BACKGROUND: Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignancy primarily affecting the gastrointestinal tract. Upon cross-sectional imaging, it can be easily confused with other mesenchymal tumors. This article presents a case of duodenal GNET and reviews the current literature on this rare entity.
METHODS: A 73-year-old female patient presented with a 4 cm duodenal mass on CT scan. With a presumptive diagnosis of GIST, a D3-D4 duodenectomy with cholecystectomy were performed. Subsequent pathological analysis of the surgical specimen revealed a 4.5 cm malignant gastrointestinal neuroectodermal tumor (GNET), also known as clear cell sarcoma-like gastrointestinal tumor (CCSLGT).
CONCLUSIONS: While there are less than 115 cases of GNET reported worldwide, prognosis is usually poor with a 50 % survival at 3 years, and mortality rate described is as high as 75 %. To the authors\' knowledge, this duodenal GNET case represents the first one ever described for this location.
CONCLUSIONS: Early recognition of GNET is essential due to its poor prognosis and its ability to metastasize. Awareness of its existence and diagnostic criteria by every member of the medical team is key to obtain optimal patient care.

OBJECTIVE: Schwannoma is a rare mesenchymal tumor. In this study, we analyzed clinicopathologically 99 schwannomas.This retrospective study delves into the clinical, pathological, and immunohistochemical dimensions of abdominal schwannomas.
RESULTS: A cohort of 99 cases, comprising 4 malignant and 95 benign schwannomas, was meticulously examined. Clinical analysis revealed a notable gender distribution (1:1.7, male to female) and an average age of 58.5 years. The majority of cases were asymptomatic. A cohort of 99 cases, comprising 4 malignant and 95 benign schwannomas, was meticulously examined. Clinical analysis revealed a notable gender distribution (1:1.7, male to female) and an average age of 58.5 years. The majority of cases were asymptomatic. Tumor sizes ranged from 0.5 to 30 cm, with distinct locations in the stomach for most benign cases and the abdomen/small intestine for malignancies. Initial misdiagnoses were frequent. Pathological evaluations revealed distinct features, including Antoni A and B patterns, spindle cells, and lymphatic sheath structures in benign schwannomas. Malignant cases exhibited atypical cells, ulcers, and invasive growth. Immunohistochemical markers, such as S100, SOX10, and vimentin, consistently demonstrated positivity by contributing to accurate diagnoses. Treatment outcomes indicated a poor prognosis in malignant cases, with overall survival ranging from 10 to 41 months. Conversely, benign cases displayed no recurrence or metastasis during follow-up, despite atypical behaviors.
CONCLUSIONS: This study underscores the rarity of abdominal schwannomas and underscores the need for a comprehensive diagnostic morphology and immunohistochemistry. SOX10 emerges as a crucial and specific marker for accurate diagnosis. Further research is imperative to refine diagnostic protocols and enhance our understanding of the clinical behavior of abdominal schwannomas.

Gastric neuroendocrine tumors (G-NET) are rare tumors arising from enterochromaffin-like cells of the gastric mucosa. They belong to a larger group called gastroenteropancreatic neuroendocrine tumors and are classified as low, intermediate, or high-grade tumors based on their proliferative indices. They are further categorized into three subtypes based on their morphologic characteristics, pathogenesis, and behavior. Types 1 and 2 tumors are characterized by elevated serum gastrin and are usually multifocal. They typically occur in the setting of atrophic gastritis or MEN1/Zollinger Ellison syndrome, respectively. Type 2 tumors are associated with the most symptoms, such as abdominal pain and diarrhea. Type 3 tumors are associated with normal serum gastrin, are usually solitary, and occur sporadically. This type has the most aggressive phenotype and metastatic potential. Treatment and prognosis for G-NET is dependent on their type, size, and stage. Type 1 has the best prognosis, and Type 3 has the worst. This review discusses the presentation, workup, and surgical management of these tumors.

Gastrointestinal neuroectodermal tumors (GNETs) are extremely rare and intriguing malignancies originating from neural crest cells in the digestive tract. The digestive tract\'s neural crest cells can give rise to incredibly unusual and interesting gastrointestinal neuroectodermal tumors (GNETs). GNETs present considerable hurdles in diagnosis and management because of their rarity and varied expression. In this case report, a 45-year-old male patient is described who had signs of GNET, such as exhaustion, weight loss, and abdominal pain. A 7-cm jejunum tumor and related thickening of the gut wall were discovered using imaging investigations. The diagnosis of malignant GNET was confirmed by surgical resection, and adjuvant treatment was given. A recurring tumor required a second surgical procedure despite an initial disease-free period. The report emphasizes the difficulties involved in the diagnosis, treatment, and long-term effects of GNETs. The rarity of GNETs necessitates the development of standardized treatment protocols as well as additional research to enhance diagnostic precision and explore novel therapeutic approaches for this aggressive malignancy.

