Giant plexiform fibromyxoma of the esophagus

Plexiform fibromyxoma is a very rare gastrointestinal tumor, usually located in the stomach, predominantly in the antrum region. There are some cases reporting the tumor location in the small intestine, whereas originating from the esophagus has been described only twice up to now. This is the first report of a fast growing and symptomatic giant esophageal plexiform fibromyxoma requiring transthoracic esophagectomy and reconstruction by gastric pull-up.A 73-year-old patient was admitted with the clinical symptoms of dysphagia, chest pain, fever and weight loss for at least 2 months. A computed tomography (CT) scan revealed a giant mediastinal tumor measuring 11 × 7 × 7 cm with subsequent compression of the esophagus, the trachea and the heart. Endoscopy showed an ulcerated esophagus with signs of necrosis and obstruction of the esophageal lumen. A 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) scan detected a massive tracer uptake of this esophageal tumor suggesting malignancy.After discussion in the interdisciplinary tumor board transthoracic esophagectomy and reconstruction by gastric pull-up were performed. The patient fully recovered and was discharged on the 12th postoperative day. Contrary to the intraoperative frozen section indicating an esophageal sarcoma the final histopathological examination confirmed a giant esophageal plexiform fibromyxoma.