PMID- 36149136
OWN - NLM
STAT- MEDLINE
DCOM- 20220928
LR  - 20220928
IS  - 1998-4138 (Electronic)
IS  - 1998-4138 (Linking)
VI  - 18
IP  - 4
DP  - 2022 Jul-Sep
TI  - Malignant gastrointestinal neuroectodermal tumor: A case-based review of 
      literature.
PG  - 885-897
LID - 10.4103/jcrt.JCRT_829_19 [doi]
AB  - Malignant gastrointestinal (GI) neuroectodermal tumor is an extremely rare entity 
      that was first described by Zambrano et al. in 2003 as "clear cell sarcoma 
      (CCS)-like tumor of the GI tract." It shares some of the histopathological 
      features of CCS but lacks the immunohistochemical (IHC) reactivity for 
      melanocytic markers. Most mesenchymal neoplasms of the GI tract belong to the 
      category of GI stromal tumors and are characterized by the IHC expression of 
      c-KIT. In cases, without detectable KIT receptor expression, several differential 
      diagnoses have to be taken into consideration. In this article, we describe such 
      a case and present a review of all the reported cases till date. We also present 
      the current available knowledge on its pathology and molecular genetics along 
      with the limitations in its diagnosis. Here, we report a case of a 32-year-old 
      man with a tumor of the small bowel composed of polygonal tumor cells arranged in 
      solid nests, alveolar pattern, and pseudopapillary and admixed with numerous 
      osteoclast-like multinucleated giant cells. Immunohistochemically, the tumor 
      cells strongly expressed S-100 protein only. HMB-45, melan-A, CD117, cytokeratin, 
      desmin, smooth muscle actin, and CD-34 were absent. Ki-67 index was 15%. The 
      diagnosis was further confirmed by fluorescence in situ hybridization (FISH) 
      demonstrating the presence of EWSR1 (22q12) translocation. A final diagnosis of 
      malignant gastroneuroectodermal tumor was rendered. The patient is disease-free 
      for 20 months of postsurgery. The diagnosis of this entity should be considered 
      in the presence of S-100-positivity and multinucleated osteoclastic giant cells 
      and the absence of melanocytic differentiation in a tumor arising from GI tract. 
      Further confirmation can be done by performing FISH analysis.
FAU - Mishra, Pritinanda
AU  - Mishra P
AD  - Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha, India.
FAU - Biswas, Dipanwita
AU  - Biswas D
AD  - Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha, India.
FAU - Pattnaik, Sandeep Abhijit
AU  - Pattnaik SA
AD  - Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha, India.
FAU - Patra, Susama
AU  - Patra S
AD  - Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha, India.
FAU - Muduly, Dilip Kumar
AU  - Muduly DK
AD  - Department of Surgical Oncology, AIIMS, Bhubaneswar, Odisha, India.
FAU - Balasubiramaniyan, Vigneshwaran
AU  - Balasubiramaniyan V
AD  - Department of Surgical Oncology, AIIMS, Bhubaneswar, Odisha, India.
FAU - Adhya, Amit Kumar
AU  - Adhya AK
AD  - Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha, India.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
PL  - India
TA  - J Cancer Res Ther
JT  - Journal of cancer research and therapeutics
JID - 101249598
RN  - 0 (Actins)
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Desmin)
RN  - 0 (Ki-67 Antigen)
RN  - 0 (MART-1 Antigen)
RN  - 0 (S100 Proteins)
RN  - 68238-35-7 (Keratins)
SB  - IM
MH  - Actins/metabolism
MH  - Biomarkers, Tumor/metabolism
MH  - Desmin/metabolism
MH  - *Gastrointestinal Neoplasms/diagnosis/genetics/pathology
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Keratins
MH  - Ki-67 Antigen/metabolism
MH  - MART-1 Antigen/metabolism
MH  - *Neuroectodermal Tumors/chemistry/diagnosis/genetics
MH  - S100 Proteins/analysis
MH  - *Sarcoma, Clear Cell/diagnosis/pathology/surgery
OTO - NOTNLM
OT  - Clear cell sarcoma
OT  - fluorescence in situ hybridization
OT  - multinucleated giant cells
OT  - translocation
COIS- None
EDAT- 2022/09/24 06:00
MHDA- 2022/09/28 06:00
CRDT- 2022/09/23 09:08
PHST- 2022/09/23 09:08 [entrez]
PHST- 2022/09/24 06:00 [pubmed]
PHST- 2022/09/28 06:00 [medline]
AID - JCanResTher_2022_18_4_885_321714 [pii]
AID - 10.4103/jcrt.JCRT_829_19 [doi]
PST - ppublish
SO  - J Cancer Res Ther. 2022 Jul-Sep;18(4):885-897. doi: 10.4103/jcrt.JCRT_829_19.