Clinicopathologic features of embryonal tumor with multilayered rosettes and gene analysis on chromosome 19q13.42 / 中华病理学杂志
Chinese Journal of Pathology
; (12): 889-894, 2015.
Article
in Zh
| WPRIM
| ID: wpr-278506
Responsible library:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinicopathologic features and 19q13.42 gene changes in embryonal tumors with multilayered rosettes (ETMR).</p><p><b>METHODS</b>Immunohistochemistry and fluorescence in situ hybridization (FISH) were performed in three ETMRs.</p><p><b>RESULTS</b>The average age of the patients were 34 months. Imaging revealed huge masses with inhomogeneous enhancement and two cases showed cystic lesions. Follow-up data showed 14 and 38 months survival in two children, the third had a recurrence 4 months after operation. Morphologically, the tumor was mainly composed of dense small primitive neuroepithelial cells in patchy or multilayer rosettes within a background of advanced neuronal differentiation, containing neurocytes, ganglion cells, and neuropil-like background. Immunohistochemical staining showed the neuronal marker, synaptophysin, was positive in differentiated areas. Nestin as a neural stem cell marker was immunoreactive in the primitive neuroepithelial cells including multilayered rosettes. Neurons with positive expression of NeuN were observed occasionally. Ki-67 index was up to 40%-80% in the undifferentiated cells and rosettes, but was only 1%-3% in the differentiated areas. CD99 was positive in perivascular papillary pattern areas in one case. 19q13.42 amplification was detected in more than 30% of tumor cells in all cases.</p><p><b>CONCLUSIONS</b>ETMR is a unique entity with distinctive clinical and pathological features. Chromosome 19q13.42 abnormality is valuable for confirming the diagnosis and for further treatment research.</p>
Full text:
1
Index:
WPRIM
Main subject:
Pathology
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Chromosomes, Human, Pair 19
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Immunohistochemistry
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Genetic Testing
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Synaptophysin
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In Situ Hybridization, Fluorescence
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Neoplasms, Germ Cell and Embryonal
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Neuropil
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Antigens, Nuclear
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Genetics
Limits:
Child, preschool
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Humans
Language:
Zh
Journal:
Chinese Journal of Pathology
Year:
2015
Type:
Article