—  SPECIALTY CONFERENCE HANDOUT  —

Pulmonary Pathology
Diffuse Lung Disease: Orphans and Rare Complications
Sunday, March 7, 2004 - 7:30 p.m.
Ballroom A




Moderator:

HENRY D. TAZELAAR
Mayo Clinic
Rochester, MN



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

THOMAS V. COLBY
Mayo Clinic
Scottsdale, AZ

Clinical Summary:

There are two fragments on the slide and they come from two different patients. The piece that shows the more dramatic changes is from a 38-year-old woman with increasing dyspnea, decreased diffusing capacity, and severe obstruction functionally. Radiologic studies showed emphysema and cystic change. The patient was a never smoker.

The other second tissue is from a woman who presented at age 31 with hemoptysis and cough. She developed progressive dyspnea and two years later underwent single lung transplant from a male donor. The explanted lung showed the same features as were present in the other specimen on your slide. One year after transplantation, she developed evidence of bronchiolitis obliterans and a year after that, she had refractory pneumothoraces and other complications that could not be managed and she died. This lung tissue is from the allograft taken at the autopsy.


Case 1 - Figure 1 - Low power of the allograft lung shows a few small cystic spaces with focal regions of hypercellularity in their walls.

Case 1 - Figure 2 - The hypercellularity seen in Figure 1 corresponds to a spindle cell proliferation.

Case 1 - Figure 3 - The spindle cell proliferations seen in figure 2 is HMB 45 positive.


Case 1 - Figure 4 - Classical lymphangiomyomatosis with large cystic spaces.

Case 1 - Figure 5 - The wall of the cysts in figure 4 shows spindle cells resembling smooth muscle cells.

Case 1 - Figure 6 - The spindle cells in Figure 5 show HMB 45 positivity with some polygonal cells more darkly staining.




Case 2

MARIE-CHRISTINE AUBRY
Mayo Clinic
Rochester, MN

Clinical Summary:

A 68 year-old man was investigated for recent persistent chest pain. He also complained of progressive shortness of breath and dry cough for several months. He had no significant past medical or familial history. He was a current smoker of 90 pack-years. His pulmonary function test showed mild restriction. Chest X-ray and CT Scan were performed and showed a spiculated peripheral mass in the left lower lobe. Bilateral fibrotic infiltrates were also noted. He underwent a left lower lobectomy, which was complicated by prolonged ventilator dependence, bronchopleural fistula and infection of muscle flap. He was eventually dismissed to hospice care and died 6 months following his surgery.



Case 2 - Figure 1 - Usual interstitial pneumonia. Interstitial pneumonia with heterogeneous appearance at low magnification, with alternating areas of normal lung, fibrosis and honeycomb change (H&E, 20x).

Case 2 - Figure 2 - Usual interstitial pneumonia. Interstitial inflammation and fibrosis is patchy, with fibrosis mainly comprised of collagen deposition (H&E, 100x).

Case 2 - Figure 3 - Usual interstitial pneumonia. Scattered foci comprised of proliferating fibroblasts in a myxoid background can also be seen (H&E, 200x).


Case 2 - Figure 4 - Usual interstitial pneumonia. Areas of honeycomb change are comprised of dilated airspaces, filled with mucin, and surrounded by fibrosis resulting in architectural distortion (H&E, 40x).

Case 2 - Figure 5 - Usual interstitial pneumonia. The dilated airspaces are frequently lined by bronchiolar epithelium but squamous metaplasia can also be seen (H&E, 200x).


Case 2 - Figure 6 - Squamous cell carcinoma. The mass is comprised of large anastomosing nests of cells with central necrosis, embedded in a fibrotic stroma (H&E, 40x).

Case 2 - Figure 7 - Squamous cell carcinoma. At high power, dyskeratosis and intercellular bridges are seen (H&E, 400x).




Case 3

ANDREW CHURG
University of British Columbia
Vancouver, BC, Canada

Clinical Summary:

A 34 yo male presented with a several month history of increasing shortness of breath. The shortness of breath was not precipitated by an acute illness. He was a nonsmoker. He had no pets at home. There was no recent travel history. He had worked for 10 years grinding saw blades at a small factory. CT scan showed diffuse ground glass infiltrates with small centrilobular nodules. Pulmonary function tests demonstrated a restrictive pattern with reduced diffusing capacity. A thoracoscopic lung biopsy was performed.



Case 3 - Figure 1 - Whole mount image showing sharply demarcated airway centered lesions.

Case 3 - Figure 2 - Lower power view showing interstitial inflammation and fibrosis centered around a respiratory bronchiole. Note intense lymphoid infiltrate. Characteristic airspace macrophages and giant cells can be seen.

Case 3 - Figure 3 - High power view showing macrophages and giant cells in airspaces. Note that some of the giant cells have phagocytized macrophages, a typical finding in hard metal disease.




Case 4

ANNA-LUISE KATZENSTEIN
SUNY Upstate Medical Center
Syracuse, NY

Clinical Summary:

This 30 year old man presented with increasing shortness of breath and cough over one week. He had a history of pneumonia treated with antibiotics three months previously. On admission, his hematocrit was 60 and hemoglobin 20. His p02 on room air was 50. Chest radiographs showed bilateral ground glass infiltrates. He was initially treated with antibiotics but worsened and developed respiratory failure requiring mechanical ventilation. An open lung biopsy was performed.

(Case contributed by Dr. Robin Bideau, Louisville, KY).


Case 4 - Figure 1 - Low magnification view showing eosinophilic material filling airspaces.

Case 4 - Figure 2 - Higher magnification photomicrograph showing the granular appearance of the airspace exudate. Note that the alveolar septa are only minimally thickened, and there is no significant inflammation.

Case 4 - Figure 3 - High magnification photomicrograph showing the coarsely granular nature of the intraalveolar eosinophilic material. The adjacent alveolar septa are normal except for mild hyperplasia of type 2 pneumocytes.




Case 5

JEFFREY L. MYERS
Mayo Clinic
Rochester, MN

Clinical Summary:

A 21 year old woman who recently quit smoking was self-referred for further evaluation of progressive shortness of breath due to diffuse interstitial lung disease. Transbronchial lung biopsy performed elsewhere was reported as "suspicious for" or "consistent with" Langerhans cell histiocytosis. While in an outpatient waiting area she developed increased back pain and resting sinus tachycardia. She was urgently admitted to a hospital cardiology service for further care and monitoring. Chest x-ray demonstrated left tension pneumothorax with mediastinal shift associated with diffuse interstitial infiltrates. A chest tube was placed. Cardiac evaluation demonstrated sinus tachycardia without evidence of intrinsic myocardial dysfunction. On the second hospital day she underwent video assisted thoracoscopic surgery with wedge lung biopsy and talc pleurodesis.



Case 5 - Figure 1 - Low magnification photomicrograph showing bronchiolocentric infiltrate of predominantly mononuclear cells in Langerhans' cell histiocytosis.

Case 5 - Figure 2 - Higher magnification photomicrograph of pulmonary Langerhans' cell histiocytosis showing peribronchiolar and intraluminal infiltrate in which histiocyte-like cells predominate.


Case 5 - Figure 3 - High magnification photomicrograph showing numerous Langerhans' cells characterized by convoluted nuclei, inconspicuous nucleoli, and abundant eosinophilic cytoplasm with indistinct cytoplasmic borders.

Case 5 - Figure 4 - High magnification photomicrograph showing strong diffuse immunoreactivity for CD1a in Langerhans' cells.