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Pulmonary Pathology
Sunday, February 27, 2011, 7:30 PM
CC 006 A/B
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
An interesting case that taught me something I did not know before
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Moderator:
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THOMAS COLBY
Mayo Clinic Arizona
Scottsdale, AZ
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Disclosure:
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In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose.
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Panelists:
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Andrew Churg, University of British Columbia, British Columbia
Patrizia Morbini, University of Pavia, Pavia, Lombardy, Italy
Frank Schneider, University of Pittsburgh, Pittsburgh, PA
Andrea Valeria Arrossi, Cleveland Clinic, Cleveland, OH
Thomas V. Colby, Mayo Clinic Arizona, Scottsdale, AZ
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Clinical histories are displayed below.
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for Text and References
Submitted by: Andrew Churg -
This is a 50 yo man with a history of traumatic perforation of the esophagus in 2003 in a motor vehicle accident. The esophagus was repaired with a colonic interposition. In early 2008 the patient presented with an upper lobe pneumonia and cultures grew an organism identified as Rhodococcus. The patient was HIV negative and had normal T cell counts. He improved on antibiotic therapy. In late 2008 the patient presented with a clinical picture of bilateral pneumonia and a lung biopsy was performed. Culture of the biopsy grew an organism identified as a Gordonia species.
Case 1 - Figure 1
CT scan from the December 2008 admission showing patchy areas of peripheral
consolidation and a pleural effusion.
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Case 1 - Figure 2
Progressively higher power views showing necrotizing granulomas containing large lipid droplets
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Case 1 - Figure 3
Progressively higher power views showing necrotizing granulomas containing large lipid droplets
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Case 1 - Figure 4
Progressively higher power views showing necrotizing granulomas containing large lipid droplets
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Case 1 - Figure 5
Non-necrotizing granuloma containing a lipid droplet in the center
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Case 1 - Figure 6
Non-necrotizing granuloma containing a lipid droplet in the center
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for Text and References
Submitted by: Patrizia Morbini -
A 52-years old male presented with persistent exertion dyspnea and chronic cough. His past clinical records were unremarkable; smoking history was negative. Lung function tests showed mild restrictive dysfunction and 14% DLCO reduction. CT scan showed diffuse ground glass opacities with centrilobular reticular infiltrates and bronchiectases. Minimal apical honeycombing was observed. Serology for ANA, ENA and ANCA was within normal limits. No bird exposure was reported. A videothoracoscopic lung biopsy was performed. A patient’s younger brother had long been suffering of fatigue, attributed to congestive heart failure. Their second degree female cousin had been diagnosed with interstitial lung fibrosis at the age of 13. The patient is alive 5 years after disease presentation, with minimal progression of the disease at functional and imaging studies.
Case 2 - Figure 1
Low power view of lung parenchyma showing topographic heterogeneity characterized by the alternance of fibrotic areas and normal alveolar structures.
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Case 2 - Figure 2
Centrilobular scarring extending from the peribronchiolar area associated with mucus plugging.
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Case 2 - Figure 3
Diffuse homogeneous scarring of a subpleural lobule mostly consistent with NSIP. Peribronchial metaplasia is present in the centrilobular area. Other lobules are minimally altered.
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Case 2 - Figure 4
High power view of the fibrosis shown in figure 3.
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Case 2 - Figure 5
Centrilobular scarring extending from the peribronchiolar area associated with inflammatory infiltrates.
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Case 2 - Figure 6
Centrilobular scarring extending from the peribronchiolar area associated with prominent peribronchiolar metaplasia. A membranous bronchus in the lower left corner presents a mild infilammatory infiltrate.
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Case 2 - Figure 7
Isolated constrictive bronchiolitis with normal surrounding parenchyma.
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Case 2 - Figure 8
Isolated constrictive bronchiolitis with inflammatory infiltrates in a otherwise unaffected lobule.
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Case 2 - Figure 9
CT scan of the index patient showing reticulo-nodular infiltrates and ground glass opacities.
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Case 2 - Figure 10
Low power view of the lung biopsy taken from the left lower lobe of the younger cousin of the index patient showing severe interstitial fibrosis with honeycombing.
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Case 2 - Figure 11
High power view of the sample shown in figure 9, highlighting fibroblast foci with peribrochiolar location and diffuse fibrosis.
