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Plexiform fibromyxoma (PF), also referred to as plexiform angiomyxoid myofibroblast tumor, is an exceedingly rare mesenchymal neoplasm primarily affecting the stomach. Herein, we present a case of PF diagnosed in a 71-year-old male with a history of lung cancer, initially suspected to have a gastrointestinal stromal tumor (GIST) of the stomach, who subsequently underwent subtotal gastrectomy. The histopathological and molecular features of the tumor, including mutations in , , , , , and MALAT1-GLI1 fusion, are elucidated and discussed in the context of diagnostic, prognostic, and therapeutic considerations.

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Plexiform angiomyxoid myofibroblast tumour (PAMT) is a rare mesenchymal tumour of the stomach. To date, about 45 cases of PAMT have been reported in the literature. This is an extremely rare mesenchymal gastric tumour with definite histological pattern and is a tumour typified by spindle cells with myofibroblast characteristics.

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Background: Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a rare mesenchymal tumor characterized by multiple nodular plexiform growth patterns and an immunophenotype with myofibroblasts. The pathological characteristics, immunohistochemistry, diagnostic criteria, differential diagnosis, and gene-level changes of PAMT have been reported in many studies. At present, the main treatment for PAMT in the reported cases is surgery; only eight cases were treated endoscopy (excluding 1 thoracoscopic resection), and the lesions were all smaller than 5 cm.

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Objective: This article aimed to study the clinicopathological features, immunophenotypes, and differential diagnoses of plexiform fibromyxoma (PF).

Methods: We searched clinical and pathology databases of our hospital for patients with histologically confirmed PF from 2007 to 2020 and reviewed the relevant English and Chinese language literature.

Results: Two cases of PF were identified, a 67-year-old woman and a 23-year-old man.

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