Primary esophageal CD30-positive ALK-positive anaplastic large cell lymphoma with MUM1 expression

Abstract

We report a case of primary esophageal CD30+/ALK+/MUM1+ anaplastic large cell lymphoma (ALCL) and discuss its diagnosis and treatment, specifically in regard to the unusual expression of the B cell marker, MUM1. A 29-year-old Mexican male presented with an esophageal mass with stenosis and dysphagia. Endoscopic samples were sent for hematoxylin–eosin staining and immunohistochemical analysis. Pathologic and immunohistochemical examination resulted in a diagnosis of primary ALCL of the esophagus with CD30+/ALK+/MUM1+ phenotype. This diagnosis was confirmed by molecular analysis [fluorescence in situ hybridization (FISH)]. Initially, two cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy were given, but the patient exhibited progressive disease. Subsequent treatment consisted of four cycles of ifosfamide, carboplatin, and etoposide (ICE). As of the 12-month follow-up examination, the patient is in complete remission. This case suggests that ALCL of the esophagus should be considered in the differential diagnosis of esophageal neoplasms. Biopsy through esophagoscopy or surgical exploration is recommended, and treatment with chemotherapy or radiotherapy can achieve long-term survival.