The Malignant Gastrointestinal Neuroectodermal Tumor (GNET): A Distinct Entity and the Challenging Differential Diagnosis with Mesenchymal, Lymphoid, and Melanic Tumors: A Case Report and Brief Review of the Literature

被引:3
作者
Bosoteanu, Madalina [1 ,2 ,3 ]
Cristian, Miruna [1 ,2 ,4 ,5 ]
Aschie, Mariana [2 ,4 ,6 ]
Baz, Radu Andrei [1 ,7 ]
Zielonka, Alina Marta [8 ]
Cozaru, Georgeta Camelia [2 ,4 ]
Bosoteanu, Luana Andreea [5 ,9 ]
机构
[1] Ovidius Univ Constanta, Fac Med, Constanta 900470, Romania
[2] Sf Apostol Andrei Emergency Cty Hosp, Dept Clin Pathol, Constanta 900591, Romania
[3] Ovidius Clin Hosp, Dept Pathol, Constanta 905900, Romania
[4] Ovidius Univ Constanta, Ctr Res & Dev Morphol, Genet Studies Malignant Pathol, CEDMOG, Constanta 900591, Romania
[5] Ovidius Univ Constanta, Inst Doctoral Studies, Sch Med, Constanta 900573, Romania
[6] Acad Med Sci, Bucharest 030167, Romania
[7] Sf Apostol Andrei Emergency Cty Hosp, Dept Radiol, Constanta 900591, Romania
[8] Clin Sante, Bucharest 060754, Romania
[9] Elias Emergency Univ Hosp, Dermatol Dept, Bucharest 011461, Romania
关键词
diagnosis differential; gastrointestinal neoplasms; neuroectodermal tumors; sarcoma; clear cell; CLEAR-CELL SARCOMA; OSTEOCLAST-RICH TUMOR; TRACT; ASSOCIATION; FUSION;
D O I
10.3390/diagnostics13061131
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
(1) Background: A malignant gastrointestinal neuroectodermal tumor (GNET) is an ultra-rare primary neoplasm with a distinctive histopathological, immunohistochemical, molecular, and ultramicroscopic profile, synonymous terminology with clear cell sarcoma-like tumor of the gastrointestinal tract. This case report aims to describe a case of GNET with challenging mesenchymal, lymphoid, and melanic tumor differential diagnosis. (2) Case presentation: We discuss the case of a 67-year-old male patient who presented with diffuse abdominal pain, intermittent lack of intestinal transit, and frequent episodes of nausea, followed by segmental resection of the jejunum and sigmoid colon. The patient had no relevant medical history. The surgical specimen underwent immunohistochemical staining and morphological evaluation. (3) Results: Histopathological analysis reveals a moderately homogeneous polyhedral-epithelioid and spindle cell neoplastic proliferation with a zonal discohesive pattern and extensive and focal fasciculated architecture. Twenty monoclonal antibodies were used for immunostaining, which allowed GNET to be diagnosed on the basis of the tumoral immune profile, characterized by positive reactivity of S100, SOX10, and CD 56. (4) Conclusions: The poor prognosis of GNET is highlighted in the present study, along with the vital importance of differential diagnosis issues with mesenchymal, lymphoid, and melanic tumors, which make the diagnosis difficult for both pathologists and clinicians.
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页数:11
相关论文
共 23 条
[1]   Malignant gastrointestinal neuroectodermal tumor: a case report and review of the literature [J].
Alyousef, Mohammed J. ;
Alratroot, Jumana A. ;
ElSharkawy, Tarek ;
Shawarby, Mohamed A. ;
Al Hamad, Mohammad A. ;
Hashem, Tarek M. ;
Alsayyah, Ahmed .
DIAGNOSTIC PATHOLOGY, 2017, 12
[2]   EWS-CREB1:: A recurrent variant fusion in clear cell sarcoma -: Association with gastrointestinal location and absence of melanocytic differentiation [J].
Antonescu, Cristina R. ;
Nafa, Khedoudja ;
Segal, Neil H. ;
Dal Cin, Paola ;
Ladanyi, Marc .
CLINICAL CANCER RESEARCH, 2006, 12 (18) :5356-5362
[3]   Malignant Gastrointestinal Neuroectodermal Tumor Clinicopathologic, Immunohistochemical, and Molecular Analysis of 19 Cases [J].
Chang, Bin ;
Yu, Lin ;
Guo, Wen-Wen ;
Sheng, Wei-Qi ;
Wang, Lei ;
Lao, Iweng ;
Huang, Dan ;
Bai, Qian-Ming ;
Wang, Jian .
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 2020, 44 (04) :456-466
[4]   Clear cell sarcoma of the ileum: report of a case and review of literature [J].
Comin, Camilla E. ;
Novelli, Luca ;
Tornaboni, Daniela ;
Messerini, Luca .
VIRCHOWS ARCHIV, 2007, 451 (04) :839-845
[5]   EWS-ATF1 fusion transcripts in gastrointestinal tumors previously diagnosed as malignant melanoma [J].
Covinsky, M ;
Gong, S ;
Rajararn, V ;
Perry, A ;
Pfeifer, J .
HUMAN PATHOLOGY, 2005, 36 (01) :74-81
[6]   Malignant Gastrointestinal Neuroectodermal Tumour-Case Report with Review of Literature [J].
Damle, Aditi ;
Sreenija, Yarlagadda ;
Mathews, Niha Rebecca ;
Nair, Krishnanunni ;
Philp, Arun ;
Pavithran, Keechilat ;
Paulose, Roopa Rachel .
JOURNAL OF GASTROINTESTINAL CANCER, 2021, 52 (03) :1125-1130
[7]   Clear cell sarcoma-like tumor with osteoclast-like giant cells in the small bowel:: Further evidence for a new tumor entity [J].
Friedrichs, N ;
Testi, MA ;
Moiraghi, L ;
Modena, P ;
Paggen, E ;
Plötner, A ;
Wiechmann, V ;
Mantovani-Löffler, L ;
Merkelbach-Bruse, S ;
Buettner, R ;
Wardelmann, E .
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY, 2005, 13 (04) :313-318
[8]   Clear cell sarcoma arising in the transverse colon [J].
Fukuda, T ;
Kakihara, T ;
Baba, K ;
Yamaki, T ;
Yamaguchi, T ;
Suzuki, T .
PATHOLOGY INTERNATIONAL, 2000, 50 (05) :412-416
[9]  
Goldblum J.R., 2019, WHO CLASSIFICATION T, VVolume 1
[10]   Clear cell sarcoma of the gastrointestinal tract and malignant gastrointestinal neuroectodermal tumour: distinct or related entities? A review [J].
Green, Celia ;
Spagnolo, Dominic V. ;
Robbins, Peter D. ;
Fermoyle, Soraya ;
Wong, Daniel D. .
PATHOLOGY, 2018, 50 (05) :490-498