels)

Case 3 - Figure 5 - Congo red stain, intermediate magnification

Case 3 - Figure 6 - Gomorri methenamine silver stain (left panel), Movat stain (right panel) 40X magnification, both panels)




Case 4

Submitted by:
Henry D. Tazelaar
Mayo Clinic
Scottdsdale, AZ

Clinical Summary:

A 42 year old man presented with hemoptysis. He had a history of bilateral pleural effusions and at one point in time had received a diagnosis of sclerosing mediastinits. Two previous lung biopsies had shown pleural fibrosis. A bronchoscopy and pulmonary angiogram failed to show a source of bleeding. While in the hospital he died and underwent a thorax only autopsy. The submitting pathologist stated that both the pleura and pericardium were thickened.



Case 4 - Figure 1 - Low power of bronchus with anastomosing vascular spaces.

Case 4 - Figure 2 - Medium power of bronchial wall and adjacent lung with spongy vascularity.

Case 4 - Figure 3 - Medium power highlighting compact spindle cells along spaces.



Case 4 - Figure 4 - Compact spindle cells with bland elongate nuclei.

Case 4 - Figure 5 - Actin stain.




Case 5

Submitted by:
Andrew Churg
University of British Columbia
Vancouver, BC, Canada

Clinical Summary:

A 56 year old woman presented with shortness of breath which had been steadily increasing for the past 2 years. CT scan on admission showed irregular consolidation superimposed on ground glass opacities. In the past she had been admitted for what was thought to be pneumonia on several occasions and a CT scan 2 years before admission showed Abilateral airspace disease@. The patient had a history of chronic renal failure requiring dialysis, and a diagnosis of pauci-immune cresentic glomerulonephritis made 4 years before admission. A lung biopsy was performed. After the lung biopsy, serum ANCA was obtained; this was positive for p-ANCA ( myeloperoxidase). (Case courtesy Dr. Angus Kirby, Regina, Saskatchewan)



Case 5 - Figure 1 - CT scan from current admission showing areas of consolidation on a background of ground glass infiltrates

Case 5 - Figure 2 - Low power view of surgical lung biopsy. Note diffuse interstitial fibrosis and evidence of chronic hemorrhage.

Case 5 - Figure 3 - Higher power view of surgical lung biopsy. Hemosiderin deposition is better seen.



Case 5 - Figure 4 - Iron and calcium-encrusted elastica in a small pulmonary vessel. This finding is typical of chronic hemorrhage.

Case 5 - Figure 5 - Higher power view of another vessel showing the encrusted elastica.