PMID- 11688461
OWN - NLM
STAT- MEDLINE
DCOM- 20011204
LR  - 20190822
IS  - 0147-5185 (Print)
IS  - 0147-5185 (Linking)
VI  - 25
IP  - 10
DP  - 2001 Oct
TI  - Progression to large B-cell lymphoma in splenic marginal zone lymphoma: a 
      description of a series of 12 cases.
PG  - 1268-76
AB  - Splenic marginal zone lymphoma (SMZL) is considered to be an indolent extranodal 
      B-cell lymphoma. Despite its low aggressivity, histologic progression has been 
      described in sporadic reports, although the frequency, characteristics, and 
      underlying molecular abnormalities of this phenomenon are largely unknown. We 
      review here the clinical, morphologic, immunohistochemical, and molecular 
      features of a series of 12 SMZL cases that showed progression to large B-cell 
      lymphoma (LBCL). The most frequent location of secondary LBCL was in peripheral 
      lymph node. This occurred between 12 and 85 months after diagnosis of SMZL. 
      However, in two cases LBCL was diagnosed at the initial stage of the disease (one 
      spleen tumoral nodule and one hilar lymph node). The histologic and 
      immunophenotypic features of these cases were similar to those of transformed 
      LBCL at other sites. In four cases the immunoglobulin heavy chain gene polymerase 
      chain study revealed the same rearrangement pattern in both primary and secondary 
      tumors, thereby confirming their identity and excluding the possibility of a 
      second malignancy. As is the case with other low-grade lymphoproliferative 
      disorders, SMZL may undergo high-grade transformation. These 12 cases represent 
      13% of our series of SMZL with adequate follow-up. The incidence of large cell 
      transformation in SMZL seems to be lower than in follicular lymphoma (25-60%) and 
      mantle cell lymphoma (11-39%), although it is similar to the frequency of 
      transformation in B-chronic lymphocytic lymphoma/small lymphocytic lymphoma 
      (1-10%). The mean proliferative index (MIB1 staining) in initial SMZL specimens 
      of cases with LBCL transformation was 28.6%, higher than that of MIB1 staining in 
      the overall SMZL series (21.8%), although not statistically significantly so. p53 
      or p16INK4a inactivation in this series was observed in only one case, in 
      contrast with the situation observed in chronic lymphocytic leukemia, follicular 
      lymphoma, and mantle cell lymphoma. It seems that progression in SMZL is mainly 
      independent of p53 or p16INK4a inactivation. The frequency of the 7q deletion in 
      this series was 3 of 7 (42%). 7q loss may play an alternative role in the 
      inactivation of the p53 and p16INK4a pathway, thereby favoring tumoral 
      progression.
FAU - Camacho, F I
AU  - Camacho FI
AD  - Molecular Pathology Program, Fundacion Centro Nacional de Investigaciones 
      Oncologicas Carlos III-CNIO, Madrid, Spain.
FAU - Mollejo, M
AU  - Mollejo M
FAU - Mateo, M S
AU  - Mateo MS
FAU - Algara, P
AU  - Algara P
FAU - Navas, C
AU  - Navas C
FAU - Hernandez, J M
AU  - Hernandez JM
FAU - Santoja, C
AU  - Santoja C
FAU - Sole, F
AU  - Sole F
FAU - Sanchez-Beato, M
AU  - Sanchez-Beato M
FAU - Piris, M A
AU  - Piris MA
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
RN  - 0 (Antigens, Nuclear)
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Cyclin-Dependent Kinase Inhibitor p16)
RN  - 0 (DNA, Neoplasm)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Proto-Oncogene Proteins)
RN  - 0 (Proto-Oncogene Proteins c-bcl-6)
RN  - 0 (Transcription Factors)
RN  - 0 (Tumor Suppressor Protein p53)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Alleles
MH  - Antigens, Nuclear
MH  - Biomarkers, Tumor/analysis
MH  - Chromosome Deletion
MH  - Chromosomes, Human, Pair 7
MH  - Cyclin-Dependent Kinase Inhibitor p16/analysis/genetics
MH  - DNA Mutational Analysis
MH  - DNA, Neoplasm/analysis
MH  - DNA-Binding Proteins/analysis/genetics
MH  - Disease Progression
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunohistochemistry
MH  - Immunophenotyping
MH  - Lymphoma, B-Cell/chemistry/genetics/mortality/*pathology
MH  - Lymphoma, Large B-Cell, Diffuse/chemistry/genetics/mortality/*pathology
MH  - Male
MH  - Middle Aged
MH  - Nuclear Proteins/analysis
MH  - Proto-Oncogene Proteins/analysis/genetics
MH  - Proto-Oncogene Proteins c-bcl-6
MH  - Splenic Neoplasms/chemistry/genetics/mortality/*pathology
MH  - Survival Rate
MH  - Transcription Factors/analysis/genetics
MH  - Tumor Suppressor Protein p53/analysis/genetics
EDAT- 2001/11/02 10:00
MHDA- 2002/01/05 10:01
CRDT- 2001/11/02 10:00
PHST- 2001/11/02 10:00 [pubmed]
PHST- 2002/01/05 10:01 [medline]
PHST- 2001/11/02 10:00 [entrez]
AID - 10.1097/00000478-200110000-00007 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 2001 Oct;25(10):1268-76. doi: 10.1097/00000478-200110000-00007.