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Pediatric Pathology
Sunday, February 27, 2011, 7:30 PM
CC 101 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



Pediatric Cardiac Biopsies
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Moderators:
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DAVID PARHAM
University of Oklahoma Health Sciences Center College of Medicine, Oklahoma City, OK and

BAHIG SHEHATA
Children’s Heathcare of Atlanta, Atlanta, GA
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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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David Parham, University of Oklahoma Health Sciences Center, Oklahoma, OK
Bahig Shehata, Children’s Healthcare of Atlanta, Atlanta, GA
Glenn P. Taylor, Hospital for Sick Children, Toronto, Ontario, Canada
Aliya N. Husain, University of Chicago Hospitals, Chicago, IL
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Clinical histories are displayed below.
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for Text and References

Submitted by: David Parham -


The patient is a 2 year, 3 month old African American male admitted for apneic episodes during sleep. On physical examination, vital signs included a heart rate of 123 beats/min, respiratory rate 32 breaths/min, and blood pressure 100/70 mm Hg. The only physical finding of note was a liver border 2-3 cm below the right costal margin. Echocardiography revealed cardiomegaly with left ventricular diastolic and systolic dysfunction, biatrial dilatation, pulmonary venous and venocaval diastolic flow reversal, moderate tricuspid stenosis, tricuspid regurgitation, and a small pericardial effusion. Cardiac catheterization found markedly elevated right and left ventricular pressures. Following biopsy and diagnosis, heart transplantation was performed, with removal of an explant consisting of right and left ventricle and the lower portions of dilated right and left atria. Case history and special stains courtesy of Dr. Ali Saad, Arkansas Children’s Hospital.

 Case 1 - Figure 1 Gross image, pen and ink drawing. There is marked dilatation of the right atrium.
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 Case 1 - Figure 2 Low power views of heart, showing unusual pattern of cytoplasmic myocyte inclusions.
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 Case 1 - Figure 3 Low power views of heart, showing unusual pattern of cytoplasmic myocyte inclusions.
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 Case 1 - Figure 4 Various high-power views of the myocytes, showing markedly eosinophilic cytoplasmic inclusions.
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 Case 1 - Figure 5 Various high-power views of the myocytes, showing markedly eosinophilic cytoplasmic inclusions.
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 Case 1 - Figure 6 Various high-power views of the myocytes, showing markedly eosinophilic cytoplasmic inclusions.
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 Case 1 - Figure 7 Various high-power views of the myocytes, showing markedly eosinophilic cytoplasmic inclusions.
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 Case 1 - Figure 8 The inclusions are strongly fuscinophilic with trichrome stains.
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 Case 1 - Figure 9 Desmin staining highlights scattered inclusions.
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for Text and References

Submitted by: Bahig Shehata -


A 7 month old previously healthy born at term Caucasian male infant presented to the emergency room due to bloody loose stool, loss of appetite, and lethargy. A work up for intussusceptions was negative. An abdominal CT scan demonstrated ascites and mesenteric lymphadenopathy. The superior cut of the CT scan showed pericardial effusion. An echocardiogram was performed and demonstrated large pericardial effusion with signs of early tamponade (Figure 1). The bloody clear pericardial fluid was drained. A follow up echo showed a large left ventricular mass protruding beneath the mitral valve, bulging into the pericardial sac (Figure 2). Cytology of the fluid was not definitive and therefore a trucut biopsy of the mass was obtained. The majority of the specimen was necrotic, however in some areas a spindle-cell lesion was identified (Figures 3-5).

 Case 2 - Figure 1Echo showing tumor mass protruding from the left ventricular wall
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 Case 2 - Figure 2Intraoperative photo showing the tumor bulging out of the upper part of the left ventricle wall (see arrow)
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 Case 2 - Figure 3Spindle cell neoplasm with a staghorn blood vessel(200X)
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 Case 2 - Figure 4Spindle cell neoplasm with occasional lymphocytic infiltrate (400X)
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for Text and References

Submitted by: Glenn Paul Taylor -


A 15 year-old boy was seen by a neurologist for the complaint of leg weakness that was characterized as episodes of his “knees giving away”. This had developed in the past 2 or 3 months. Prior, he had no health problems. Examination revealed mild to moderate proximal muscle weakness, but no other significant findings. Family review disclosed that the boy’s mother had developed dilated cardiomyopathy in her late thirties and had recently received an implantable cardioverter-defibrillator. This information prompted the boy’s urgent referral to a cardiologist. An echocardiogram showed marked concentric hypertrophic cardiomyopathy and a large pericardial effusion. He was admitted to hospital for pericardiocentesis and additional investigations. Endomyocardial biopsies and biopsy of vastus lateralis muscle were performed. The virtual slide and other images are from the endomyocardial biopsy.

 Case 3 - Slide 1
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 Case 3 - Figure 1 Intermediate power image, elastic trichrome stain. The cardiac myocytes have cytoplasmic vacuoles of varying size, most appearing empty.
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 Case 3 - Figure 2 High power image, hematoxylin and eosin stain. The high power image shows myocyte hypertrophic change with "blocky" enlarged nuclei, in addition to the cytoplasmic vacuoles.
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 Case 3 - Figure 3 High power image, PAS stain. Abundant cytoplasmic glycogen is demonstrated, but many vacuoles have irregular or absent PAS positive contents.
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 Case 3 - Figure 4 High power image, PAS with diastase predigestion. The large majority of the PAS staining is removed by predigestion with diastase. However, some lipofuscin-like granules remain.
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 Case 3 - Figure 5 Intermediate power electron micrograph. Ultrastructural examination shows membrane-bound vacuoles having heterogeneous contents.
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 Case 3 - Figure 6 High power electron micrograph. High power demonstrates the autophagosome-like appearance of the cytoplasmic vacuoles.
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for Text and References

Submitted by: Aliya N. Husain -


The patient is a 3-year-old male born with complex heart anomalies, status post Norwood and Fontan repairs admitted to the University of Chicago Hospitals for heart transplantation. Post-operatively the patient was supported with ECMO due to poor cardiac function. The first endomyocardial biopsy was performed at 7 days post- transplant images from which are submitted for your review. The immunohistochemical stain for C4d is submitted for scanning as virtual slide. In addition, digital images are submitted as follows:

 Case 4 - Figure 4 Endomyocardial biopsy on day 7 post- transplant, IHC stain for C4d
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 Case 4 - Figure 5 H&E stained section of left anterior descending coronary artery at autopsy shows recent thrombus
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 Case 4 - Figure 6 EVG stain of coronary artery at autopsy shows focal internal elastica disruption and intimal fibrosis (chronic rejection).
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Handouts for all Specialty Conferences will be accessible via the
"Educational Materials" section on the homepage the morning after each respective conference. Printed
copies of the handout will not be available at the meeting.
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