PMID- 26379152
OWN - NLM
STAT- MEDLINE
DCOM- 20160225
LR  - 20180425
IS  - 1532-0979 (Electronic)
IS  - 0147-5185 (Linking)
VI  - 39
IP  - 12
DP  - 2015 Dec
TI  - Primary CNS T-cell Lymphomas: A Clinical, Morphologic, Immunophenotypic, and
      Molecular Analysis.
PG  - 1719-1729
LID - 10.1097/PAS.0000000000000503 [doi]
AB  - Primary central nervous system (CNS) lymphomas are relatively rare with the most 
      common subtype being diffuse large B-cell lymphoma. Primary CNS T-cell lymphomas 
      (PCNSTL) account for <5% of CNS lymphomas. We report the clinical, morphologic,
      immunophenotypic, and molecular characteristics of 18 PCNSTLs. Fifteen cases were
      classified as peripheral T-cell lymphoma, not otherwise specified, 2 of which
      were of gammadelta T-cell derivation and 1 was TCR silent; there was 1 anaplastic
      large cell lymphoma, ALK-positive and 2 anaplastic large cell lymphoma,
      ALK-negative. Median age was 58.5 years (range, 21 to 81 y), with an M:F ratio of
      11:7. Imaging results showed that 15 patients had supratentorial lesions.
      Regardless of subtype, necrosis and perivascular cuffing of tumor cells were
      frequently observed (11/18 cases). CD3 was positive in all cases but 1; 10/17
      were CD8-positive, and 5/17 were CD4-positive. Most cases studied had a cytotoxic
      phenotype with expression of TIA1 (13/15) and granzyme-B (9/13). Polymerase chain
      reaction analysis of T-cell receptor gamma rearrangement confirmed a T-cell clone
      in 14 cases with adequate DNA quality. Next-generation sequencing showed somatic 
      mutations in 36% of cases studied; 2 had >1 mutation, and none showed overlapping
      mutations. These included mutations in DNMT3A, KRAS, JAK3, STAT3, STAT5B, GNB1,
      and TET2 genes, genes implicated previously in other T-cell neoplasms. The
      outcome was heterogenous; 2 patients are alive without disease, 4 are alive with 
      disease, and 6 died of disease. In conclusion, PCNSTLs are histologically and
      genomically heterogenous with frequent phenotypic aberrancy and a cytotoxic
      phenotype in most cases.
FAU - Menon, Madhu P
AU  - Menon MP
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Nicolae, Alina
AU  - Nicolae A
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Meeker, Hillary
AU  - Meeker H
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Raffeld, Mark
AU  - Raffeld M
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Xi, Liqiang
AU  - Xi L
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Jegalian, Armin G
AU  - Jegalian AG
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Miller, Douglas C
AU  - Miller DC
AD  - Department of Pathology and Anatomical Sciences, University of Missouri School of
      Medicine.
FAU - Pittaluga, Stefania
AU  - Pittaluga S
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
FAU - Jaffe, Elaine S
AU  - Jaffe ES
AD  - Laboratory of Pathology, Center for Cancer Research, National Cancer Institute,
      National Institute of Health.
LA  - eng
GR  - ZIA SC000550-33/NULL/Intramural NIH HHS/United States
PT  - Journal Article
PL  - United States
TA  - Am J Surg Pathol
JT  - The American journal of surgical pathology
JID - 7707904
RN  - 0 (Biomarkers, Tumor)
RN  - 0 (Receptors, Antigen, T-Cell, gamma-delta)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - *Biomarkers, Tumor/analysis/genetics
MH  - Biopsy
MH  - Central Nervous System
      Neoplasms/chemistry/*diagnosis/genetics/immunology/mortality/pathology
MH  - DNA Mutational Analysis
MH  - Female
MH  - Gene Rearrangement, delta-Chain T-Cell Antigen Receptor
MH  - Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
MH  - Genetic Predisposition to Disease
MH  - Humans
MH  - Immunohistochemistry
MH  - *Immunophenotyping
MH  - In Situ Hybridization
MH  - Lymphoma, T-Cell/chemistry/*diagnosis/genetics/immunology/mortality/pathology
MH  - Male
MH  - Middle Aged
MH  - *Molecular Diagnostic Techniques
MH  - Mutation
MH  - Phenotype
MH  - Polymerase Chain Reaction
MH  - Predictive Value of Tests
MH  - Prognosis
MH  - Receptors, Antigen, T-Cell, gamma-delta/genetics
MH  - *T-Lymphocytes/chemistry/immunology/pathology
MH  - Time Factors
MH  - Young Adult
PMC - PMC4644095
MID - NIHMS696126
EDAT- 2015/09/18 06:00
MHDA- 2016/02/26 06:00
CRDT- 2015/09/18 06:00
PHST- 2015/09/18 06:00 [entrez]
PHST- 2015/09/18 06:00 [pubmed]
PHST- 2016/02/26 06:00 [medline]
AID - 10.1097/PAS.0000000000000503 [doi]
PST - ppublish
SO  - Am J Surg Pathol. 2015 Dec;39(12):1719-1729. doi: 10.1097/PAS.0000000000000503.