Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis
Malignant gastrointestinal neuroectodermal tumor (GNET) is an infrequent soft-tissue sarcoma, formerly referred to as clear-cell sarcoma-like gastrointestinal tumor (CCSLGT) and frequently reported in the literature as clear-cell sarcoma of the gastrointestinal tract (CCS-GI); it is characterized by...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-05-01
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| Series: | Surgeries |
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| Online Access: | https://www.mdpi.com/2673-4095/4/2/24 |
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| author | Cinzia Baccaro Noemi Zorzetti Manuela Cuoghi Adele Fornelli Tania Franceschini Sara Coluccelli Vincenzo Cennamo Giuseppe Giovanni Navarra |
| author_facet | Cinzia Baccaro Noemi Zorzetti Manuela Cuoghi Adele Fornelli Tania Franceschini Sara Coluccelli Vincenzo Cennamo Giuseppe Giovanni Navarra |
| author_sort | Cinzia Baccaro |
| collection | DOAJ |
| description | Malignant gastrointestinal neuroectodermal tumor (GNET) is an infrequent soft-tissue sarcoma, formerly referred to as clear-cell sarcoma-like gastrointestinal tumor (CCSLGT) and frequently reported in the literature as clear-cell sarcoma of the gastrointestinal tract (CCS-GI); it is characterized by an absence of melanocytic differentiation and the presence of nontumoral osteoclast-like giant cells (OLGCs). The current study reports a case of a 79 year old woman admitted to the emergency department (ED) with symptoms of constipation and intestinal obstruction; a mass was found within the ileal wall necessitating of surgical approach. Immunohistochemically, tumor cells surprisingly had the hallmark of GNETs. Unfamiliarity with tumors with the features of GNETs can easily lead to a misdiagnosis by surgical pathologist. Therefore, comprehensive evaluation, including morphology and additional studies, is required for an appropriated diagnosis. Furthermore, without a high index of suspicion, there is actually no consensus on staging or treatment. |
| first_indexed | 2024-03-11T01:54:40Z |
| format | Article |
| id | doaj.art-12091c571bf143dcab13052abb24a52d |
| institution | Directory Open Access Journal |
| issn | 2673-4095 |
| language | English |
| last_indexed | 2024-03-11T01:54:40Z |
| publishDate | 2023-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Surgeries |
| spelling | doaj.art-12091c571bf143dcab13052abb24a52d2023-11-18T12:39:42ZengMDPI AGSurgeries2673-40952023-05-014223524510.3390/surgeries4020024Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential DiagnosisCinzia Baccaro0Noemi Zorzetti1Manuela Cuoghi2Adele Fornelli3Tania Franceschini4Sara Coluccelli5Vincenzo Cennamo6Giuseppe Giovanni Navarra7Department of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, ItalyDepartment of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, ItalyDepartment of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, ItalyDepartment of Pathological Anatomy, Maggiore “Carlo Alberto Pizzardi” Hospital, 40133 Bologna, ItalyDepartment of Pathological Anatomy, Maggiore “Carlo Alberto Pizzardi” Hospital, 40133 Bologna, ItalySolid Tumor Molecular Pathology Laboratory, Department of Medical and Surgical Sciences (DIMEC), IRCCS Azienda Ospedaliero-Universitaria, 00144 Bologna, ItalyDepartment of Gastroenterology, Maggiore “Carlo Alberto Pizzardi” Hospital, 40133 Bologna, ItalyDepartment of General Surgery, “A. Costa” Hospital—Alto Reno Terme, 40046 Bologna, ItalyMalignant gastrointestinal neuroectodermal tumor (GNET) is an infrequent soft-tissue sarcoma, formerly referred to as clear-cell sarcoma-like gastrointestinal tumor (CCSLGT) and frequently reported in the literature as clear-cell sarcoma of the gastrointestinal tract (CCS-GI); it is characterized by an absence of melanocytic differentiation and the presence of nontumoral osteoclast-like giant cells (OLGCs). The current study reports a case of a 79 year old woman admitted to the emergency department (ED) with symptoms of constipation and intestinal obstruction; a mass was found within the ileal wall necessitating of surgical approach. Immunohistochemically, tumor cells surprisingly had the hallmark of GNETs. Unfamiliarity with tumors with the features of GNETs can easily lead to a misdiagnosis by surgical pathologist. Therefore, comprehensive evaluation, including morphology and additional studies, is required for an appropriated diagnosis. Furthermore, without a high index of suspicion, there is actually no consensus on staging or treatment.https://www.mdpi.com/2673-4095/4/2/24malignant gastrointestinal neuroectodermal tumorsoft-tissue sarcomaintestinal obstructionileum S-100 protein |
| spellingShingle | Cinzia Baccaro Noemi Zorzetti Manuela Cuoghi Adele Fornelli Tania Franceschini Sara Coluccelli Vincenzo Cennamo Giuseppe Giovanni Navarra Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis Surgeries malignant gastrointestinal neuroectodermal tumor soft-tissue sarcoma intestinal obstruction ileum S-100 protein |
| title | Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis |
| title_full | Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis |
| title_fullStr | Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis |
| title_full_unstemmed | Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis |
| title_short | Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literary Review for a Rare Differential Diagnosis |
| title_sort | malignant gastrointestinal neuroectodermal tumor a case report and literary review for a rare differential diagnosis |
| topic | malignant gastrointestinal neuroectodermal tumor soft-tissue sarcoma intestinal obstruction ileum S-100 protein |
| url | https://www.mdpi.com/2673-4095/4/2/24 |
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