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Pediatric Pathology
Sunday, March 8, 2009, 7:30 PM
Convention Center 309
Clinical histories are printed below.
Click on the case numbers for text and references of each case.
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Pediatric Pathology in Boston
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Moderator:
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CHERYL M. COFFIN
Vanderbilt University School of Medicine, Nashville, TN
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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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SARA O. VARGAS, Children's Hospital, Boston, MA
PAVEL J. JEDLICKA, The Children's Hospital, Aurora, CO
ROBERT B. FRASER, IWK Health Centre, Halifax, NS, Canada
LOUIS P. DEHNER, Washington University Medical Center, St. Louis, MO
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Clinical histories are displayed below.
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for Text and References
Submitted by: Sara O. Vargas - Children's Hospital, Boston, MA
This 9-year-old boy had an incidentally discovered lung mass. He had been a healthy active child, and one day while swimming in a pool he came up for air directly in front of a chlorine jet. This caused him to cough and feel as though his throat was closing, and soon thereafter his parents brought him to his pediatrician. At this visit, the patient appeared well, and physical examination was unremarkable. A chest radiograph showed a left intrathoracic mass. A CT scan better defined the mass as a left upper lobe peripheral heterogeneous mass measuring 4.0 x 3.8 x 3.6 cm; enlarged mediastinal lymph nodes were also seen.
The patient lived in a suburban Midwestern environment and had no significant travel history. His parents, a lawyer and a restauranteur, were nonsmokers. There was no family history of cancer.
A needle biopsy of the pulmonary mass was interpreted as showing malignancy. A week later, the patient underwent thoracoscopic resection of the lingular mass and a mediastinal lymph node. Grossly, the lung contained a well circumscribed and slightly cystic tan mass (4.1 x 3.8 x 2 cm). The mediastinal lymph node was enlarged (3 cm).
Case 1 - Slide 1
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for Text and References
Submitted by: Robert B. Fraser - IWK Health Centre, Halifax, NS, Canada
A 6-year old boy presented with a one-week history of intermittent abdominal pain and abdominal swelling. No other constitutional symptoms. Past medical history was unremarkable and there was no significant family history or associated dysmorphic features. CT of the chest and abdomen confirms the presence of multiple pulmonary nodules and a large left-sided renal mass. He was admitted to hospital for a left nephrectomy (Case 2 - Figures 1, 2, 3). Following surgery he received chemotherapy with pulmonary and flank radiotherapy. Eighteen months off therapy, he presented with a 6-week history of a limp and an x-ray, CT and bone scan demonstrated a lytic lesion in the right proximal tibia. A bone biopsy of the leg lesion was undertaken (Case 2 - Figures 4, 5, 6).
for Text and References
Submitted by: Paul Jedlicka - The Children’s Hospital, Aurora, CO
A previously healthy 2-year old female presented with a 1-month history of progressive bilateral lower extremity weakness and a neurogenic bladder. Imaging revealed an epidural tumor, which was biopsies and debulked to relieve the symptoms of spinal cord compression. Metastatic work-up was negative. She underwent chemotherapy and radiotherapy with good response and is currently in disease remission 4 years later.
Case 3 - Slide 1
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Case 3 - Figure 1
Touch preparation performed on tumor biopsy tissue, showing discohesive population of relatively uniform, small to medium-sized cells with high nuclear/cytoplasmic ratio and apoptotic activity.
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Case 3 - Figure 2
Low-power histomorphologic features of tumor, showing a dense, small blue cell population with patchy necrosis, infiltrating fibrocollagenous tissue.
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Case 3 - Figure 3
Intermediate-power histomorphologic features of tumor, showing a relatively uniform cell population with clear cytoplasm.
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Case 3 - Figure 4
High-power histomorphologic features of tumor, showing cells with fine chromatin, inconspicuous nuclei, amphophilic to clear cytoplasm and scattered apoptotic activity.
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Case 3 - Figure 5
CD99 immunohistochemical staining of tumor, showing uniform membranous staining of tumor cells in well-preserved areas.
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Case 3 - Figure 6
Higher-power view of tumor CD99 immunostaining.
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Case 3 - Figure 7
Ultrastructural features of tumor, showing cells with high nuclear-cytoplasmic ratio, and organelle-poor cytoplasm containing abundant glycogen in the polyparticulate form.
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for Text and References
Submitted by: Louis P. Dehner - Washington University Medical Center,
St. Louis, MO
The patient is a 5-year-old female who was seen in the emergency department with a 4-5 day history of moderate to severe, cramping intermittent pain in the periumbilical region. There was regularity to the pain especially over the prior two days. She had had several bouts of vomiting over the last 24 hours. Physical examination revealed an afebrile child who did not appear to be in acute distress, was well nourished and was normally developed without any signs of endocrine dysfunction. Thoracic and abdominal scars were noted. The physical examination was otherwise unremarkable including a negative abdominal examination.
A hemogram was performed with a hemoglobin of 12.1 g/dl and a white cell count of 16,500 with 85% segmented neutrophils. CT examination revealed a multicystic mass in the left kidney measuring 4.0 x 2.5 cm and a rounded soft tissue mass in the left pelvis immediately adjacent and anterior to the rectum which measured 3.4 x 3.2 cm. This mass was questioned as possibly arising in the left ovary.
The pre-operative diagnosis was torsion of the left ovary. At the time of surgery, there was torsion of the left ovary with an intensely hemorrhagic appearance to the ovary as well as the presence of a 3-4 cm mass which was contiguous to the ovary.
The section is from the resected left ovary which was described as weighing 48 gm and a lobulated, hemorrhagic mass measuring 8 x 4 x 2 cm.
Case 4 - Figure 1
Hemorrhagic external surface and hemorrhagic contents of the cystic left ovary also demonstrates bulging nodules as seen through the capsular surface.
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Case 4 - Figure 2
Extensive areas of the left ovary have undergone hemorrhagic necrosis which is the typical pathology of torsion. In some cases, it may be difficult to appreciate any underlying additional pathology, but not in this specimen.
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Case 4 - Figure 3
Though the nodules of tumor also displayed the presence of interstitial hemorrhage, there is sufficient preservation to appreciate both architectural and individual microscopic features. The multinodular character is appreciated in this low magnification view. There is variation in the histologic composition of this tumor from one area to another.
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Case 4 - Figure 4
This microscopic field shows the presence of uniformly small tubules adjacent to the loosely textured spindle cell mesenchyme.
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Case 4 - Figure 5
Other foci are composed of well formed tubules and poorly formed cord-like structures.
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Case 4 - Figure 6
This microscopic field shows the presence of poorly formed cords of basophilic cells which blend into a fibroma-like stroma.
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Case 4 - Figure 7
Cystic areas show the presence of papillary profiles whose papillae are lined by a similar population of basophilic cells seen elsewhere in the tubules.
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Case 4 - Figure 8
Immunohistochemistry confirms the diagnosis of a sex-cord stromal tumor with a panel of stains including cytokeration.
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Case 4 - Figure 9
Vimentin expression by the tumor cells is less intense than the cytokeratin in Figure 8.
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Case 4 - Figure 10
Calretinin staining is focally positive in tumor cells. The immunophenotype of sex-cord stromal tumors has some overlap with surface epithelial tumors of the ovary and mesothelioma except for the expression of inhibin. These three tumor types have considerable overlap since they share a common embryogenesis.
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Case 4 - Figure 11
Inhibin positivity is a useful marker in the differentiation of a sex-cord stromal tumor from a surface epithelial neoplasm of the ovary.
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Case 4 - Figure 12
The tumor demonstrates strong and diffuse nuclear reactivity for WT-1.
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