Gastrointestinal neuroectodermal tumors (GNETs) are malignant tumors frequently arising within the muscularis propria of the gastrointestinal tract and often extend into the submucosa and subserosa. The stomach is the second most common site of incidence of GNETs after the small intestine. The most important differential of GNET is the clear cell sarcoma-gastrointestinal (CCS-GI). Both share similar morphological as well as molecular features and show S100 positivity; however, the lack of melanocytic differentiation in GNET distinguishes it from CCS-GI. Both typically show rearrangements of the EWSR1 gene, with t(12;22) (q13;q12) EWSR1-ATF1 or t(2;22) (q34;q12) EWSR1-CREB1 fusions. We present a case of a 71-year-old man with an incidentally discovered GNET in the gastric cardia and fundus.

Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignant primary gastrointestinal mesenchymal tumor which can be diagnosed via fine-needle aspiration (FNA) cytology. In the context of FNA, the diagnosis requires a cell block and the use of significant resources including immunohistochemical stains and molecular testing. The differential diagnosis of GNET includes clear cell sarcoma (CCS), gastrointestinal stromal tumor (GIST), gastric schwannoma, metastatic melanoma, malignant perivascular epithelioid cell tumor (PEComa) and granular cell tumor, among others. Here we describe a case which was initially diagnosed as malignant granular cell tumor by FNA which was later revised to GNET following the finding of an EWSR1-ATF1 fusion gene rearrangement.

UNASSIGNED: Gastrointestinal neuroectodermal tumors (GNETs) are uncommon malignant tumors derived from ectodermal primitive neural cells.
UNASSIGNED: We retrospectively analyzed 2 GNET cases at our hospital and the remaining 94 cases in the literature to determine clinicopathological prognostic factors.
UNASSIGNED: 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.
UNASSIGNED: 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.

Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare neoplasm. Immunohistochemically, GNET typically demonstrates neural differentiation but lacks melanocytic differentiation, making it distinct from clear cell sarcoma of the soft tissues (CCS). Herein we report for the first time the cytomorphologic features of lymph node metastasis from presumably liver GNET. A 36-year-old female presented with fevers, night sweats, loss of appetite, and a 20-lbs weight loss. Radiographic imaging showed a 13 cm heterogeneously enhancing mass in the right lobe of the liver and a hypermetabolic 0.9 cm periportal lymph node on positron emission tomography-computed tomography (PET/CT). Initially, a CT-guided liver biopsy was performed followed by right hepatic lobectomy and portal lymphadenectomy. The liver biopsy and resection showed an S100-protein and SOX10 positive malignant neoplasm and genomic profiling of liver biopsy revealed EWSR1-CREB1gene rearrangement. These findings in conjunction with the morphologic and immunohistochemical profile were diagnostic of GNET. Two months later, she presented with recurrent lymphadenopathy in the upper abdomen. Fine needle aspiration of the periportal nodal mass revealed single and clusters of primitive, large to medium-sized neoplastic cells with round to oval nuclei, high nuclear-cytoplasmic ratio, vesicular chromatin, and prominent nucleoli. The tumor cells were S100 protein and SOX10 positive, consistent with metastasis of the patient\'s recently diagnosed malignant digestive system GNET. Palliative chemotherapy was administered but the patient died a few days later, 4 months from the initial diagnosis. Awareness of this entity and judicial use of ancillary studies including molecular testing are essential for achieving accurate diagnosis.

From early 2003 to mid-2013, the total mass of ice in Greenland declined at a progressively increasing rate. In mid-2013, an abrupt reversal occurred, and very little net ice loss occurred in the next 12-18 months. Gravity Recovery and Climate Experiment (GRACE) and global positioning system (GPS) observations reveal that the spatial patterns of the sustained acceleration and the abrupt deceleration in mass loss are similar. The strongest accelerations tracked the phase of the North Atlantic Oscillation (NAO). The negative phase of the NAO enhances summertime warming and insolation while reducing snowfall, especially in west Greenland, driving surface mass balance (SMB) more negative, as illustrated using the regional climate model MAR. The spatial pattern of accelerating mass changes reflects the geography of NAO-driven shifts in atmospheric forcing and the ice sheet\'s sensitivity to that forcing. We infer that southwest Greenland will become a major future contributor to sea level rise.

Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignant tumor. It is also referred to as clear cell sarcoma-like gastrointestinal tumor (CCSLGT). It is an aggressive tumor with a high rate of local recurrence, metastases, and early death from disease. Its pathogenesis is not known. It shows evidence of neural differentiation and lacks immunohistochemical and ultrastructural evidence of melanocytic differentiation. It needs to be distinguished from various mimickers owing to its aggressive course. Herein, we report a case of GNET in a 55-year-old female patient.