Primary Marginal Zone B-cell Lymphoma Research Articles

Intracranial hematolymphoid malignancies: A case series with molecular characterization

ObjectiveCentral nervous system (CNS) manifestations of hematologic malignancies are uncommon and often have a poor prognosis. As hematologic neoplasms are typically chemotherapy- and radiotherapy-sensitive, surgical resection is usually not indicated; thus, opportunities for in-depth characterization of CNS hematologic tumors are limited. Here, we report four cases of rare intracranial hematologic tumors requiring surgical intervention, allowing for histopathologic and genomic characterization. MethodsThe clinical course, genetic perturbations, and histopathological features are described for a case of 1) primary marginal zone B-cell lymphoma of the dura as well as cases of brain metastases of 2) cutaneous T-cell lymphoma, 3) acute myeloid leukemia/myeloid sarcoma, and 4) multiple myeloma. Targeted DNA sequencing, fluorescence in situ hybridization, cytogenetic analysis, flow cytometry and immunohistochemical staining were used to assess the lesions. ResultMolecular and histopathological characterizations of four unusual presentations of hematolymphoid diseases involving the CNS are presented. Genetic abnormalities were identified in each lesion, including chromosomal aberrations and single nucleotide variants resulting in missense or nonsense mutations in oncogenes. ConclusionsOur case series provides insight into unique pathological phenotypes of hematologic neoplasms with atypical CNS involvement. We offer targets for future studies by identifying potentially pathogenic genetic variants in these lesions, as the full implications of the novel molecular abnormalities described remain unclear.

Intradural primary marginal zone lymphoma of the cervical spine 2years following a posterior cervical instrumented fusion.

Primary low grade central nervous system lymphoma is a rare entity, with the majority being intracranial marginal zone B-cell lymphoma. Primary spinal lymphoma is unusual, and commonly presents as an epidural lesion with diffuse large B-cell histology, as described in prior literature. We present a case of a 56year-old woman diagnosed with a primary intradural spinal lymphoma two years after a posterior cervical fusion at the same level. She was treated successfully with surgical resection and postoperative radiotherapy. To our knowledge, this is the first case in the literature describing an intradural primary marginal zone B-cell lymphoma of the spine.

Primary marginal zone B-cell lymphoma of the cavernous sinus: a case report and review of the literature

BackgroundPrimary lymphoma of the cavernous sinus is a rare form of extranodal non-Hodgkin lymphoma, of which very few cases have been reported in the published literature. This report presents the MRI findings with apparent diffusion coefficient (ADC) value in an exceedingly rare primary marginal zone B-cell lymphoma (MZBCL) of the cavernous sinus.Case presentationThe case in this study is a 59-year-old immunocompetent male patient with a 2-month history of right ptosis and blurred vision. Right third cranial nerve palsy and binocular diplopia were observed upon neurological examination. Preoperative brain CT showed an extra-axial enhancing mass lesion in the right cavernous sinus. On MRI, ipsilateral internal carotid arterial encasement was noted without causing stenosis of the vessel. Isointense signal on T1-weighted and T2-weighted images, homogeneous contrast enhancement, and diffusion restriction were also observed. The mean ADC value of the tumor is 0.64 × 10–3 mm2/s (b value = 1000 s/mm2). Subtotal resection of the tumor was performed, and improvement of clinical symptoms were observed. The pathologic diagnosis of MZBCL was established by immunohistochemical examinations.ConclusionsPrimary MZBCL of the cavernous sinus is exceedingly rare, and preoperative confirmation poses a major challenge with CT and conventional MRI only. In this case, preoperative quantitative ADC value is shown to offer valuable additional information in the diagnostic process.

Primary Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue of the Lacrimal Sac Mimicking Dacryocystitis

Primary Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue of the Lacrimal Sac Mimicking Dacryocystitis

Slow-growing primary marginal zone B-cell lymphoma arising in the chest wall in a patient without a history of tuberculosis

A 57-year-old man with a 15-year history of a right chest wall mass lesion without follow-up for 5 years was admitted to our hospital without any symptoms or evidence of malignancy. On MRI, an additional small subcutaneous mass lesion was found. Histology of both lesions revealed marginal zone B-cell lymphomas. Primary chest wall marginal zone B-cell lymphomas are rare; this report discusses the case and the literature on chest wall lymphomas.

Primary Marginal Zone B-Cell Lymphoma of the Larynx

Extranodal non-Hodgkin lymphomas limited to the larynx are rare, accounting for less than 1% of all laryngeal neoplasms. The most common site of development of primary laryngeal lymphomas is the supraglottic region. In most cases, the presenting symptoms are hoarseness, dysphagia, dyspnea, and cervical lymphadenopathy. In these cases, larynx lymphoma was the mucosa-associated lymphoid tissue type and located in the supraglottic area.

Lymphome cutané de la zone marginale : étude histologique et immunophénotypique de 49 cas

No histological or clinical criteria allow distinction between primary cutaneous marginal zone B-cell lymphoma (MZL) and secondary cutaneous forms of systemic marginal zone B-cell lymphoma. Consequently, staging alone can indicate the origin of lymphoma. Lymphoma is considered as primary cutaneous only if no other extracutaneous sites are found. We studied the histological appearance of 49 cutaneous lymphomas in order to find distinctive criteria indicative of an extracutaneous origin. This was a retrospective descriptive study of histological appearance for 49 patients with cutaneous marginal lymphoma: 29 cases of the primary form and 20 cases with extracutaneous involvement. Comparison of histological criteria did not reveal any differences between the primary cutaneous form and others forms. No prognostic criteria for relapse were found. A cutaneous tropism of primary MZL suggested the hypothesis of specific cutaneous receptors on B-cells that could have led to different histological appearances depending on the origin of the lymphoma. However, our study did not confirm this hypothesis.

