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Neuropathology
Wednesday, March 21, 2012, 7:30 PM
Convention Centre 220-222
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
Surgical Neuropathology: Lessons Learned from Dr. Bernd Scheithauer
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Moderator:
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Daniel Brat
Emory Univ Hosp
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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Arie Perry, University of California, San Francisco, San Francisco, CA
Rebecca D. Folkerth, Brigham & Women’s Hosp, Boston, MA
Gregory N. Fuller, MD Anderson Cancer Center, Houston, TX
Eyas M. Hattab, Indiana University, Indianapolis, IN
Caterina Giannini, Mayo Clinic, Rochester, MN
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Clinical histories are displayed below.
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for Text and References
Submitted by: Arie Perry - University of California, San Francisco, San Francisco, CA
This 51-yo woman presented with new onset of headaches and left hemiparesis. MRI studies revealed a 5 x 3 x 3.5 cm. partially cystic, partially solid contrast enhancing right temporo-parietal mass with surrounding edema and a roughly 1 cm midline shift to the left. The lesion was in close proximity to the lateral ventricle and the radiologist favored a high-grade ependymoma or astrocytoma. A craniotomy and open biopsy was performed.
Case 1 - Slide 1
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Case 1 - Figure 1
Post-gadolinium T1-weighted MRI demonstrating a rim enhancing mass in the right temporo-parietal white matter, including a periventricular region. Evidence of mass effect includes right to left midline shift and compression of the right lateral ventricle. |
Case 1 - Figure 2
Area of pallor with abundant clear foamy cells mimicking macrophages, as well as perivascular lymphocytic cuffing. This focus resembles active inflammatory demyelinating disease. |
Case 1 - Figure 3
Higher magnification image of histiocyte-like clear foamy cells with mild nuclear atypia and increased mitotic activity. |
Case 1 - Figure 4
Focus of tumor cells with granular eosinophilic cytoplasm. |
for Text and References
Submitted by: Rebecca D. Folkerth -
Brigham & Women’s Hosp, Boston, MA
55yo M with hypertrophic cardiomyopathy, status post orthotopic heart transplant 6 weeks prior - On mycophenylate mofetil, prednisone, and cyclosporine - At routine post-op visit, complained of 2 days of frontal headache and lethargy - Headache constant, 8/10, bifrontal, and unaffected by position; alleviated slightly by acetaminophen - No fever, neck stiffness, or vomiting - Wife reported he was less “like himself”, with paucity and slowing of speech, and emotional blunting - No recent travel or sick contacts; cats at home - Employed as a high school woodshop teacher, on leave for several weeks surrounding his heart transplant On examination - T 96.7F - BP 160/70 mmHg - Alert, oriented, and appropriately interactive, though with a reduced range of affect - Occasional paraphasic errors, and difficulty relaying complex portions of his history - Mild flattening of right nasolabial fold - Strength decreased in right triceps (4+/5) and bilateral ankle dorsiflexion (4/5) - Slight right pronator drift and decreased rapid finger- tapping with the right hand Labs: - WBC count 7.36; cyclosporine trough level was 243 ng/mL (therapeutic range: 100-400 ng/mL). Imaging (see Fig 2_a): - Axial T1 with gadolinium showed 3cm lesion with nodular signal heterogeneity - Axial FLAIR showed increased relative cerebral blood volume, moderate vasogenic edema, and 7mm midline shift
Case 2 - Slide 1
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for Text and References
Submitted by: Gregory N. Fuller -
MD Anderson Cancer Center, Houston, TX
A 20-year-old man presented with a two-day history of intermittently slurred speech. There was no history of other signs or symptoms, including no history of headache, nausea, vomiting, motor or sensory deficits, visual disturbance, seizures or altered level of consciousness. A non-contrast CT scan performed in the Emergency Department revealed a 4-cm-diameter heterogeneous mass in the region of the left basal ganglia (putamen and globus pallidus). Subsequent MR imaging demonstrated that the basal ganglia mass displayed heterogeneous contrast enhancement, central hypointense areas of apparent necrosis, and surrounding vasogenic edema. Stereotactic biopsy was performed.
