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Pediatric Pathology
Monday, February 28, 2005 - 7:30 PM
Convention Center, Ballrooom B
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Moderator:
Deborah Perry
Children's Pathology
Omaha, NE
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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
Submitted by:
Raj P. Kapur
Children's Hospital and Medical Center
Seattle, WA
A 3-month-old male was admitted with thrombocytopenia, anemia, conjunctivitis, and
hepatosplenomegaly. The patient was delivered at full-term after an uncomplicated first pregnancy. He
required phototherapy for perinatal jaundice. Approximately 2 weeks prior to presentation, he developed
fever and upper respiratory symptoms and was evaluated for sepsis at another institution. No evidence of
bacterial infection was found. One day prior to admission, he developed bilateral ocular discharges and
fever. The results of laboratory studies at the time of admission included a white blood cell count of
19,300 cells/mm3, peripheral blast count of 6%, and elevated serum alkaline phosphatase. A
bone marrow biopsy was performed.
Case 1 - Figure 1 - An iliac crest biopsy demonstrates relatively uniform persistence of primitive woven bone with intervening fibrous marrow. Ineffective remodeling of bone is evident from the paucity of laminated osteoid and foci of mineralized matrix that resemble calcified cartilage. The density of hematopoietic cells in the marrow is markedly reduced.
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Case 1 - Figure 2 - At high magnification, numerous osteoclasts are easily identified along the surfaces of bony trabeculae. The surfaces of bone facing these multinucleate cells are flat, as opposed to the deep Howship lacunae that characterize normal osteoclast-mediated bone resorption.
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Case 1 - Figure 3 - A low magnification image of the core biopsy demonstrates the diffuse uniform nature of the underlying defect in osteoid resorption, reduced hematopoiesis, and marrow fibrosis.
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Submitted by:
Carole A. Vogler
Cardinal Glennon Children's Hospital
St. Louis, MO
A ten-month-old African-American boy presented with rhinorrhea and fever. The child was the product
of a normal term pregnancy born to a 22-year-old G2 P2 mother. He had a history of bilateral inguinal
hernias repaired at age two months. Family history was negative for developmental disease. Three
half-siblings were all in good health. Physical examination showed a weight > 50th
percentile, height > 10th percentile, head circumference > 95th percentile
for his age. He had an abnormal facies with frontal bossing, a flat nasal bridge, and coarse features,
and "spade-like" hands with short "stubby" fingers. He had no gibbus. A 1.5 cm umbilical hernia and
hepatosplenomegaly were present; the liver was 6 cm below the right coastal margin and the spleen was 3
cm below the left coastal margin. He had bilateral corneal clouding. A conjunctival biopsy was performed.
Case 2 - Figure 1 - The conjunctival connective tissue contains numerous fibroblasts distended by what appear by light microscopy to be empty vacuoles. This extensive cytoplasmic vacolization is very suggestive of a lysosomal storage disease. (Toluidine blue)
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Case 2 - Figure 2 - By electron microscopy, the vacuoles distending the fibroblasts in the conjunctival stroma are surrounded by a single membrane and contain fine fibrillogranular storage material and occasional electron dense granules. This type of storage material is nonspecific but very characteristic of the mucopolysaccharidoses. It can also be seen in other lysosomal storage diseases, including mucolipidoses, mannosidosis, and sialadosis. (Uranyl acetate-lead citrate)
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Submitted by:
Charles Timmons
Children's Medical Center, Dallas, TX
The patient, at presentation to her primary physician, was a 15-month old girl whose parents had begun
noticing an increased firmness in the right side of her abdomen approximately two months prior to
admission. This firmness persisted, although she had no complaints related to it and was otherwise
asymptomatic. The primary physician suspected a renal tumor and referred her for further evaluation.
Radiography demonstrated an approximately 8-cm discrete multicystic mass originating in the right kidney,
extending across the midline and compressing the inferior vena cava. The residual right kidney was
compressed and hydronephrotic. The left kidney was unremarkable. There was no abdominal
lymphadenopathy. An exploratory laparotomy followed by right nephrectomy was performed.
The resected kidney weighed 462 grams and measured 12 x 10 x 8 cm. Bisection in a coronal plane
(Figure 1) revealed an 8.5 x 8.0 x 6.0 cm, sharply demarcated, multiloculated cystic mass, with a
discrete rim of residual renal parenchyma. The cysts ranged up to 3.5 cm in diameter. Their septa were
delicate and contained no obvious expansile masses. Polypoid "tongues" of tissue composed of cysts and
myxoid soft tissue projected from the main mass into the renal calyces and extended 4 cm down the ureter
with mural attachment. The histology is illustrated (Figures 2-4). There was neither hilar nor capsular
invasion, and a hilar lymph node was free of tumor.
