Adult ; CD56 Antigen ; metabolism ; Carcinoma ; metabolism ; pathology ; surgery ; Carcinoma, Medullary ; pathology ; Carcinoma, Papillary ; metabolism ; pathology ; DNA-Binding Proteins ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Paraganglioma ; metabolism ; pathology ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; methods ; Transcription Factors ; Triglycerides ; metabolism

CARMA1, BCL10 and MALT1 are lymphocyte-specific signaling molecules of NF-?B pathway.The abnormalities of those molecules such as gene mutation, rearrangement, translocation or amplification often connect with the lymphoma genesis. This article reviewed the physiological function of those molecules and the relationship between genetic abnormalities and lymphoma genesis, also with present target-treatment research and new gene medicine development.

Carcinoma ; diagnosis ; Carcinoma, Papillary ; diagnosis ; Humans ; Thyroid Neoplasms ; diagnosis

Aged ; Diagnosis, Differential ; Dura Mater ; pathology ; ultrastructure ; Female ; Humans ; Lymphoma, B-Cell, Marginal Zone ; diagnosis ; pathology ; therapy ; Magnetic Resonance Imaging ; Male ; Meningioma ; pathology ; Middle Aged ; Plasma Cells ; ultrastructure ; Plasmacytoma ; pathology ; Tomography, X-Ray Computed

Background and purpose: Primary renal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue(MALT lymphoma) are rare tumors in the kidney.Till now,less than 50 cases have been reported all over the world.This article introduced 2 cases of MALT lymphoma based on the morphological and immunophenotype features,aimed to highlight this rare tumor in terms of clinical characteristics and pathology.Methods:2 cases of renal MALT lymphomas were treated in our hospital.Clinical history were collected,morphological analysis was evaluated based on the HE section,and immunohistochemistry were performed by CD20,CD79a,CD5,CD10,CD43,CD23,BCL10 and cyclinD1 antibodies.Results:2 female patients with the age of 48 and 55,respectively,both of them had a history of chronic pyelonephritis.Under the B ultrasonic and CT scanning,a lump in the kidney was found and whole nephrectomy was performed.In the macroscopic view,tumors were in the renal medulla with dark red color and ill-defined boundary.In the microscopic view,lymphoid cells infiltrate from the pelvis to the medulla of kidney,lymphoid cells mainly consisted of small lymphocytes,centrocyte-like cells,lymphoplasmacytoid and plasma cells.Reactive follicles and lymphoepithelial lesions also could be seen in the tumor.Immunohistochemistry showed that the tumor cells were CD20 and CD79a positive,CD43 was weak positive,but CD5,CD10,CD23,BCL10 and cyclinD1 were all negative.Conclusions:Primary renal MALT lymphomas are very rare diseases.According to the clinical manifestation,it is hard to differentiate from renal cell carcinoma.But the morphological features were consistent with the classic MALT lymphomas in other sites.Immunophenotype profiles were helpful for diagnosis.

Purpose　To study the clinicopathologic and immunohistochemical characteristics of mantle cell lymphomas(MCL). Methods　Six cases of MCL in lymph nodes were collected and immunohistochemical stains for CD45,CD20,CD79,CD45RO,CD30,CD68,CD43,CD5,cyclinD1,bcl-2,c-myc,IgD and IgM performed. Results　According to the histological pattern, MCL could be divided into 4 subtypes, mantle zone type(1 case), nodular type (1 case), diffuse type (2 cases) and blastoid type (2 cases). The tumor cells were pan-B+, IgD+, CD43+, cyclinD1 + (5/6),CD5+(4/6). Conclusions　MCL is a kind of special immunophenotypic B cell lymphoma. The different histiological subtype may have a different prognosis. The differential diagnosis includes the others B cell lymphomas, for instance, marginal zone B cell lymphomas (MZL),follicular lymphomas(FL), and choronic lymphocytic leukemia/small lymphocytic lymphomas (CLL/SLL).

Objective To study the expression of BCL10 and associated chromosomal aberration in primary cutaneous marginal zone B-cell lymphoma (PCMZL). Methods Tissue specimens were collected from 17 patients with PCMZL. Immunohistochemistry was used to detect the expression of BCL10. Fluorescence in situ hybridization (FISH) was performed to examine the presence of API2-MALT1 fusion gene and chromosomal aberration in BCL10, MALT1 as well as IgH genes in these cases. Results Of these patients,94.1% (16/17) expressed BCL10 protein. The cytoplasmic expression of BCL10 was observed in 64.7% (11/17) of the patients, and nuclear expression in 29.4% (5/17). As shown by FISH test, neither API2-MALT1 fusion gene nor chromosomal aberration in BCL10, MALT1 or IgH genes was present in these patients. Conclusions Compared with MALT lymphomas originating from tissues other than skin, PCMZL is uncommonly associated with chromosomal abnormalities; it is possible that there are unknown factors contributing to its tumorigenesis. Nuclear BCL10 is unrelated to the presence of chromosomal aberration in BCL10, MALT1 or IgH genes. Further follow-up is required to clarify the association between nucle ar BCL10 and poor prognosis of PCMZL.

Primary central nervous system lymphoma (PCNSL) is an uncommon non-Hodgkin's lymphoma with poor prognosis. This study aimed to depict the genetic landscape of Chinese PCNSLs. Whole-genome sequencing was performed on 68 newly diagnosed Chinese PCNSL samples, whose genomic characteristics and clinicopathologic features were also analyzed. Structural variations were identified in all patients with a mean of 349, which did not significantly influence prognosis. Copy loss occurred in all samples, while gains were detected in 77.9% of the samples. The high level of copy number variations was significantly associated with poor progression-free survival (PFS) and overall survival (OS). A total of 263 genes mutated in coding regions were identified, including 6 newly discovered genes (ROBO2, KMT2C, CXCR4, MYOM2, BCLAF1, and NRXN3) detected in ⩾ 10% of the cases. CD79B mutation was significantly associated with lower PFS, TMSB4X mutation and high expression of TMSB4X protein was associated with lower OS. A prognostic risk scoring system was also established for PCNSL, which included Karnofsky performance status and six mutated genes (BRD4, EBF1, BTG1, CCND3, STAG2, and TMSB4X). Collectively, this study comprehensively reveals the genomic landscape of newly diagnosed Chinese PCNSLs, thereby enriching the present understanding of the genetic mechanisms of PCNSL.
Humans ; DNA Copy Number Variations ; Nuclear Proteins/genetics* ; Central Nervous System Neoplasms/pathology* ; Transcription Factors/genetics* ; Prognosis ; Lymphoma/genetics* ; Genomics ; China ; Central Nervous System/pathology* ; Bromodomain Containing Proteins ; Cell Cycle Proteins/genetics*