期刊
BLOOD
卷 121, 期 8, 页码 1394-1402出版社
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2012-08-452284
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资金
- Instituto de Salud Carlos III, Fondo de Investigaciones Sanitarias [PI08/0077, PI11/01177]
- Alexander von Humboldt Foundation
- Subprograma Juan de la Cierva Ministerio de Ciencia e Innovacion [JCI-2011-10232]
- Instituto de Salud Carlos III, Beca Predoctoral de Formacion en Investigacion en Salud Comision Interministerial de Ciencia y Tecnologia Espanola [FI08/00347, SAF08/3630]
- Red Tematica de Investigacion Cooperativa del Cancer [RD06/0020/0039 ISCIII]
- Spanish Ministry of Science and Innovation
- European Regional Development Fund (Unio Europea Una manera de fer Europa)
Cyclin D1(-) mantle cell lymphomas (MCLs) are not well characterized, in part because of the difficulties in their recognition. SOX11 has been identified recently as a reliable biomarker of MCL that is also expressed in the cyclin D1(-) variant. We investigated 40 lymphomas with MCL morphology and immunophenotype that were negative for cyclin D1 expression/t(11;14)(q13;q32) but positive for SOX11. These tumors presented clinically with generalized lymphadenopathy, advanced stage, and poor outcome (5-year overall survival, 48%). Chromosomal rearrangements of the CCND2 locus were detected in 55% of the cases, with an IG gene as partner in 18 of 22, in particular with light chains (10 IGK@ and 5 IGL@). No mutations in the phosphorylation motifs of CCND1, CCND2, or CCND3 were detected. The global genomic profile and the high complexity of the 32 cyclin D1(-)SOX11(+) MCL patients analyzed by copy number arrays were similar to the conventional cyclin D1(+)/SOX11(+) MCL. 17p deletions and high Ki67 expression conferred a significantly worse outcome for the patients. This comprehensive characterization of a large series of cyclin D1(-) MCL patients indicates that these tumors are clinically and biologically similar to the conventional cyclin D1(+) MCL and provides a basis for the proper identification and clinical management of these patients.
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