title: A prospective study of mediastinal gray-zone lymphoma.
abstract: Mediastinal B-cell lymphomas present in the mediastinum and are most frequent in young patients. Nodular sclerosis Hodgkin lymphoma (NSHL) and primary mediastinal B-cell lymphoma (PMBL) are the common types, whereas mediastinal gray-zone lymphoma (MGZL) is extremely rare and has pathological features intermediate between NSHL and PMBL. The indeterminate pathobiology of MGZL has led to uncertainty regarding therapeutic strategy, and its clinical characteristics and treatment have not been characterized. We conducted a prospective study of infusional dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine, prednisone, and rituximab (DA-EPOCH-R) and filgrastim in untreated MGZL. We analyzed biomarkers of outcome and compared their clinical and biological characteristics to PMBL. Twenty-four MGZL patients had a median age of 33 years (range, 14 to 59 years), and 46% had mediastinal masses ≥10 cm. At 59 months median follow-up, the event-free survival and overall survival were 62% and 74%, respectively. The serum absolute lymphocyte count, the presence of tumor-infiltrating dendritic cells, CD15 expression on the malignant cells, and tumor morphology were biomarkers of outcome in MGZL. Compared with PMBL, MGZL patients were more likely to be male, express CD15, have lower expression of CD20, and have a worse outcome. DA-EPOCH-R alone is effective in MGZL. The trial was registered at ClinicalTrials.gov (NCT00001337).
authors.affiliation: Lymphoid Malignancy Branch.
authors.forename: Wyndham H
authors.initials: WH
authors.lastname: Wilson
authors.affiliation: Laboratory of Pathology.
authors.forename: Stefania
authors.initials: S
authors.lastname: Pittaluga
authors.affiliation: Laboratory of Pathology.
authors.forename: Alina
authors.initials: A
authors.lastname: Nicolae
authors.affiliation: Radiation Oncology Branch, and.
authors.forename: Kevin
authors.initials: K
authors.lastname: Camphausen
authors.affiliation: Lymphoid Malignancy Branch.
authors.forename: Margaret
authors.initials: M
authors.lastname: Shovlin
authors.affiliation: Center for Cancer Research, National Cancer Institute, Bethesda, MD.
authors.forename: Seth M
authors.initials: SM
authors.lastname: Steinberg
authors.affiliation: Lymphoid Malignancy Branch.
authors.forename: Mark
authors.initials: M
authors.lastname: Roschewski
authors.affiliation: Lymphoid Malignancy Branch.
authors.forename: Louis M
authors.initials: LM
authors.lastname: Staudt
authors.affiliation: Laboratory of Pathology.
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.affiliation: Lymphoid Malignancy Branch.
authors.forename: Kieron
authors.initials: K
authors.lastname: Dunleavy
mesh_terms: D000293:Adolescent; D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007167:Immunotherapy; D016393:Lymphoma, B-Cell / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008479:Mediastinal Neoplasms / Q000188:drug therapy* / Q000401:mortality; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D000069283:Rituximab; D016019:Survival Analysis; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D014750:Vincristine; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone