title: Primary mediastinal large B-cell lymphoma: clinical study of a distinct clinical entity and treatment outcome in 20 patients: review of the literature.
abstract: Primary mediastinal B-cell lymphoma (PMBCL) is a discrete subset of large B-cell lymphoma with unique clinicopathologic features. The question of optimal treatment emerges because it is an uncommon but not rare occurrence. A retrospective study was therefore conducted in a group of patients in Greece to evaluate the clinical features and treatment outcome in this disease. Twenty patients with PMBCL, with a median age of 42 years, treated at centers participating in the Hellenic Cooperative Oncology Group over the last 20 years, were reviewed. Thirteen (65%) had bulky disease at the time of presentation, 7 (35%) had superior vena cavae obstruction, and 15 (75%) had extranodal involvement. All received doxorubicin-containing chemotherapy, followed in 11 cases by mediastinal radiotherapy. With a median follow-up of 91 months, the median survival is 67.7 months. These data are consistent with those reported from other centers concerning the patient's characteristics, natural history, response pattern to chemoradiation therapy, and prognosis. Response to therapy proved of prognostic significance. A key question that remains is the prompt identification of patients who would benefit from innovative or more intensive therapies.
authors.affiliation: Department of Haematology/Oncology, St. Bartholomew's Hospital, London, UK.
authors.forename: Eleni
authors.initials: E
authors.lastname: Andreopoulou
authors.forename: Dimitris
authors.initials: D
authors.lastname: Pectasides
authors.forename: Meletios A
authors.initials: MA
authors.lastname: Dimopoulos
authors.forename: Costas
authors.initials: C
authors.lastname: Nikolaides
authors.forename: George
authors.initials: G
authors.lastname: Fountzilas
authors.forename: Serena
authors.initials: S
authors.lastname: Valsami
authors.forename: Theofanis
authors.initials: T
authors.lastname: Economopoulos
authors.forename: Helen
authors.initials: H
authors.lastname: Andreopoulou
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000401:mortality / Q000473:pathology / Q000628:therapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008297:Male; D008479:Mediastinal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome