title: Lymphoblastic lymphoma: an updated review on biology, diagnosis, and treatment.
abstract: Lymphoblastic lymphoma is a rare aggressive neoplasm of T-/B-precursors resembling acute lymphoblastic leukemia, with no or limited bone marrow involvement (<25%), that develops more frequently in children and young adults and is typically characterized by a grossly enlarged mediastinum, and whose diagnostic hallmark is the expression of a T-/B-precursor cell immunophenotype, the T-cell subset accounting for 90% of all cases. The adoption of pediatric-derived, intensive lymphoblastic leukemia-like protocols led to significantly improved results, with survival rates of about 70% and 90% in adults and children, respectively. Adequate central nervous system prophylaxis and mediastinal irradiation contributed to the therapeutic success; however, the role of radiation therapy is debated due to toxicity concerns and the excellent results obtained with radiation-free programs especially in pediatric patients. With these modern schedules, localized radiotherapy and/or hematopoietic stem cell transplants could be generally omitted, and considered only for high-risk patients identified through postinduction computed tomography/positron-emission tomography scans, minimal residual disease analysis, and new genetics and genomics. New clinical studies will have to confirm the value of these assays for risk-oriented therapy, while further therapeutic progress is expected from the introduction of new drugs and targeting agents.
authors.affiliation: Unità Operativa Complessa di Ematologia, Ospedale dell'Angelo & Ospedale SS. Giovanni e Paolo, Mestre-Venezia, Italy.
authors.forename: Renato
authors.initials: R
authors.lastname: Bassan
authors.affiliation: Unità Operativa Complessa di Ematologia, Ospedale dell'Angelo & Ospedale SS. Giovanni e Paolo, Mestre-Venezia, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Maino
authors.affiliation: Unità di Oncologia, Cliniche Humanitas/Gavazzeni, Bergamo, Italy.
authors.forename: Sergio
authors.initials: S
authors.lastname: Cortelazzo
mesh_terms: D000818:Animals; D015415:Biomarkers; D003131:Combined Modality Therapy; D003952:Diagnostic Imaging; D055808:Drug Discovery; D019008:Drug Resistance, Neoplasm; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing; D006801:Humans; D009367:Neoplasm Staging; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy*; D012008:Recurrence; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D015415:Biomarkers
keywords: biology; diagnosis; lymphoblastic lymphoma; risk factors; treatment