Inflammatory myofibroblastic tumor as adverse outcome of eosinophilic esophagitis

Abstract

An emergency esophagogastroduodenoscopy performed in a dyspeptic 19-year-old girl with hematemesis, abdominal pain, and anemia ([Fig. 1]) revealed a polypoid esophageal mass with distal erosion. A subsequent CT scan confirmed the bulky lesion (14 × 4 × 3.5 cm) extending from the carina to the cardia level. Both extraesophageal extension and nodal and extranodal metastases were excluded. The extent and location of the mass ruled out any endoscopic resection and a total esophagectomy with gastric pull-up was performed. Both histology and immunophenotyping (positive for vimentin, MNF116, and smooth muscle actin; negative for S100 and CD117) were consistent with the “classical variant” of inflammatory myofibroblastic tumor (IMT). A prominent eosinophilic infiltrate ([Fig. 2 a – c]) was also present, spreading from the lesional to the extralesional area, where the squamous epithelium featured rich eosinophic infiltrate, fully consistent with eosinophilic esophagitis ([Fig. 2 d]).