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Pulmonary Pathology
Sunday, March 2, 2008, 7:30 PM
Convention Center 201/203 Unusual Mesenchymal Proliferations







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Moderator:
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DANI ZANDER Penn State Milton S. Hershey Medical Center Hershey, PA
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Disclosure:
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The speakers have indicated they have nothing to disclose.
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Clinical histories are displayed below.
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for Text and References

Submitted by: Samuel A. Yousem - University of Pittsburgh School of Medicine, Pittsburgh, PA

 43-year-old woman with pleuritic chest pain associated with a hemorrhagic pleural effusion, who was found on CT scan to have a pleural based mass with associated diffuse pleural thickening. A pleural biopsy was performed.

 Case 1 - Slide 1
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 Case 1 - Figure 1 A cellular process thickens the visceral pleura and expands and extends down interlobular septa.
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 Case 1 - Figure 2 The thickened pleura has a vague nodularity with increased cellularity in these zones.
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 Case 1 - Figure 3 Focally, myxomatous change is observed in the pleural "plaque".
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 Case 1 - Figure 4 The cellular infiltrate is paucivascular with a whirling storiform architecture.
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 Case 1 - Figure 5 At the pulmonary parenchymal and visceral pleural interface, focal necrosis is observed and air spaces are filled by a dyscohesive cellular infiltrate.
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 Case 1 - Figure 6 The cellular infiltrate consists of mildly pleomorphic epithelioid and spindle cells that spill into air spaces and cuff small blood vessels.
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 Case 1 - Figure 7 Cytologically, the epithelioid cells have round to oval nuclei, diffuse chromatin, and abundant glassy eosinophilic cytoplasm.
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 Case 1 - Figure 8 Occasional cells have cytoplasmic vacuoles which indent their nuclei.
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 Case 1 - Figure 9 The spindle cells have similar cytologic qualities to the epithelioid elements.
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for Text and References

Submitted by: Carlyne D. Cool - University of Colorado Health Sciences Center, Denver, CO

 A 56-year-old woman with a past medical history of migraines, coronary artery disease, and depression presented to her primary care physician with complaints of shortness of breath for four months. She developed increasing dyspnea and edema. She was diagnosed with pulmonary arterial hypertension, but continuous intravenous prostacylin therapy failed to alleviate her symptoms, and she died soon after initiating treatment.

 Case 2 - Slide 1
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 Case 2 - Figure 1 Low power magnification illustrating a patchy area of congestion. The surrounding alveolar septa are thin and delicate. No significant inflammatory infiltrates are identified. No airspace abnormalities are present.
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 Case 2 - Figure 2 An area of apparent congestion with red blood cells and hemosiderin-laden macrophages within airspaces.
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 Case 2 - Figure 3 High power magnification demonstrating increased density of capillary-like vessels within the septa. There are numerous hemosiderin-laden macrophages within airspaces.
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 Case 2 - Figure 4 Another high power field illustrates the numerous capillary-like vessels within the septa. There is no cytologic atypia or mitoses.
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 Case 2 - Figure 5 A cluster of multinucleated giant cells engulf an iron coated elastic fiber. There is a surrounding cuff of chronic inflammatory cells with occasional eosinophils. Additional iron coated fibers are present in the upper right and lower left fields of the image.
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for Text and References

Submitted by: Richard Attanoos - Llandough Hospital, Penarth, South Glamorgan, United Kingdom

 A 37-year-old female, previously healthy, presented with a 2-month history of increasing dyspnea. CXR revealed a unilateral pleural effusion, and CT scan showed an effusion, chest wall/pleural mass, and pulmonary infiltrates. At thoracoscopy, the pleura appeared diffusely nodular. She underwent pleural biopsy.

 Case 3 - Slide 1
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 Case 3 - Figure 1 Sheets of small to intermediate hyperchromatic cells, undifferentiated.
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 Case 3 - Figure 2
Higher power reveals tumour cells have variable morphology. Some cells show vacuolated clear cytoplasm, other have pseudoinclusions of nuclei. In some, there is a plasmacytoid/ haematolymphoid appearance.
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 Case 3 - Figure 3
Focal positive AE1/AE3 (Cytokeratin).
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for Text and References

Submitted by: Megan Dishop - Baylor College of Medicine, TX Children's Hospital, Houston, TX

 Newborn boy, twin A, delivered at 28 weeks gestation to a 31-year-old mother with respiratory distress and severe hydrops, requiring intubation, high frequency oscillatory ventilation, and nitric oxide therapy. Maternal history and obstetric history were unremarkable, except for discordant fetal growth (twin A larger, twin B smaller). Chest x-ray showed a hyperinflated right lung and a mass in the right upper lung field, displacing the heart. No pleural or pericardial effusions were detected at delivery. Echocardiogram showed evidence of persistent pulmonary hypertension. He had rapid deterioration despite support and died less than 24 hours after delivery. A similar mass was not detected in Twin B.

At autopsy, the baby was hydropic and non-dysmorphic. The lungs were discrepant in size, with a very large right lung weighing 70 grams (12 grams expected) and a hypoplastic left lung. The right upper lobe was enlarged, covering the anterior mediastinum, and replaced by a firm solid pale tan mass. The right lower lobe was hemorrhagic. No other visceral anomalies were identified.

A section of the right upper lobe is provided.

 Case 4 - Slide 1
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 Case 4 - Figure 1 Gross. A section of the right upper lobe mass showed a solid tan tumor with a fascicular pattern.
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 Case 4 - Figure 2 Microscopic (H&E, 2x). The mass is composed of broad bundles of spindled cells dissecting the lung parenchyma along interlobular septa and along bronchovascular bundles. Abnormal proliferation of cartilage plates is associated with the small bronchi.
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 Case 4 - Figure 3 Microscopic (H&E, 4x). The spindle cell proliferation distorts the architecture of the lung and compresses some bronchioles.
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 Case 4 - Figure 4 Microscopic (H&E, 20x). The spindled cells shows a haphazard arrangement of fascicles and uniform bland cytologic features.
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 Case 4 - Figure 5 Microscopic (H&E, 20x). There are occasional mitotic figures and no significant inflammatory cell infiltrates.
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for Text and References

Submitted by: Aliya N. Husain - University of Chicago, Chicago, IL

 On prenatal ultrasound, this baby boy was found to have a left lung sequestration/mass. There was no other malformation seen. The lesion was resected at 8 weeks of age. The specimen consisted of tan-pink tissue measuring 3.3 x 2.6 x 1.7 cm. Serial sectioning showed multiple cysts varying in size from 0.1 to 0.5 cm.

 Case 5 - Slide 1
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 Case 5 - Figure 1 Low power view of lung section showing bronchus in the lower part and cystic structures in the upper part.
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 Case 5 - Figure 2 Medium power photomicrograph of bronchus and cysts, No alveolated lung is seen.
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 Case 5 - Figure 3 Low power view of peripheral lung completely replaced by small to intermediate sized cysts.
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 Case 5 - Figure 4 High power of cysts showing ciliated columnar epithelial lining. No smooth muscle or mucogenic cells are present in the cyst wall. The intervening fibrovascular stroma contains pink cells.
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 Case 5 - Figure 5 Highest power photomicrograph shows skeletal muscle cells with cross striations within the stroma between the cysts.
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