Endoscopic Mucosal Resection (EMR) for the Management of Solitary Extramedullary Plasmacytoma of the Esophagus

Abstract

Introduction: We present a first report of EMR for management of Solitary Extramedullary Plasmacytoma (EMP) of the esophagus. Case Report: A 50-year-old man with chronic intermittent dysphagia secondary to eosinophilic esophagitis (EoE) was referred for management of an incidental mid-esophageal mass which was biopsy proven to be an EBV+ plasma cell neoplasm. Extensive work-up for an underlying plasma cell dyscrasia including multiple myeloma confirmed this to be a solitary EMP. Endoscopic reassessment demonstrated an irregular sessile 1.0-1.5 cm mid-esophageal nodule at 25 cm. EUS showed a hypoechoic nodule involving the mucosa and submucosa with intact muscularis propria. The lesion was successfully resected by EMR using a multi-band mucosectomy device without submucosal injectate. The primary lesion was resected with a single band ligation although a second ligation was performed given a grossly close margin. No additional thermal ablation therapy was used. No endoclip closure was required. No immediate or delayed complications were observed. Pathologic and immunohistochemistry assessment was compatible with solitary EMP with marked expansion of the submucosa by sheets of large plasmacytoid cells without blastic appearance. Despite EBV+, there were no features of plasmablastic lymphoma. Peripheral and deep resected margins from the primary band ligation were free of tumor. Only 5-13% of EMPs occur in the gastrointestinal tract. Standard therapy for solitary plasmacytoma involving extramedullary sites involves definitive radiation therapy, followed by surgery if necessary with an overall good prognosis. Endoscopic management has not been well described given limited data. Only one prior case report has described successful endoscopic management of a subcentimeter mucosal EMP using endoscopic submucosal resection (ESD). However this technique is not yet widely performed and is associated with a higher risk for perforation. EMR is already widely used for the diagnostic and therapeutic management of mucosal and submucosal neoplastic lesions. EMR appears to be a feasible and less invasive alternative to radiation and surgery for select small, well defined EMP lesions limited to the mucosa and submucosa of the esophagus. EUS can help assess those lesions appropriate for EMR. Long term follow up of endoscopic treatment of solitary esophageal EMPs is needed. Surveillance EGD with biopsies will be repeated at three months. Repeat endoscopic management, radiation and surgery remain potential salvage therapeutic options.