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Case 339 - Discussion |
Uploaded: 2009-09-24, Updated: 2009-09-24 |
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Medulloblastoma, Large cell/Anaplastic variant |
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IPX: synaptophysin scattered positivity, GFAP negative |
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Ependymoma |
Medulloblastoma |
Atypical teratoid/rhabdoid tumor |
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Age |
6.4 years |
7 years |
2 years |
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Location |
4th ventricle |
Vermis, projects into 4th ventricle |
Supra- or infratentorial |
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Gross |
Soft tan, well-circumscribed mass |
Pink, grey mass |
Soft, pink, red, bulky. Often extensive necrosis and hemorrhage. |
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Micro |
Well-circumscribed. Alternative cellular and less cellular areas. Perivascular pseudorosettes, common. True ependymal rosettes, uncommon. Monomorphic, round/oval nuclei, salt and pepper chromatin. Frequent more fibrillarity. Rare or absent mitosis. Common hyalinized vessels and calcification |
Densely cellular, relatively small cell. Round, oval, carrot shape, hyperchromatic. Scant cytoplasm. Many mitoses. Homer-Wright rosettes Ganglioid or ganglion cells Calcification is uncommon |
Sheets of tumor cells Broad and edematous fibrovascular septa. Areas of epithelial and PNET-like Large areas of hemorrhage and necrosis Abundant mitoses Rhabdoid cells |
| WHO Grade | II | IV | IV |
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IPX |
GFAP: + in pseudorosettes. EMA: + dot like S100: + CK AE1/3: focally + CD99: + |
Synaptophysin:+ NSE: + Neurofilament: less commonly + GFAP: + in fibrillar areas |
EMA: + Vimentin: + SMA: + GFAP: + CK: + Synaptophysin occasionally + INI1- |
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EM |
Cilia 9+2 arrangement, luminal microvilli |
Neurite cytoplasmic processes. |
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Cytogenetics |
del(22q11) , NF2 gene, 30% |
del(17p), 30-40% |
Mutation/deletion of INI1 on 22q11 |