Malignant gastrointestinal neuroectodermal tumor: A case report and review of the literature.

Abstract:

:Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare soft tissue sarcoma, previously referred to as clear cell sarcoma-like gastrointestinal tumor (CCSLGT) and also commonly reported in the literature as clear cell sarcoma of the gastrointestinal tract (CCS-GI). The current study reports a case of GNET arising in the stomach of a 17-year-old male, who presented with symptoms of fatigue, anemia and low temperature. Examination with positron emission tomography-computed tomography revealed a soft tissue mass in the gastric antrum. Subsequently, radical distal gastric resection was performed, and the mass measured 6.0×4.0×3.5 cm3. Histopathological analysis revealed that the tumor cells were arranged in nests and focally formed fascicular, pseudopapillary, pseudoalveolar and rosette-like growth patterns. Osteoclast-like giant cells were also observed. Immunohistochemically, the tumor cells were positive for S-100 protein, vimentin and BCL-2, and negative for HMB45, Melan-A, CD117, CD34 and CD99. Additionally, the osteoclast-like giant cells were positive for CD68. Fluorescence in situ hybridization demonstrated EWSR1 gene rearrangement. After 10 months of follow-up, no evidence of recurrence or metastasis was observed. As GNET is currently classified differently and under various names in the literature, the information provided by this case study and review is predicted to be useful towards the accurate diagnosis, treatment and prognosis of this rare tumor type.

journal_name

Oncol Lett

journal_title

Oncology letters

authors

Kong J,Li N,Wu S,Guo X,Gu C,Feng Z

doi

10.3892/ol.2014.2524

subject

Has Abstract

pub_date

2014-12-01 00:00:00

pages

2687-2690

issue

6

eissn

1792-1074

issn

1792-1082

pii

ol-08-06-2687

journal_volume

8

pub_type

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