Malignant gastrointestinal neuroectodermal tumour (GNET): neural mesenchymal tumours of the gastrointestinal tract with striking histology and EWSR1 gene rearrangement

Toon, Christopher W.; Cooper, Wendy A.; Selinger, Christina; Berney, Christophe R; Tomlinson, Jeanne

doi:10.1016/j.pathol.2018.09.066

Cited by 3 publications

(4 citation statements)

References 7 publications

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“…Malignant gastrointestinal neuroectodermal tumors (GNETs) are rare primitive mesenchymal neoplasms of the tubular gut previously considered to be gastrointestinal manifestations of soft tissue clear cell sarcomas (CCS) [ 1 ]. GNETs were previously known as CCSLTGT (with osteoclast-like giant cells) given their overlapping histological, immunophenotypical, and molecular features with soft tissue clear cell sarcomas.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…GNETs are postulated to arise from primitive neural crest progenitor cells of the gastrointestinal tract autonomic nervous system. The prognosis appears poor, although published data with follow-up information are scant, given the rarity of this disease [1]. GNET was first described in 2003 by Zambrano et al who reported 6 cases as malignant mesenchymal neoplasm of gastrointestinal tract that were characterized by the presence of osteoclast-like multinucleated giant cells that expressed S100 protein, but staining for CD117 and melanocytic markers was negative by immunohistochemistry.…”

Section: Discussionmentioning

confidence: 99%

“…Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare primitive mesenchymal neoplasm of the gut. It was previously known as clear cell sarcoma-like tumor of the GI tract (CCSLTGT) [ 1 ]. In the recent years, it has been recognized as a separate entity with specific histologic, immunohistochemistry, and molecular features [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy

Singh

Atieh

Russell

et al. 2020

Case Reports in Oncological Medicine

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Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare disease with a handful of cases described in literature. GNET has only become a well-known/widely accepted entity in the recent years, but it is still not listed in the database of rare diseases. Due to the rarity of disease, there are no guidelines on standard therapeutic approaches in the adjuvant or metastatic setting. Here, we describe a unique case of GNET with a 7-year disease-free survival following adjuvant cisplatin and etoposide chemotherapy. This is the longest disease-free survival that has ever been described in literature and may support using this combination in a larger cohort of patients in the context of a global clinical trial. We will also review the histopathologic features of GNET and potential therapeutic options in the metastatic setting.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy

Singh

Atieh

Russell

et al. 2020

Case Reports in Oncological Medicine

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Malignant gastrointestinal neuroectodermal tumor-A case report

Saishalini

Pavithra

et al. 2021

Indian Journal of Pathology and Microbiology

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Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare neoplasm with unknown etiology. It was previously referred to as Clear cell sarcoma of gastrointestinal tract. This tumor is characterized by a higher rate of local recurrence and metastasis. Due to its aggressive clinical course, distinguishing this entity from various other mimickers is very essential. Herein, we present a case of malignant GNET in a 33-year-old male patient.

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Malignant gastrointestinal neuroectodermal tumor

Mishra

Biswas

Pattnaik

et al. 2022

Journal of Cancer Research and Therapeutics

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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.

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Malignant gastrointestinal neuroectodermal tumour (GNET): neural mesenchymal tumours of the gastrointestinal tract with striking histology and EWSR1 gene rearrangement

Cited by 3 publications

References 7 publications

Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy

Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy

Malignant gastrointestinal neuroectodermal tumor-A case report

Malignant gastrointestinal neuroectodermal tumor

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