PMID- 26888507 OWN - NLM STAT- MEDLINE DCOM- 20160706 LR - 20181202 IS - 0529-5807 (Print) IS - 0529-5807 (Linking) VI - 44 IP - 12 DP - 2015 Dec TI - [Clinicopathologic features of embryonal tumor with multilayered rosettes and gene analysis on chromosome 19q13.42]. PG - 889-94 AB - OBJECTIVE: To investigate the clinicopathologic features and 19q13.42 gene changes in embryonal tumors with multilayered rosettes (ETMR). METHODS: Immunohistochemistry and fluorescence in situ hybridization (FISH) were performed in three ETMRs. RESULTS: 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. CONCLUSIONS: 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. FAU - Wang, Junmei AU - Wang J AD - Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing 100050, China; E-mail: wwwjjjmmm1180@sina.com. FAU - Liu, Zhaoxia AU - Liu Z FAU - Fang, Jingyi AU - Fang J FAU - Chen, Qian AU - Chen Q FAU - Du, Jiang AU - Du J FAU - Xu, Li AU - Xu L FAU - Li, Guilin AU - Li G LA - chi PT - Journal Article PL - China TA - Zhonghua Bing Li Xue Za Zhi JT - Zhonghua bing li xue za zhi = Chinese journal of pathology JID - 0005331 RN - 0 (Antigens, Nuclear) RN - 0 (Nerve Tissue Proteins) RN - 0 (Synaptophysin) RN - 0 (neuronal nuclear antigen NeuN, human) SB - IM MH - Antigens, Nuclear/genetics MH - Child, Preschool MH - Chromosomes, Human, Pair 19/*genetics MH - Genetic Testing MH - Humans MH - Immunohistochemistry MH - In Situ Hybridization, Fluorescence MH - Neoplasm Recurrence, Local MH - Neoplasms, Germ Cell and Embryonal/*genetics/*pathology MH - Nerve Tissue Proteins/genetics MH - Neuropil/pathology MH - Synaptophysin/genetics EDAT- 2016/02/19 06:00 MHDA- 2016/07/07 06:00 CRDT- 2016/02/19 06:00 PHST- 2016/02/19 06:00 [entrez] PHST- 2016/02/19 06:00 [pubmed] PHST- 2016/07/07 06:00 [medline] PST - ppublish SO - Zhonghua Bing Li Xue Za Zhi. 2015 Dec;44(12):889-94.