TY - JOUR
T1 - Malignant Gastrointestinal Neuroectodermal Tumor
T2 - A New Kid on the Block?
AU - Morani, Ajaykumar C.
AU - Ramani, Nisha S.
AU - Yedururi, Sireesha
AU - Prasad, Srinivasa R.
N1 - Publisher Copyright:
© Wolters Kluwer Health, Inc. All rights reserved.
PY - 2022/9/1
Y1 - 2022/9/1
N2 - Also referred to as "osteoclast-rich, clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT),"malignant gastrointestinal neuroectodermal tumor is a newly described, rare, aggressive sarcoma that commonly arises in the small bowel, stomach, and colon. Histogenesis is likely from an autonomous nervous system-related primitive cell of neural crest origin. The hallmark genetic finding of EWS-CREB1 or EWS-ATF1 fusion transcripts clinches the diagnosis. Annular constrictive lesions tend to be smaller, show homogenous contrast enhancement on computed tomography, and may present with bowel obstruction. Larger, expansile masses tend to be exophytic and show heterogeneous contrast enhancement. Surgical resection is the mainstay of treatment. Frequent recurrences, metastases, and death from disease in 75% of patients portend a poor prognosis. Targeted chemotherapy based on specific tumor pathways is being developed.
AB - Also referred to as "osteoclast-rich, clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT),"malignant gastrointestinal neuroectodermal tumor is a newly described, rare, aggressive sarcoma that commonly arises in the small bowel, stomach, and colon. Histogenesis is likely from an autonomous nervous system-related primitive cell of neural crest origin. The hallmark genetic finding of EWS-CREB1 or EWS-ATF1 fusion transcripts clinches the diagnosis. Annular constrictive lesions tend to be smaller, show homogenous contrast enhancement on computed tomography, and may present with bowel obstruction. Larger, expansile masses tend to be exophytic and show heterogeneous contrast enhancement. Surgical resection is the mainstay of treatment. Frequent recurrences, metastases, and death from disease in 75% of patients portend a poor prognosis. Targeted chemotherapy based on specific tumor pathways is being developed.
KW - CCSLGT
KW - MGNET
KW - clear cell sarcoma-like tumor of the gastrointestinal tract
KW - malignant gastrointestinal neuroectodermal tumor
KW - small bowel malignancy
UR - http://www.scopus.com/inward/record.url?scp=85138448565&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85138448565&partnerID=8YFLogxK
U2 - 10.1097/RCT.0000000000001350
DO - 10.1097/RCT.0000000000001350
M3 - Article
C2 - 35759779
AN - SCOPUS:85138448565
SN - 0363-8715
VL - 46
SP - 676
EP - 681
JO - Journal of computer assisted tomography
JF - Journal of computer assisted tomography
IS - 5
ER -