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Pediatric Pathology
Sunday, March 2, 2008, 7:30 PM
Convention Center 111/113
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Moderator:
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CHERYL COFFIN
Primary Children's Medical Center
Salt Lake City, UT
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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: Julie Fanburg-Smith - Armed Forces Institute of Pathology, Washington, DC
This 6-year old boy had a 2.5 cm intramuscular mass removed. The tumor was grossly circumscribed, multinodular, grey-pink and fleshy to firm.
Case 1 - Slide 1
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Case 1 - Figure 1
At low power, this alveolar soft part sarcoma of the tongue in a child is surrounded by tongue skeletal muscle and separated by thick fibrous septa into large lobules.
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Case 1 - Figure 2
Higher magnification reveals the sheet like, non-alveolar pattern of this tumor. The tumor cells are polygonal with vesicular smooth to focally concave dark nuclear membranes and prominent nucleoli. There is a relative paucity of mitotic activity.
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Case 1 - Figure 3
Even higher magnification reveals clear to eosinophilic, cytoplasm and apparent thin fibrous septa around each cells or small 2-4 cell clusters. The vessels of the septa are not obvious and there is no loss of cellular cohesion or central necrosis. The cells with clear or webbed eosinophilic cytoplasm mimic lipoblastlike or spiderlike cells.
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Case 1 - Figure 4
Some cells appear to have a rhabdoid appearance with eccentric eosinophilic cytoplasm, mimicking rhabdomyosarcoma. Together with the MyoD1 appearance, one may mistake this for rhabdomyosarcoma.
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Case 1 - Figure 5
Nuclear contours are round to focally concave, revealing the "cookie bite" nuclear appearance.
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Case 1 - Figure 6
Mitotic activity is extremely difficult to find, despite the prominent nucleoli and vascular invasion (see Figure 7).
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Case 1 - Figure 7
Vascular invasion outside of tumor capsule is usually found in these tumors.
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Case 1 - Figure 8
Periodic acid Schiff with diastase (PAS-D) reveals granular to rhomboid crystalline material in most cases, from involvement of a few to nearly all cells.
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Case 1 - Figure 9 - MyoD1
These tumors are notoriously vimentin negative and reveal sometimes strong cytoplasmic MyoD1 staining (as depicted here), not to be confused with a positive interpretation. MyoD1, a skeletal muscle myoregulatory protein, is a nuclear marker and is only read as positive when nuclear.
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for Text and References
Submitted by: Rita Alaggio - Universita di Padova, Padova, Italy
A 2-year old female was referred to the local hospital for failure to thrive. Family and personal health histories were negative. Ultrasound performed due to a suspicion of a urinary infection showed a voluminous abdominal mass. CT scans confirmed a solid lesion with necrotic areas in the right abdomen, extending to the left and right iliac fossa, adherent to liver, right kidney, cava and abdominal wall, and measuring 12 x 60 cm. An open biopsy was done. Histology was reviewed at the University of Padova and the child received 2 courses of chemotherapy (IVA2). Due to the poor response to therapy, child underwent surgery. The mass was resected with part of the omentum. Nodules from the anterior peritoneum, right iliac fossa, pelvis and sigma were also removed and gastric, peri-duodenal and mesentery lymph nodes were biopsied. After surgery, a new chemotherapy regimen (Ifosfamide/Adriamycin) was given. Six months later, the tumor recurred. A debulking operation was not considered possible. The child is alive with disease progression (multiple masses in the abdomen) after 2 years. The seminar slide originates from the primary resection.
Case 2 - Slide 1
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Case 2 - Figure 1
The lesion is well circumscribed and focally surrounded by a thin fibrous pseudocapsule.
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Case 2 - Figure 2
This highly cellular neoplasm is composed of spindle cells with a vaguely fascicular pattern. There is a complex vascular network, formed by small capillaries, mostly arranged longitudinally.
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Case 2 - Figure 3
Foci of necrosis are present.
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Case 2 - Figure 6
Areas with epithelioid cells, arranged in a nested pattern with a typical chicken-wire vascular pattern, are also seen.
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Case 2 - Figure 7
At higher power the epithelioid cells are characterized by clear cytoplasm, and nuclei show mild variation in shape and size with frequent nucleoli.
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for Text and References
Submitted by: Mark Lovell - Children's Hospital, University of Colorado Denver, Aurora, CO
An obese 14-year old Hispanic male presented with 24 hours of gross hematuria, right flank pain, and frequent urination with blood present. He had mild, constant dull right flank pain without radiation. His BUN was 37 and creatinine was 3.8. CT scan showed a 3 cm well-circumscribed homogenous, hypervascular mass in the anterior medial-superior pole of the right kidney, which was excised.
Multiple skin colored papules were noted on his cheeks and chin, with some small nodules on the nose, and a hypopigmented macule was present on his back. Multigenerational family members have similar skin lesions and his brother also had seizures.
Case 3 - Slide 1
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Case 3 - Figure 1
Gross photograph showing cut surface of renal mass.
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Case 3 - Figure 2
Low power view shows multiple cysts and abundant chronic interstitial inflammation in the non-neoplastic kidney.
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Case 3 - Figure 3
High power view shows several cysts with variably atypical lining.
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Case 3 - Figure 7 - CAM 5.2
Strong membranous immunoreactivity for CAM 5.2 (Cell Adhesion Molecule 5.2) is present in the tubular component of the tumor.
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Case 3 - Figure 8 - epithelial membrane antigen
Strong membranous immunoreactivity for EMA (Epithelial Membrane Antigen) is present in the tubular component of the tumor.
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Case 3 - Figure 9 - melanoma-associated antigen
The tumor cells do not show immunoreactivity for MAA (Melanoma Associated Antigen).
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Case 3 - Figure 10 - Oil Red O
The tumor cells do not show positivity for Oil Red O.
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Case 3 - Figure 11
A: Ultrastructural analysis shows epithelial cells without abundant lipid. B: High power ultrastructural view shows several cup-shaped, electron-dense granules.
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for Text and References
Submitted by: Liliane Boccon-Gibod - Hopital d'Enfants A. Trousseau, Paris, France
This young boy had unilateral gynecomastia, lentiginosis, and nasal polyposis. A testicular biopsy was performed.
Case 4 - Slide 1
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Case 4 - Figure 1
At low power, cluster of expanded seminiferous tubules with thickened peritubular basement membrane. The stroma is denser than that associated with normal tubules at bottom right
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Case 4 - Figure 2
Intratubular proliferation of large Sertoli cells, with eosinophilic or clear cytoplasm, replacing all germ cells and filling the lumen.
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Case 4 - Figure 3
On the right, two seminiferous tubules that are filled by large Sertoli cells, with eosinophilic or vacuolated cytoplasm and euchromatic, ovoid nuclei. Their thickened basement membrane extends and invaginates into the lumen.
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Case 4 - Figure 4
Among normal seminiferous tubules, one tubule with a PAS-positive, thickened and invaginated basement membrane
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Case 4 - Figure 5
A small cluster of expanded seminiferous tubules with intratubular Sertoli cell proliferation and globular eosinophilic basement membrane deposits, partially calcified in the larger tubule.
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Case 4 - Figure 6
A group of expanded tubules with numerous intraluminal psammomatous calcifications replacing the large proliferated Sertoli cells.
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Case 4 - Figure 7
(Inhibin) Strong inhibin reactivity of the intratubular large proliferated Sertoli cells. In normal seminiferous tubules, weaker reactivity of normal Sertoli cells.
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