Case 3 - Slide 1
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Case 3 - Figure 1
MRI. Axial T1-weighted image (T1WI) with contrast. A multicystic enhancing mass located in the left basal ganglia region is seen. |
Case 3 - Figure 2
MRI. Coronal T1WI with contrast. A multicystic enhancing mass located in the left basal ganglia region is seen. |
Case 3 - Figure 3
MRI. Sagittal T1WI with contrast. A multicystic enhancing mass located in the left basal ganglia region is seen. |
Case 3 - Figure 4
MRI. Axial T2-weighted image (T2WI). Very little surrounding edema (hyperintensity on T2-weighted sequences) is seen. |
Case 3 - Figure 5
H&E. Intraoperative consultation cytologic smear preparation shows a biphasic population, with large atypical germinoma cells interspersed with small reactive lymphocytes. |
Case 3 - Figure 6
H&E. Intraoperative consultation frozen section confirms the representative nature of the smear prep, with clusters of large atypical germinoma cells interspersed with small reactive lymphocytes. |
Case 3 - Figure 7
H&E. FFPE permanent tissue sections also exhibit characteristic architectural and cytologic features of germinoma. |
Case 3 - Figure 8
CD117 (c-KIT). Germinoma shows strong reactivity for CD117 in a prominent membranous staining pattern with associated cytoplasmic positivity. |
Case 3 - Figure 9
SALL4. The transcription factor SALL4 is strongly expressed in germinoma nuclei. |
Case 3 - Figure 10
OCT4. The currently prerferred marker for confirming a diagnosis of germinoma is the transcription factor OCT4, which is a very robust marker showing strong nuclear positivity. |
Case 3 - Figure 11
Placental alkaline phosphatase (PLAP). Once the mainstay of germinoma immunodiagnosis, PLAP is a relatively weak marker (compare this representative field with similar fields in figures 8, 9 and 10 showing OCT4, c-KIT, and SALL4 reactivity) that has fallen into disuse. |
Case 3 - Figure 12
Beta-hCG. Germinomas are negative for CD30 (which is strongly and diffusely positive in embryonal carcinoma), alpha-fetoprotein (which is strongly and diffusely positive in yolk sac tumor) and beta-hCG (which is strongly and diffusely positive in choriocarcinoma), with the exception that many germinomas contain b-hCG-positive syncytiotrophoblastic cells (these were not seen in the present case). |
for Text and References
Submitted by: Eyas M. Hattab
- Indiana University, Indianapolis, IN
The patient is a 75-year-old Caucasian man with past medical history significant for right shoulder “high grade pleomorphic sarcoma,” including right lower lobe metastasis. He presented to the emergency room with right- sided weakness but refused admission at that time. He had a similar episode approximately 48 hours later, prompting a return to the emergency room and admission. The patient's wife has also noted some word-finding difficulty for approximately a week accompanied by some decrease in appetite and weight loss. An MRI of the brain revealed a 6.4 x 2.5 x 4.5 cm extra-axial, vividly enhancing lesion centered at the high left frontoparietal region, which crossed the midline posteriorly. There were both prominent extradural and subdural components with erosion of the calvarium and elevation of the scalp, as well as an 8mm thick periosteal fluid collection. Susceptibility weighted imaging demonstrated signal changes within the mass consistent with hemorrhage. The patient underwent left parietal craniotomy for tumor resection.
Case 4 - Figure 1
Sagittal and axial T1-weighted images showing an extra-axial, homogenously enhancing, dural-based mass in the parietal parafalcine region (T1 with contrast). |
Case 4 - Figure 2
Low power microscopic image showing a cellular spindle cell tumor with abundant dilated "staghorn" blood vessels distended by blood (hematoxylin and eosin). |
Case 4 - Figure 3
A medium power view illustrating the somewhat haphazard arrangement of the tumor cells around the branching thin-walled blood vessels (hematoxylin and eosin). |
Case 4 - Figure 4
Zones of geographic necrosis, including infiltration by macrophages, were widely present (medium power, hematoxylin and eosin). |
Case 4 - Figure 5
Areas of hemorrhage and necrosis (medium power, hematoxylin and eosin). |
Case 4 - Figure 6
The spindle shaped tumor cells are irregularly arranged surrounding branching capillaries. Note the tumor cell necrosis on the right (high power, hematoxylin and eosin). |
Case 4 - Figure 7
The tumor cell nuclei showing overlap, hyperchromasia and apoptosis (high power, hematoxylin and eosin). |
Case 4 - Figure 8
Irregular bundles of tumor cells with the tumor cell nuclei exhibiting prominent nuclear atypia, including giant multinucleated cells (high power, hematoxylin and eosin). |
Case 4 - Figure 9
Note the frequent mitoses, including an atypical tripolar form (left) (high power, hematoxylin and eosin). |
Case 4 - Figure 10
This tumor is characterized by a rich network of intercellular reticulin deposition (medium power, silver impregnation, reticulin) |
Case 4 - Figure 11
A diffuse immunoreactivity for Bcl-2 was noted (medium power, Bcl-2 immunostain). |
Case 4 - Figure 12
The tumor cells were also immunoreactive for CD99 (medium power, CD99 immunostain). |
Case 4 - Figure 13
An immunohistochemical stain for CD34 highlights the branching capillaries but fails to stain the tumor cells (medium power, CD34 immunostain). |
Case 4 - Figure 14
The tumor cells were negative for smooth muscle actin (high power, SMA immunostain). |
for Text and References
Submitted by: Caterina Giannini -
Mayo Clinic, Rochester, MN
The patient is a 74-year-old man, who began noting intermittent difficulty with expressive language. He had problems coming up with the name of a familiar neighbor as well as remembering the name of an ingredient for stew. There was no associated weakness, alteration in awareness, vision, or sensation. A brain CT and subsequently an MRI were obtained. The CT showed an area of low attenuation in the left temporal lobe with tiny associated area of hyperattenuation. A subsequent MRI showed a left temporal large mildly enhancing lesion with mass effect as well as several other areas of abnormal T2 signal in both hemispheres. He underwent a stereotactic biopsy of the left temporal lobe lesion with the clinical suspicion of low- to intermediate-grade glioma, likely multifocal.
Case 5 - Slide 1
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Case 5 - Slide 2
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Case 5 - Figure 5
Preoperative-CT |
Case 5 - Figure 6
Preoperative-MRI |
Case 5 - Figure 7
A-beta amyloid immunostain shows marked A-beta deposits in the vascular walls as well as a moderate number of amyloid plaques surrounding the vessels. Macrophages are present in the vascular walls. Tau protein is negative. There is no evidence of neuritic plaques or neurofibrillary tangles. |
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