Transcript neurorad-neuropath_12-12

Neuroradiology Neuropathology UNC
Conference, Dec 2012
Josh Gibson, MD
Fellow in Neuroradiology
Case 1, History
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52-year-old male with history of heart
transplant July 2012
Presented in August 2012 with seizure, felt to
possibly be related to tacrolimus
Imaging at time of admission demonstrated a
mass in the fourth ventricle
Surgery initially delayed secondary to recent
heart transplant
Operative findings
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The lesion was somewhat firm and easily
delineated from the surrounding tissues
except at the inferior anterior portion where it
appeared to be attached to the brainstem
with no clear plane. This was initially easily
friable at the surface, but otherwise, resected
by debulking without significant blood loss.
Choroid plexus papilloma
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Choroid plexus tumors (CTTs) are rare, with an annual US incidence of one to three cases
per 10 million people per year, but they account for up to 20% of pediatric brain tumors
arising in the first year of life.
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Lateral or third ventricle in children, often under 2 years of age;
fourth ventricle or the cerebellopontine angle in adults
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Histologically, choroid plexus may be classified as follows:
• Choroid plexus papilloma, WHO grade I (60% of CTTs)
• Atypical choroid plexus papilloma, WHO grade II (15%)
• Choroid plexus carcinoma, WHO grade III (25%)
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Chance of recurrence is greater with atypical adenoma or carcinoma
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Gross total removal is best chance for cure. Malignant progression is rare.
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Children with CPC appear to have a high frequency of TP53 germline mutations in
association with Li–Fraumeni Syndrome (LPS). This raises the question whether all children
with CPC should be tested for TP53 germline mutations in order to institute screening to
enhance early detection and treatment of subsequent cancers.
Burger PC and Scheithauer BW, eds. Diagnostic Pathology: Neuropathology. Amirsis Publishing Inc., 2012.
Paulus W and Janisch W. Acta Neuropathol (1990) 80:635- 641.
Gozali A, et al. Pediatr Blood Cancer 2012;58:905–909.
Wolff JE, Finlay JL. Choroid plexus tumors. In: Carroll WL, Finlay JL, editors. Cancer in children and adolescents.
Massachusetts: Jones and Bartlett Publishers; 2009. p. 299.
Case 2, History
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44-year-old female with right flank and low
back pain with radiation to the right lower
extremity for 2 days
Found to have right paraspinal mass on
outside CT, transferred to our hospital (CT not
available)
Pre Gd
Post Gd
Operative findings
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Vascular tumor with good plane around the
normal anatomy. The mass expanded the
foramen. The bone was thinned out and very
soft.
S100 IHC
Melanotic schwannoma
• Melanotic schwannoma is a pigmented tumor composed of Schwann cells
capable of melanogenesis
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Most commonly occur in spinal nerves, cranial nerves, and autonomic nerves
• Median age at presentation is 38 years
• May be sporadic or associated with Carney complex, which is a multiple
neoplasia syndrome characterized by spotty pigmentation, cardiac myxomas,
endocrine overactivity, and psammomatous melanotic schwannomas.
• Melanotic schwannomas associated with Carney complex typically show
psammoma bodies
• Aggressive or malignant varieties of melanotic schwannoma comprise about
10–25% of cases
Burger PC and Scheithauer BW, eds. Diagnostic Pathology: Neuropathology.
Amirsis Publishing Inc., 2012.
Arvanitis LD. Pathology – Research and Practice 206 (2010) 716–719.
Case 3, History
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62-year-old male with history of AML s/p
bone marrow transplant in 2010
Had resection of cervical ganglioneuroma in
July 2012 and then presented (Sept) with
altered mental status and seizures was found
to have a new right frontal lobe lesion.
Myeloperoxidase IHC
Myeloid sarcoma
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Myeloid sarcoma is a solid tumor composed of extramedullary (outside the bone marrow)
myeloid precursor cells. Previously named “chloroma” due to its greenish hue, a result of
the presence of myeloperoxidase, or “granulocytic sarcoma”.
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Myeloid sarcoma is often associated with acute myeloid leukemia (AML), chronic myeloid
leukemia, or myeloproliferative disorders/myelodysplastic syndrome (MDS).
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Myeloid sarcoma may present prior to or along with bone marrow involvement by
leukemia. Most commonly occurs concomitantly with AML or as the first sign of relapse of
AML. Myeloid sarcoma has been observed in 3–5% of AML.
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In 88% of the patients with no evidence of a hematological abnormality at presentation,
AML develops within 11 months (range 1–49 months); however, in a small group of
patients with myeloid sarcoma, AML never appears.
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Almost any organ can be affected, with the most common being skin, lymph nodes, testis
and bone. The CNS is rarely affected, accounting for approximately 3% of all cases.
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Bone involvement is most frequently seen subperiosteally and is commonly localized in
the vertebrae, sternum, orbits, and cranium.
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Intracranial myeloid sarcomas usually present as extra-axial masses and are believed to
emerge from the bone marrow of the skull.
Widhalm G, et al. J Neurosurg 105:916–919, 2006.
Bain EE, et al. J Cutan Pathol 2012.
Hodges G, et al. 44:573-4, 2012.
Case 4, History
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43-year-old male presented in October 2012
with 2-3 month history of worsening
headaches and blurry vision.
Denied weakness, numbness, personality
change, and seizures.
Operative Findings
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As we proceeded deeper and deeper into the
neoplasm, we did not find an obvious plane
that corresponded to the one seen on the
imaging. We searched carefully for this, in
fact, did not notice any real difference in
consistency between the normal white matter
and the lesion.
Low-grade (grade II) glioma
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Seizure is the presenting symptom in more than 80% of patients with LGG.
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Median age at presentation was 37 years.
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In one series of 1249 grade II gliomas, the histological types were as follows:
24% — astrocytoma
56% — oligodendroglioma
20% — oligoastrocytoma (mixed glioma)
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18% of the LGGs involved the corpus callosum and 5% crossed the midline
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“Incidental” LGGs represented 3.7% of the population of adults presenting with LGGs.
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Incidence of transformation of a LGG to a high-grade glioma ranges from 17% to 73%,
with the timing of this transformation ranging from 2.1 to 10.1 years.
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Oligoastrocytoma has an intermediate prognosis relative to astrocytoma & oligodendroglioma.
Pallud J, et al. Ann Neurol 68:727–733, 2010.
Sanai N, et al. J Neurosurg 115:948–965, 2011
Shaw et al. Neurosurgery 34:577–582, 1994