Malignant Gastrointestinal Neuroectodermal Tumor

Chang, Bin; Yu, Lin; Guo, Wen Wen; Wei, Sheng; Wang, Lei; Lao, I Weng; Huang, Dan; Bai, Qian; Wang, Jian

doi:10.1097/pas.0000000000001396

Cited by 39 publications

(63 citation statements)

References 29 publications

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“…6 The majority of GNET patients are young adults aged 20 to 40 years. 11 Only 5 patients were over 60 years old, and 1 was under 10 years. 8,12 Distinct from the male predominance of CCS-GIT lesions, women prevailed in the GNET group, with a ratio of 1:1.5 between males and females (P < 0.01).…”

Section: Discussionmentioning

confidence: 98%

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<p>Malignant Gastrointestinal Neuroectodermal Tumors: Clinicopathological and Prognostic Features of 96 Patients</p>

Cao

Chen

et al. 2020

OTT

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Gastrointestinal neuroectodermal tumors (GNETs) are uncommon malignant tumors derived from ectodermal primitive neural cells. Patients and Methods: We retrospectively analyzed 2 GNET cases at our hospital and the remaining 94 cases in the literature to determine clinicopathological prognostic factors. Results: The patients had a mean age of 36 years and a median tumor size of 4.5 cm. A total of 67.0% of the tumors were located in the small intestine, and 76.4% of the patients presented recurrence or metastasis. There was a significant difference in sex and presence of osteoclast-like cells (P<0.01). Microscopically, most cells were round or short spindle-like in shape, with weak eosinophilic or clear cytoplasm. Neoplastic cells were always arranged in solid sheets, nests, and pseudoalveoli. Immunohistochemistry showed strong, diffuse S100 and SOX10 expression, with a complete absence of HMB45 and Melan-A expression. A total of 72.9% of the cases revealed genetic EWSR1 recombination, including our 2 cases. The median time to death and first metastasis was 61 months and 12 months, respectively. K-M analysis showed a great difference in survival according to lymph node invasion or distant metastasis (M+N), independent lymph node metastasis (N), lower histological grades (G2), and aggressive chemoradiotherapy (P=0.026, P=0.027, P=0.039 and P=0.037). However, independent T, independent M, and postoperative routine adjuvant therapy showed no statistical influence on overall survival or disease-free survival. Conclusion: GNET is a new entity distinct in its clinical, morphological, immunochemical, and genetic features. Radical excision, close follow-up and adjuvant therapy may be effective for prolonged survival.

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Section: Discussionmentioning

confidence: 98%

“…21 Drug resistance and the lack of specific targeted treatments are the main problems of clinical treatment. 11 According to the K-M analysis, aggressive chemotherapy or radiotherapy was effective in increasing the time to death but not to first metastasis. Postoperative routine adjuvant therapy showed no effect on survival.…”

Section: Discussionmentioning

confidence: 99%

<p>Malignant Gastrointestinal Neuroectodermal Tumors: Clinicopathological and Prognostic Features of 96 Patients</p>

Cao

Chen

et al. 2020

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“…It is well-known that GNET is a unique clinicopathological entity harboring EWSR1-ATF1/CREB1 fusions, and it can arise at any location in the GI tract, with the small intestine (particularly ileum) being the most common (4,6). According to published literatures, only a minority of cases originated from the upper aerodigestive tract (e.g., tongue, parapharyngeal space, and bronchia) (1, 4, 7-9), which we regard as location extension in a broad sense.…”

Section: Discussionmentioning

confidence: 99%

“…GNET is highly malignant and prone to develop local recurrence and metastasis (3,4,6). At the present, surgical resection is the mainstay of treatment for GNET, and a small number of patients have been reported to show a partial response to apatinib and anlotinib (6).…”

Section: Discussionmentioning

confidence: 99%

Malignant Gastrointestinal Neuroectodermal Tumor in the Right Heart: A Report of an Extremely Rare Case Presenting With a Cardiac Mass

et al. 2021

Front. Cardiovasc. Med.

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Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare soft tissue sarcoma and has been designated as a new entity recently. At present, GNET virtually exclusively occurs in the gastrointestinal tract. Here we report a case of extra-GNET that arose in the right heart. A 62-year-old male complained of chest distress and breathing difficulty while lying down at night for over 1 month at admission. The radiological findings revealed an occupying lesion involving the right atrium and the right ventricle without any abdominal abnormalities. The patient then underwent a surgical resection. Microscopically, neoplastic cells proliferated in the pattern of nests and sheets with fibrous separation. Focal areas with cellular dyscohesion imparted a vague pseudopapillary pattern. These tumor cells were small to medium in size with fine chromatin and predominantly pale eosinophilic cytoplasm. The nuclei were typically round to oval with somewhat irregular contours and contained small nucleoli. The mitotic figures were easily found. Immunohistochemically, the neoplastic cells were positive for S100 and SOX-10 but negative for HMB-45, A103, and CD99. EWSR1–AFTF1 rearrangement was detected by fluorescence in situ hybridization and further confirmed by whole-transcriptome sequence analysis. The patient had pulmonary metastasis 8 months later and soon died of the disease. The overall survival of the patient was 20 months. In summary, we reported an extremely rare case of cardiac GNET, indicating that the location of GNET should not be confined to the GI tract as initially defined. Due to the lack of a specific effective treatment and the occurrence of early metastasis, cardiac GNET conferred a poor prognosis. More clinical and experimental studies are warranted to better manage this disease in the future.

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“…The common management was a radical excision of the tumor with wide lymphadenectomy followed by close monitoring for local recurrence and metastasis. Clinically, there were 3 patients treated with apatinib or anlotinib in these series who experienced preliminary clinical benefit (1 partial response and 1 stable disease to apatinib, 1 partial response to apatinib), indicating that apatinib and anlotinib might be reasonable options for the treatment of advanced GNET and could prolong patient survival [ 9 ]. Another study reported a case of a 30-year-old female with distal ileum GNET s/p resection and adjuvant chemotherapy with ifosfamide and epirubicin.…”

Section: Discussionmentioning

confidence: 99%

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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Malignant Gastrointestinal Neuroectodermal Tumor

Cited by 39 publications

References 29 publications

<p>Malignant Gastrointestinal Neuroectodermal Tumors: Clinicopathological and Prognostic Features of 96 Patients</p>

<p>Malignant Gastrointestinal Neuroectodermal Tumors: Clinicopathological and Prognostic Features of 96 Patients</p>

Malignant Gastrointestinal Neuroectodermal Tumor in the Right Heart: A Report of an Extremely Rare Case Presenting With a Cardiac Mass

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

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