PMID- 18418019
OWN - NLM
STAT- MEDLINE
DCOM- 20080529
LR  - 20220331
IS  - 1423-0240 (Electronic)
IS  - 0378-584X (Linking)
VI  - 31
IP  - 4
DP  - 2008 Apr
TI  - Ewing's sarcoma and peripheral primitive neuroectodermal tumor in adults: 
      different features of a rare neoplasm.
PG  - 179-84
LID - 10.1159/000118034 [doi]
AB  - INTRODUCTION: In adults, peripheral primitive neuroectodermal tumors (pPNETs) 
      represent a rare and heterogeneous group of neoplasms exhibiting neuronal and 
      glial differentiation. PATIENTS AND METHODS: We present the clinicopathologic 
      features of four examples of the Ewing's sarcoma (EWS)/pPNET group in adults. 
      Hematoxylin and eosin staining, immunohistochemical and molecular studies were 
      reviewed in every case. Immunohistochemical stains were performed on 
      formalin-fixed, paraffin-embedded sections, molecular studies were done using 
      fluorescence in situ hybridization (FISH). RESULTS: Three patients presented with 
      tumors of the thoracopulmonary region, one patient showed EWS of the soft tissue. 
      Microscopically, tumor tissue was composed of round, small, blue cells with fine 
      granular chromatin texture and inconspicuous nucleoli. Mitotic figures and 
      rosettes were present. Tumor cells strongly coexpressed CD99 and vimentin, but 
      due to technical reasons t(22q12) translocation studies proved the presumptive 
      diagnosis of EWS/pPNET in one case only. Despite similar multimodality treatment 
      survival time ranged from 6 to 42 months, two patients were alive at the time of 
      reporting. CONCLUSIONS: As tumors of the EWS/pPNET family behave aggressively, 
      rapid diagnosis is warranted. Since diagnosis of EWS/pPNET requires ancillary 
      studies, it is necessary to consider it even in adult patients.
CI  - 2008 S. Karger AG, Basel.
FAU - Armbruster, Christine
AU  - Armbruster C
AD  - 2nd Medical Department, Otto Wagner Spital Vienna, Austria. 
      christine.armbruster@gmx.at
FAU - Huber, Monika
AU  - Huber M
FAU - Prosch, Helmut
AU  - Prosch H
FAU - Dworan, Nina
AU  - Dworan N
FAU - Attems, Johannes
AU  - Attems J
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20080320
PL  - Switzerland
TA  - Onkologie
JT  - Onkologie
JID - 7808556
SB  - IM
MH  - Adult
MH  - Bone Neoplasms/*pathology/therapy
MH  - Brain Neoplasms/*pathology/therapy
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Neuroectodermal Tumors, Primitive/*pathology/therapy
MH  - Rare Diseases
MH  - Sarcoma, Ewing/*pathology/therapy
EDAT- 2008/04/18 09:00
MHDA- 2008/05/30 09:00
CRDT- 2008/04/18 09:00
PHST- 2008/04/18 09:00 [pubmed]
PHST- 2008/05/30 09:00 [medline]
PHST- 2008/04/18 09:00 [entrez]
AID - 000118034 [pii]
AID - 10.1159/000118034 [doi]
PST - ppublish
SO  - Onkologie. 2008 Apr;31(4):179-84. doi: 10.1159/000118034. Epub 2008 Mar 20.