Malignant gastrointestinal neuroectodermal tumor presenting with recurrent ascites

Oye, Monique; Kandah, Fadi; Mikulic, Sebastian; Patel, Bijal; Villabon, Diana Castro; Gopinath, Arun

doi:10.1016/j.cpccr.2020.100017

Cited by 3 publications

(2 citation statements)

References 5 publications

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“…To date, there is still no agreement on the best systemic chemotherapy and targeted therapeutic alternatives for individuals who are not candidates for surgical excision because of significant metastases at initial presentation [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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(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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Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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“…Thus, despite sharing some features with soft tissue clear cell sarcomas, GNET was defined as a new tumor entity with specific clinical, histologic, immunohistochemistry, and molecular characteristics (2). Nevertheless, challenges remain for diagnosis, staging, treatment, and management, mainly due to the rarity of this disease and the limited to small case reports and case series having been described to date (2)(3)(4). Herein, we report a case of GNET that is a 22-year-old male, who had previously received chemotherapy for metastatic primitive neuroectodermal tumor (PNET) and subsequently developed an abdominal mass.…”

Section: Introductionmentioning

confidence: 99%

Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literature Review

Ahadi,

Ziafat,

Zahedifard

et al. 2023

Int J Cancer Manag

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Introduction: The gastrointestinal neuroectodermal tumor (GNET) is a rare mesenchymal tumor mainly arising in the gastrointestinal tract. Case Presentation: Herein, we describe a case of GNET that had been previously diagnosed as metastatic primitive neuroectodermal tumor (PNET) to the lung and liver and received 30 cycles of adjuvant chemotherapy. The physical exam showed a palpable mass in the lower right quadrant of the abdomen. The tumor mass was composed of diffuse sheets of epithelioid tumor cells, in which osteoclast-like giant cells scattered unevenly. Immunohistochemically, the tumor cells were diffusely positive for SOX10, CD99, and CD56, patchy positive for pan-CK, and negative for desmin, HMB-45, Melan-A, and chromogranin. Conclusions: Consistent with previous studies that reported a secondary GNET following chemotherapy, we assume that the chemotherapy might trigger the tumorigenesis of GNET in this case.

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Malignant Gastrointestinal Neuroectodermal Tumour—Case Report with Review of Literature

Damle

Yarlagadda

Mathews

et al. 2021

J Gastrointest Canc

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Malignant gastrointestinal neuroectodermal tumor presenting with recurrent ascites

Cited by 3 publications

References 5 publications

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

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

Malignant Gastrointestinal Neuroectodermal Tumor: A Case Report and Literature Review

Malignant Gastrointestinal Neuroectodermal Tumour—Case Report with Review of Literature

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