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Case 2 - Figure 12
Low power view of the lung biopsy taken from the left upper lobe of the younger cousin of the index patient showing interstitial fibrosis with centrilobular accentuation.
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Case 2 - Figure 13
CT scan of the younger cousin of the index patient showing interstitial fibrosis and peripheral honeycombing.
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Case 2 - Figure 14
The family tree showed a remarkable degree of cross-marriages. White symbols represent subject unavailable for genetic tests, gray symbols represent non-mutated relatives, red-and-grey symbols carriers of one mutated ABCA3 allele and red symbols the carriers of the homozygous ABCA3 gene mutation. The index patient is indicated with a black arrow, the cousin with a green arrow. All siblings of the index patient carry the mutation, one of them homozygous. This brother suffers of respiratory insufficiency and has a severely abnormal lung CT scan.
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for Text and References
Submitted by: Frank Schneider -
A 26-year-old man presented to an emergency room in Pittsburgh, PA, with a 10-day history of worsening shortness of breath, productive cough and fatigue. He was admitted and treated with azithromycin and ampicillin for presumed community-acquired pneumonia. Despite antibiotic therapy he continued to remain febrile with persistent leukocytosis (eosinophilia) and increasing supplemental oxygen requirements eventually requiring intubation. A chest CT showed extensive, bulky mediastinal lymphadenopathy and a dense airspace consolidation within the right upper lobe with small cavitation. In addition, there were innumerable widespread centrilobular nodules. A bronchoalveolar lavage showed atypical lymphoid cells with 20 percent eosinophils. At the same time the present transbronchial biopsy was obtained.
Case 3 - Slide 1
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Case 3 - Figure 1
Airspaces filled with fibrin, macrophages, a single giant cell and scattered inflammatory cells (H&E x100).
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Case 3 - Figure 2
Foamy histiocytes and fibrin in alveoli.Mononuclear inflammatory cells form a loose aggregate (H&E x200).
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Case 3 - Figure 3
Macrophages and a multinucleated giant cell. Eosinophils are present within the airspace exudate and in alveolar septa (H&E x200).
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Case 3 - Figure 4
Spherical structures within the vaguely granulomatous aggregate of airspace macrophages (center and edge of biopsy at 1 o'clock) (H&E x400).
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Case 3 - Figure 5
A spherical fungal organism with refractile wall and poorly defined internal structure (H&E x400).
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Case 3 - Figure 6
Two fungal organisms highlighted by a silver stain. The one in the lower left appears to contain some internal structures displacing the stain (Grocott methenamine silver x400).
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Case 3 - Figure 7
The wall of the organisms is highlighted by a PAS stain (PAS x400).
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Case 3 - Figure 8
A mucin stain fails to highlight a mucinous capsule around the organisms (Mucicarmine x400).
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Case 3 - Figure 9
Chest CT showing right upper lobe airspace consolidation with cavitation and innumerable small centrilobular nodules.
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for Text and References
Submitted by: Andrea V. Arrossi -
A 50 year old woman, ex-smoker, presents with weakness and shortness of breath. The patient had a past medical history of medically controlled diabetes mellitus type 2, hypothyroidism and hypertension. She has a five-pack-year smoking history, quitting 8 years ago, and works in a factory making ceramic jet engine parts. Initial laboratory tests revealed severe anemia (hemoglobin of 5.6g/dL). An esophagogastric duodenal endoscopy showed features consistent with celiac sprue, confirmed with elevated titers of gliadin IgG and IgA and transglutaminase IgA antibodies. Colonoscopy and camera endoscopy were normal. Her symptoms slightly improved on a gluten-free diet. However, she continued to have weakness, and developed abdominal pain. A subsequent abdominal CT scan showed an infiltrative retroperitoneal soft tissue mass that encased and involved the pancreas and a chest CT scan showed several lung nodules ranging from 0.3 to 3.3 cm, in the right upper, middle and lower lobes. No pleural effusions or lymphadenopathies were seen. Two pancreatic percutaneous biopsies, a percutaneous biopsy of the right middle lobe nodule and a bone marrow biopsy were performed for suspected pancreatic malignancy or lymphoma with pulmonary involvement. The biopsies were non-diagnostic and the patient underwent a video assisted thoracoscopic biopsy (slides and pictures provided).
