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Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:

2023 22

Page:

4179-4183

Research Field:

Publishing date:

Info

Title:

Clinicopathological observation of malignant gastrointestinal neuroectodermal tumor

Author(s):

NIE Jia¹; YANG Jie²; ZHANG Shiyong³; LIU Qian¹; YUAN Fengju¹; DONG Xu¹; YANG Zhirong¹

1.Department of Pathology;2.Department of Hepatobiliary and Pancreatic Surgery;3.Department of Radiology，Deyang People's Hospital,Sichuan Deyang 618000,China.

Keywords:

malignant gastrointestinal neuroectodermal tumor; gastrointestinal clear cell sarcoma (CCS) like tumor; EWSR1; ATF1; CREB1

PACS:

R735.2

DOI:

10.3969/j.issn.1672-4992.2023.22.015

Abstract:

Objective:To investigate the clinicopathological and molecular characteristics,diagnosis,differential diagnosis and prognosis of malignant gastrointestinal neuroectodermal tumor (MGNET),in order to improve the ability to diagnose of this disease.Methods:Two cases of MGNET diagnosed in our hospital were reported.The clinical and imaging features,histomorphology,immunophenotype,molecular genetics and prognosis were analyzed,and the relevant literature was reviewed.Results:Case 1 and case 2 were women aged 28 and 45 years old.CT showed pelvic mass and annular thickening of the ascending colon wall.Pathologically,both tumors were mainly located in the intestine.Microscopically,the short fusiform tumor cells arranged into flake,nest,acinous,and pseudo-papillary forms were mixed with scattered osteoclast-like multinucleated giant cells.Immunohistochemistry showed S-100(+) in both tumors.Fluorescence in situ hybridization (FISH) showed EWSR1 (22q12) translocations in both tumors.MGNET was eventually diagnosed in 2 cases.At 6 months after surgery,CT showed nodular thickening of peritoneum in case 1.In case 2,PET-CT showed multiple tumor metastases in the liver and abdominal cavity at 16 months after surgery,and she died 19 months later.Conclusion:MGNET is a rare and highly invasive soft tissue sarcoma.This kind of rare tumor should be taken into account in clinical work.The immunohistochemical indexes and genetic tests should be reasonably selected in combination with histological morphology to make accurate diagnosis.At present,surgical excision is the main treatment method,and there is no consensus on chemotherapy after surgical excision.

References:

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