Molecular pathology of lymphoma

Abstract

Ocular lymphomas can be divided into intraocular lymphomas and ocular adnexal lymphomas. The vitreoretinal lymphomausually a diffuse large B-cell lymphoma (DLBCL) of high-grade malignancy-is the most common lymphoid malignancy arising in the eye, while the extranodal marginal zone B-cell lymphoma (EMZL), an indolent often recurrent tumour, occurs most frequently in the ocular adnexal tissue. The two lymphoma subtypes differ significantly in their clinical presentation, subsequent course and outcome as well as in their underlying morphological, immunophenotypical and genetic features. The molecular processes involved in DLBCL and EMZL development are complex, and include chromosomal translocations, mutations caused by aberrant somatic hypermutation, sporadic somatic mutations, and copy number alterations, characterized by deletions and amplifications. These lead to alterations in particular signalling pathways, which in turn activate transcription factors, such as nuclear factor NF-kB. This review provides an overview of the histological features of DLBCL and EMZL, and discusses the current insights into the molecular mechanisms underlying the development of these tumours, when they occur systemically and particularly when they arise in ocular tissues.

Malignant lymphoma of the conjunctiva

“…20,30,91 Another 15%e20% of ocular adnexal EMZL patients carry the t(3;14)(p14;q32) translocation, whereas t(1;14)(p22;q32) occurs in approximately 10% of these. 20,30,123 Additionally, t(5;14)(q34;q32) and t(9;14)(p24;q32) translocations have been recognized as potentially taking part in the development of ocular adnexal EMZL and their roles need further investigation, which also regards the newly detected (5;11)(q33;p11.2) translocation in a case of conjunctival EMZL. 28,30 Trisomy of chromosomes 3 and 18 is another cytogenetic abnormality associated with EMZL.…”

“…20,30,123 Additionally, t(5;14)(q34;q32) and t(9;14)(p24;q32) translocations have been recognized as potentially taking part in the development of ocular adnexal EMZL and their roles need further investigation, which also regards the newly detected (5;11)(q33;p11.2) translocation in a case of conjunctival EMZL. 28,30 Trisomy of chromosomes 3 and 18 is another cytogenetic abnormality associated with EMZL. 86,109,134 Gains of chromosome 3 are rarely found in EMZL of the conjunctiva, whereas trisomy 18 is very common in conjunctival EMZL, found in as many as 67% of patients.…”

“…As evidence suggests that the frequency of particular molecular alterations varies according to the site of origin, more studies are needed to be able to determine their relevance. 30 …”

Expression Level of Valosin Containing Protein is Associated with Prognosis of Primary Orbital MALT Lymphoma

“…As we known, IκB is a specific inhibitor of the NFκB transcription factor (Baeuerle et al, 1988). It has become clear that aberrant deregulated NFκB activation is a hallmark of several lymphoid malignancies, particularly MALT lymphoma (Coupland et al, 2013). Moreover, it has been reported that VCP can inhibit apoptosis and facilitate metastasis of the Osteosarcoma Cell Line (Asai et al, 2002).…”

Transretinale Biopsie beim intraokularen Lymphom

“…Der Begriff primäres vitreoretinales Lymphom (PVRL) sollte für Lymphome verwendet werden, die den Glaskörper (GK), N. opticus und das retinale Pigmentepithel (RPE) beteiligen und häufig mit einem primären ZNS-Lymphom assoziiert sind [1]. Das PVRL ist meist ein diffuses großzelliges B-Zell-Lymphom von hoher Malignität [2] und meist bilateral. Jedoch können ebenfalls T-Zell-Lymphome vorkommen [3].…”