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Pediatric Pathology
Sunday, March 25, 2007, 7:30 PM
Elizabeth F
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Moderator:
CHERYL M. COFFIN
Primary Children's Medical Center
Salt Lake City, UT
Disclosure: The speakers have indicated they have nothing to disclose.
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for Text and References
Submitted by: Phyllis C. Huettner - Washington University Medical Center, St. Louis, MO
The patient is a 30-year old G4P0120 Caucasian woman. Ultrasound at 8 weeks showed a 6 x 6 x 4 cm mass thought to be a hematoma adjacent to the placenta. The mass persisted on weekly ultrasounds and became cystic on week 12. The fetus was normal. Serum hCG levels were three times normal for gestational age. Termination then karyotyping were recommended but declined. Intrauterine growth retardation developed at 24 weeks and the patient was hospitalized and placed on bed rest. Over the next few weeks the amniotic fluid index decreased. At 28 weeks gestation the baby stopped growing. The cystic mass adjacent to the placenta was unchanged. A normal 530 gram female fetus with Apgar scores of 4 at one minute and 9 at five minutes was delivered by Cesarean section. The placenta was markedly abnormal.
Case 1 - Slide 1
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Case 1 - Figure 1 - Gross photograph of the fused twin placenta with normal parenchyma on the left and molar tissue on the right
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Case 1 - Figure 2 - Close up of the pale, molar tissue with large, fluid-filled molar villi
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Case 1 - Figure 3 - Extensively necrotic, fluid-filled molar villi
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Case 1 - Figure 7 - Higher power of trophoblast hyperplasia with extensive necrosis
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Case 1 - Figure 8 - Non-molar villi with accelerated villous maturation for gestational age
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Case 1 - Figure 9 - Accelerated villous maturation with small, long villi and increased syncytiotrophoblastic knots
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for Text and References
Submitted by: Elisabeth R. Bruder - University of Basel, Basel, Switzerland
Female patient, age 16 years. Swelling of mandibular angle for three weeks. Extraction of tooth 38 and curettage of alveola. Recurrence nine months after primary diagnosis. Mandibular resection. The seminar slide originates from the mandibular resection.
Case 2 - Slide 1
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Case 2 - Figure 1 - H&E Low power: Tumor composed of vascular proliferation with lobular architecture and peripheral new bone formation.
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Case 2 - Figure 2 - H&E x100: Vascular proliferation with plump endothelial cells and loose stroma.
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Case 2 - Figure 3 - H&E x 400: Epithelioid endothelial lining. Predominantly monomorphic nuclei. Scattered stromal inflammatory cells including eosinophils.
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Case 2 - Figure 4 - H&E high power x 400: Focus of moderately pleomorphic endothelial nuclei and prominent nucleoli.
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Case 2 - Figure 5 - H&E x100: Area of strands of solid endothelial aggregates.
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Case 2 - Figure 6 - H&E high power x 400: Solid endothelial strands and small capillary vascular proliferations with plump epithelioid endothelia. Moderately pleomorphic folded nuclei with pominent nucleoli. Occasional cytoplasmic vacuoles.
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for Text and References
Submitted by: Gareth P. Jevon - British Columbia Children's Hospital, Vancouver, BC, Canada
A 5-year old girl was referred to Princess Margaret Hospital for Children, Perth WA, for evaluation of her respiratory distress. She had inspiratory and expiratory stridor, a tracheal tug, subcostal recession, good bilateral air entry, and no crepitations. There was no fever or clubbing. Examination of the ears, nose, and throat was normal. Clinically, she was thought to have a large airway obstruction and arrangements were made for an urgent flexible bronchoscopy.
She had been diagnosed with asthma 6 months prior to her presentation. She had since been admitted to the hospital 4 times. Following a streptococcal pneumonia of the right lower lobe 3 months earlier, she developed noisy breathing, with prolonged periods of wheezing and stridor, and little response to bronchodilators. Two weeks before her evaluation she was admitted to a peripheral hospital with asthma. She was afebrile, and chest X-ray showed patchy bilateral perihilar consolidations. Neck X-rays did not show features of epiglottitis. Abnormal laboratory results included an arterial blood oxygen saturation of 80%, a white cell count 2.9 x 109 (5.0-17.0), neutrophils 18.9 x 109 (1.5-8.5), and C-reactive protein 150 mg/L (>10). She responded poorly to nebulized adrenalin, intravenous dexamethasone, and azithromycin, but while stable, she was still wheezing when discharged.
