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

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 ar...

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Main Authors: Daulath Singh, Mohammed K. Atieh, Mark A. Russell, Muaiad Kittaneh
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2020/8880202
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author Daulath Singh
Mohammed K. Atieh
Mark A. Russell
Muaiad Kittaneh
author_facet Daulath Singh
Mohammed K. Atieh
Mark A. Russell
Muaiad Kittaneh
author_sort Daulath Singh
collection DOAJ
description 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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series Case Reports in Oncological Medicine
spelling doaj-art-82d8f31c51664544b114e2c4d79583e62025-08-20T02:08:46ZengWileyCase Reports in Oncological Medicine2090-67062090-67142020-01-01202010.1155/2020/88802028880202Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based ChemotherapyDaulath Singh0Mohammed K. Atieh1Mark A. Russell2Muaiad Kittaneh3Department of Medicine-Oncology, Loyola University Chicago, 2160 S. First Ave, Maywood, IL 60153, USADepartment of Pathology, Loyola University Chicago, 2160 S. First Ave, Maywood, IL 60153, USADepartment of Pathology, Loyola University Chicago, 2160 S. First Ave, Maywood, IL 60153, USADepartment of Medicine-Oncology, Loyola University Chicago, 2160 S. First Ave, Maywood, IL 60153, USAMalignant 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.http://dx.doi.org/10.1155/2020/8880202
spellingShingle Daulath Singh
Mohammed K. Atieh
Mark A. Russell
Muaiad Kittaneh
Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy
Case Reports in Oncological Medicine
title Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy
title_full Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy
title_fullStr Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy
title_full_unstemmed Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy
title_short Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy
title_sort malignant gastrointestinal neuroectodermal tumor gnet with prolonged disease free survival after platinum based chemotherapy
url http://dx.doi.org/10.1155/2020/8880202
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