The post-operative period was uneventful, and she was discharged on day #7 of hospitalization. No
chemotherapy or radiation was given. There has been no evidence of disease recurrence or metastasis,
with follow-up of ten years.
Case 3 - Figure 1 - Right kidney, bisected, illustrating a well demarcated multicystic mass with extension into adjacent renal calyces. The ureter is distended by similar tissue in its lumen. (Gross appearance)
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Case 3 - Figure 2 - Right kidney. The cysts are lined by mature epithelium, ranging from tall "hobnail" cells with abundant cytoplasm to flattened, inconspicuous epithelial cells. The septa, while generally fibrous or fibromyxoid, focally are populated by condensations of round or elongated immature cells forming occasional tiny tubules. Occasional aggregates of lymphocytes and plasma cells are also present. (H&E, original magnification x 100)
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Case 3 - Figure 3 - Right kidney. The polypoid projections of tissue into the calyces and ureter resemble the septal tissue but with a more myxoid and edematous interstitium. A cambium layer of condensed immature cells is focally present. The lining epithelium is mature and cuboidal to flat. (H&E, original magnification x 40)
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Case 3 - Figure 4 - Right kidney. In one focus of the main mass, the interstitial cells assume bizarre anaplastic features, characterized by marked cellular pleomorphism, nuclear hyperchromasia, and very large multipolar or disorganized mitotic figures. Some of the anaplastic cells show cytoplasmic hyaline globules and karyorrhexis. The anaplastic cells, while focal, tend to infiltrate along the adjacent septa rather than impinging upon the cysts. Extensive further sampling revealed no additional anaplastic foci in the mass. (H&E, original magnification x 200)
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Submitted by:
Ana M. Gomez
Children's Medical Center
Dallas, TX
The patient, a previously healthy 16 year old Hispanic female, presented to the Emergency Department
with a three-day history of abdominal pain. The pain awoke her from sleep and became progressively
severe. The pain was centered in the left upper quadrant, and was associated with nausea and vomiting.
Physical examination revealed and obese teenager in moderate distress. The abdomen was soft,
non-distended, with moderate discomfort to deep palpation of the left upper quadrant. She had normally
active bowel sounds. Laboratory examination revealed a white count of 15.7 with 86% neutrophils.
Amylase and lipase were within normal limits. An abdominal CT scan revealed a cystic mass in the
pancreatic body, diagnosed radiologicaly as a pancreatic pseudocyst versus a duplication cyst.
The patient underwent distal pancreatectomy and splenectomy on hospital day 3. She did well
postoperatively. A CT scan of the chest was performed and was normal. She was discharged on hospital
day 10, postoperative day 7.
Gross examination revealed an 11.5 x 11 x 2.5 cm/331 gm surgical specimen consisting of an 8 x 8 x 6
cm segment of distal pancreas, attached peripancreatic tissue, and an 11 x 5.8 x 5 cm/150 gm spleen
(Figure 1). Serial sectioning through the pancreas demonstrated a 7 x 6.5 x 2.5 cm well circumscribed
partially cystic mass with a yellow-tan to pink-tan variegated cut surface, and multiple areas of
necrosis and hemorrhage (Figure 2). The peripancreatic fibroadipose tissue appeared soften and necrotic,
but no extrapancreatic extension of the tumor or involvement of the spleen was demonstrated.
Histologic sections taken through the tumor are demonstrated in Figures 3-5. Vimentin staining is demonstrated in Figure 6 and beta-catenin staining in Figure 7. Cytogenetics analysis of the tumor revealed a 46, XX karyotype.
The patient received no further therapy and remains well with no evidence of disease 6 months after
her diagnosis.
Case 4 - Figure 1 - Distal pancreas, peripancreatic tissue and spleen (gross)
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Case 4 - Figure 2 - Pancreas with well-circumscribed partially cystic mass (gross)
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Case 4 - Figure 3 - Pancreas mass exhibiting a variable growth pattern with solid and papillary areas (H & E stain)
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Case 4 - Figure 4 - Pancreas mass solid areas composed of small uniform cells with eosinophilic cytoplasm, ovoid uniform nuclei with finely dispersed chromatin and inconspicuous nucleoli (H & E stain)
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Case 4 - Figure 5 - Pancreas mass with papillary-appearing areas (H & E stain)
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Case 4 - Figure 6 - Pancreas tumor cells strongly and uniform positive (Vimentin immunoperoxidase stain)
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Case 4 - Figure 7 - Pancreas tumor cells exhibit positive nuclear staining (beta-catenin immunoperoxidase stain)
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