At bronchoscopy there was a polypoid mass attached to the postero-lateral wall of the trachea, almost filling the lumen above the carina. The mass was resected leaving minor residual disease of the wall.
Case 3 - Slide 1
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Case 3 - Figure 1 - The biopsy sections show a cellular neoplasm with compact fascicles of spindle cells. Figure 2.
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Case 3 - Figure 2 - In some foci the cells are arranged a storiform pattern.
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Case 3 - Figure 3 - In one or two areas the tumor cells are more polygonal, or ganglion-like. There were no epithelioid cells.
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Case 3 - Figure 4 - The spindled cells have uniform, elongated, vesicular nuclei and indistinct cytoplasm. There are no anaplastic cells.
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Case 3 - Figure 5 - There is a background inflammatory infiltrate composed of lymphocytes and plasma cells.
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Case 3 - Figure 6 - The spindle cells are mitotically active (3 per high power field).
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Case 3 - Figure 7 - Focally beneath the respiratory epithelium the cells had an edematous background. There was no compact cambian layer.
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Case 3 - Figure 8 - The spindle cells have diffuse cytoplasmic vimentin staining.
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for Text and References
Submitted by: Milton J. Finegold - Texas Children's Hospital, Houston, TX
Twin A was born at 27 4/7 weeks weighing 1090 gm by C-section to 36-year old mother with gestational diabetes and idiopathic thrombocytopenic purpura requiring IVIG x3. Required intubation, assisted ventilation, umbilical venous catheterization. Parenteral alimentation with Trophamine, Intralipid begun on day 2, with oral trophic feeds [Pedi Enfamil] as tolerated. Day 3 treated for sepsis; recurred on day 13. Day 5 Bu 3.8, Bc 2.8, AST 1010, ALT 6, AP 331, GGT 1098, Alb 3. Ultrasound: increased hepatic echogenicity; collapsed gallbladder; CBD not visualized; intrahepatic ducts not dilated. Progressive fall in GGT, AP stayed elevated (500s); TPN stopped day 16. At one month, intubated, on CPAP, good weight gain, Bu 3.7, Bc 1.7, AP 606. Repeat US at 6 weeks - increased hepatic echogenicity; no change in GB or ducts. At 3 mo, Bu 1.6, Bc 3.8, AST 84, ALT 59, AP 417, GGT 556 prompting Ursodeoxycholate treatment; repeat US--contracted GB; hyperechoic liver; HIDA scan--no excretion [stool color never mentioned till now--yellow-brown]; Open biopsy on DOL 145 months
Case 4 - Slide 1
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Case 4A - Figure 1 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 2 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 3 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 4 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4A - Figure 5 - portal tract features are consistent with extrahepatic biliary obstruction
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Twin B born at 31 3/7 weeks weighing 828 gm. Managed in another city for 3 months with RDS evolving to BPD, complicated by Pseudomonas pneumonia. Candida, Staph and Klebsiella UTI also noted. Microcephalic but CNS functioning intact. Hepatosplenomegaly with increased Bc. Ultrasound--ascites; no organ abnormalities. At 90 days, anemic, thrombocytopenic, Bc 1.5, AST 82, ALT 1001, AP 829, GGT 66, prolonged PT, PTT. Skeletal XR-metaphyseal dysplasia. Repeat US at 100 days: contracted, thick-walled GB, normal intrahepatic ducts. HIDA: poor uptake, but excretion into intestine. Biopsy on DOL 120.
Case 4 - Slide 2
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Case 4B - Figure 1 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4B - Figure 2 - portal tract features are consistent with extrahepatic biliary obstruction
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Case 4B - Figure 3 - portal tract features are consistent with extrahepatic biliary obstruction
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