Case 4 - Slide 1
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Case 4 - Figure 1
Thin-section chest CT scan demonstrating a well defined 2.7 cm solid mass in the right lower lobe, abutting the major fissure with focal pleural thickening
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Case 4 - Figure 2
Low magnification of the lesion showing extensive sclerosing fibrosis and scattered chronic inflammatory infiltrates
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Case 4 - Figure 3
Chronic inflammatory infiltrates obliterating the lumen of a vascular structure
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Case 4 - Figure 4
Chronic inflammatory infiltrates showing numerous plasma cells
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Case 4 - Figure 5
Chronic inflammatory infiltrates showing numerous plasma cells
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Case 4 - Figure 6
Chronic inflammatory infiltrates showing numerous plasma cells
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Case 4 - Figure 7
Dense sclerosis and diffuse lymphoplasmacytic infiltrates involving fibrotic tissue
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Case 4 - Figure 8
Movat stain highlighting two vessels with obliterative inflammation in the same field in Figure 7
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Case 4 - Figure 9
High magnification of IgG4 immunostain showing greater than 10 IgG4-positive plasma cells
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Case 4 - Figure 10
CT images of the retroperitoneal mass pre (left) and post (right) treatment, showing a decrease in size after steroid therapy
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for Text and References
Submitted by: Thomas V. Colby -
A 28-year-old journalist who was a non-smoker and had never used illicit drugs presented with hemoptysis of 7 years duration. Since his initial presentation in 2003, he has had multiple recurrences of hemoptysis but it was never life threatening. This has been associated with transient infiltrates and alveolar opacities and at least two episodes of pneumothorax. At one point he had a cavitary lesion noted in the lung. Workup for alveolar hemorrhage syndromes was negative. His initial lung biopsy was taken in 2006 at the time of a left pneumothorax. Subsequently, in 2010, he presented with a second (right side) pneumothorax associated with hemoptysis and again lung biopsy was obtained and “apical bullae” were resected.
Note: images 1-9 from 2006; images 10-17 from 2010
Case 5 - Figure 1
2006 Figure 1. Low power view shows a "hole" in the lung with a disruption of the alveolar architecture and a central nodule.
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Case 5 - Figure 2
2006 Figure 2. The nodule seen in the center of 2006 Figure 1 shows dense fibrous tissue that does not resemble any usual lung repair reaction.
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Case 5 - Figure 3
2006 Figure 3. The tissue surrounding the "hole" in 2006 Figure 1 shows hemosiderin-laden macrophages.
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Case 5 - Figure 5
2006 Figures 5 – 8. Detail of fibrous nodule showing dense fibrous connective tissue that is paucicellular and includes focal metaplastic bone formation
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Case 5 - Figure 6
2006 Figures 5 – 8. Detail of fibrous nodule showing dense fibrous connective tissue that is paucicellular and includes focal metaplastic bone formation
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Case 5 - Figure 7
2006 Figures 5 – 8. Detail of fibrous nodule showing dense fibrous connective tissue that is paucicellular and includes focal metaplastic bone formation
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Case 5 - Figure 8
2006 Figures 5 – 8. Detail of fibrous nodule showing dense fibrous connective tissue that is paucicellular and includes focal metaplastic bone formation
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Case 5 - Figure 9
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Case 5 - Figure 10
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Case 5 - Figure 11
2010 Figure 1. Low power view shows a region of hemorrhage and organization in the subpleural regions.
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Case 5 - Figure 12
2010 Figure 2. Higher power microscopy of 2010 Figure 1 shows fresh hemorrhage and organization within the lung tissue.
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Case 5 - Figure 13
2010 Figures 3 and 4. In another region of organization one can see early collagenization of the organizing intraluminal polyps.
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Case 5 - Figure 14
2010 Figures 3 and 4. In another region of organization one can see early collagenization of the organizing intraluminal polyps.
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Case 5 - Figure 15
2010 Figure 5. Low power view shows a region of organization with densely collagenized fibrous nodules developing (center).
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Case 5 - Figure 16
2010 Figure 6. Detail from 2010 Figure 5 shows a paucicellular dense collagenized nodule that does not resemble usual organization in the lung.
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Case 5 - Figure 17
2010 Figure 7. No abnormalities are noted on elastic tissue staining of a bronchovascular bundle and adjacent alveoli.
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