PMID- 28320420
OWN - NLM
STAT- MEDLINE
DCOM- 20170327
LR  - 20181113
IS  - 1746-1596 (Electronic)
IS  - 1746-1596 (Linking)
VI  - 12
IP  - 1
DP  - 2017 Mar 20
TI  - Malignant gastrointestinal neuroectodermal tumor: a case report and review of the 
      literature.
PG  - 29
LID - 10.1186/s13000-017-0620-9 [doi]
LID - 29
AB  - BACKGROUND: Malignant gastrointestinal neuroectodermal tumor (GNET) is an 
      extremely rare entity that was first described by Zambrano et al. in 2003 as 
      "Clear cell sarcoma-like tumor of the gastrointestinal tract". It shares some of 
      the histological features of clear cell sarcoma (CCS) but lacks the 
      immunohistochemical reactivity for melanocytic markers. We report a case of GNET 
      that was initially misdiagnosed as gastrointestinal stromal tumor (GIST). 
      Recognizing this entity is important to avoid misdiagnosis. CASE PRESENTATION: A 
      case of an 18-year-old male presented with a small intestinal tumor. 
      Histologically it was characterized by polygonal cells arranged in pseudoalveolar 
      pattern and situated in the muscularis propria. Scattered osteoclast-like 
      multinucleated giant cells were also noted. The neoplastic cells were positive 
      for S-100 protein and negative for HMB-45, Melan A, smooth muscle actin, desmin 
      and CD117. EWSR1 gene rearrangement was detected by fluorescence in situ 
      hybridization (FISH) analysis. The patient returned with recurrence after 
      36 months' management by surgical resection and died one year later. CONCLUSIONS: 
      GNET can be mistaken histologically for other non-epithelial gastrointestinal 
      tumors. Awareness of its existence and diagnostic criteria by the pathologist is 
      necessary to avoid misdiagnosis, particularly as GIST, CCS or malignant 
      peripheral nerve sheath tumor (MPNST).
FAU - Alyousef, Mohammed J
AU  - Alyousef MJ
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia. 
      malyousef@uod.edu.sa.
FAU - Alratroot, Jumana A
AU  - Alratroot JA
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia.
FAU - ElSharkawy, Tarek
AU  - ElSharkawy T
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia.
FAU - Shawarby, Mohamed A
AU  - Shawarby MA
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia.
FAU - Al Hamad, Mohammad A
AU  - Al Hamad MA
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia.
FAU - Hashem, Tarek M
AU  - Hashem TM
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia.
FAU - Alsayyah, Ahmed
AU  - Alsayyah A
AD  - Department of Pathology and Laboratory Medicine, King Fahd Hospital of 
      University, College of Medicine, University of Dammam, Dammam, Saudi Arabia.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20170320
PL  - England
TA  - Diagn Pathol
JT  - Diagnostic pathology
JID - 101251558
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Calmodulin-Binding Proteins)
RN  - 0 (EWSR1 protein, human)
RN  - 0 (RNA-Binding Protein EWS)
RN  - 0 (RNA-Binding Proteins)
SB  - IM
MH  - Adolescent
MH  - Biomarkers, Tumor/analysis/genetics
MH  - Biopsy
MH  - Calmodulin-Binding Proteins/genetics
MH  - Diagnostic Errors
MH  - Fatal Outcome
MH  - Gastrointestinal Stromal Tumors/chemistry/genetics/*pathology
MH  - Gene Rearrangement
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Jejunal Neoplasms/chemistry/genetics/*pathology/surgery
MH  - Male
MH  - Neoplasm Recurrence, Local
MH  - Neuroectodermal Tumors/chemistry/genetics/*pathology/surgery
MH  - Predictive Value of Tests
MH  - RNA-Binding Protein EWS
MH  - RNA-Binding Proteins/genetics
MH  - Time Factors
MH  - Treatment Outcome
PMC - PMC5359837
OTO - NOTNLM
OT  - Clear cell sarcoma-like tumor of gastrointestinal tract
OT  - EWSR1
OT  - Malignant gastrointestinal neuroectodermal tumor
EDAT- 2017/03/23 06:00
MHDA- 2017/03/28 06:00
PMCR- 2017/03/20
CRDT- 2017/03/22 06:00
PHST- 2016/12/29 00:00 [received]
PHST- 2017/03/14 00:00 [accepted]
PHST- 2017/03/22 06:00 [entrez]
PHST- 2017/03/23 06:00 [pubmed]
PHST- 2017/03/28 06:00 [medline]
PHST- 2017/03/20 00:00 [pmc-release]
AID - 10.1186/s13000-017-0620-9 [pii]
AID - 620 [pii]
AID - 10.1186/s13000-017-0620-9 [doi]
PST - epublish
SO  - Diagn Pathol. 2017 Mar 20;12(1):29. doi: 10.1186/s13000-017-0620-9.