Primary marginal zone B-cell lymphoma of appendix

Primary lymphomas of appendix are extremely rare tumors. The first case of primary lymphoma of appendix was reported by Warren in the year 1898. Incidence of primary lymphoma of appendix is 0.015% of all gastrointestinal lymphomas. This is a report of primary marginal zone B-cell lymphoma of appendix which presented as appendicular mass. As some cases are incidentally discovered, this case emphasizes that histological examination of all appendicectomy specimens is mandatory.

Primary marginal zone B-cell lymphoma of gastrointestinal tract presenting as multiple lymphomatoid polyposis

Dear Editor: Multiple lymphomatous polyposis (MLP) is a distinctive pattern of gastrointestinal lymphoproliferative lesion characterized by multiple nodular or polypoid tumors infiltrating segments of the intestine. MLP is usually seen in mantle cell lymphoma (MCL). However, other lymphoproliferative diseases, including marginal zone B-cell lymphoma (MZL), can also be presented as MLP. Although MZL is rare in MLP, its differentiation from the more common MCL is clinically significant because of their remarkably different clinical course and prognosis. In this article we present a case of gastrointestinal MZL initially presented as MPL of rectum and then also involving the stomach and duodenum. A 43-year-old woman visited our institution complaining of a 1-month history of lower abdominal pain, constipation, tenesmus, and mild weight loss. Initial sigmoidoscopic examination revealed numerous smallto medium-sized, grape-like polypoid lesions covering the whole rectal mucosa. On barium enema, in addition to the polypoid lesions, there was an asymmetrical luminal narrowing and indentation in upper rectum with widened retrorectal space without mucosal destruction or irregularity. The abdominal computed tomography (CT) scan revealed a 4-cm-sized, oval, and hypodense mass-like lesion within the posterior rectal wall with a thin, hyperdense wall and septated internal structure. The entire rectal wall was also diffusely thickened. The gastroscopic examination also revealed a 2-cmsized, shallow mucosal depression with surrounding mucosal thickening in the midbody, multiple small erosions and healed erosion in the antrum of the stomach, and multiple small polypoid mucosal elevations in the duodenal bulb. Microscopically, the rectal polyps showed a nodular infiltration of lymphoid cells with very distinct patterns of marginal zone involvement around the reactive germinal centers and mantle zones. The majority of infiltrating tumor cells was monocytoid, centrocyte-like cell with clear cytoplasm. There were mixed populations of small lymphocytes and occasional large blasts also. Rare lymphoepithelial lesions (LELs) were observed. Immunohistochemically, the tumor cells were of B-cell origin and positive for CD20, CD79a, and bcl-2 antibodies. CD3and CD5-positive small lymphocytes were seen mainly around the neoplastic B cells. Some B cells were also positive for CD43 antibody. However, the tumor cells were negative for CD23 and cyclin D1. The histological findings of the gastric and duodenal lesions were similar to B. K. Shin . H. Lee . J. Choi . A. Kim . H. Kim . I. Kim (*) Department of Pathology, Korea University Guro Hospital, 80 Guro-dong Guro-gu, Seoul, 152-703 South Korea e-mail: iskim@korea.ac.kr Tel.: +82-2-8186233 Fax: +82-2-8186239

Primary Lymphoma of the Breast: A Case of Marginal Zone B-Cell Lymphoma

A case of primary marginal zone B-cell lymphoma in an elderly female patient is presented. Primary breast lymphomas are rare, comprising less than 1 per cent of all breast malignancies. These tumors have no clinical, pathologic, or radiologic pathognomonic features to distinguish them from breast adenocarcinoma. The diagnosis is usually made with an excisional biopsy, and more extensive surgery should be avoided. Delivery of radiation therapy and chemotherapy is tailored according to the histologic grade, stage of disease, and overall patient condition. This report summarizes the current knowledge reflected in the literature.

Clinicopathologic analysis of ocular adnexal lymphomas: extranodal marginal zone b-cell lymphoma constitutes the vast majority of ocular lymphomas among Koreans and affects younger patients.

The majority of ocular adnexal lymphomas (OAL) are primary marginal zone B-cell lymphomas (MALT lymphomas). The present study correlated the clinicopathological variables with the histologic subtypes by World Health Organization (WHO) classification with emphasis on MALT lymphomas in OALs of Koreans. There were 68 cases (31 males and 37 females), with a mean age of 45.9 years (range 7-89 years). Histologically, 61 MALT-type, 2 diffuse large B-cell (DLBCL), 2 mantle cell type (MCL), 1 anaplastic large-cell (ALCL), and 2 NK/T-cell lymphomas (NK/T-L) were counted among them. Fifty-seven were primary cases (P-OAL), and 11 were secondary cases (S-OAL). Nearly all P-OALs were MALT lymphomas (n = 56, 98%), with an exception of 1 MCL. Eleven S-OALs included 5 MALT type, 2 DLBCL, 1 ALCL, 1 MCL, and 2 NK/T-L. All MALT lymphoma patients were alive (n = 59) except for 2 after a mean duration of follow-up of 27.6 months (range: 0-108 months): one died of an unrelated cause and one died of recurrence. One non-MALT type P-OAL was alive with no evidence of disease (42 months). Of the 11 S-OAL, 4 had marrow involvement and 5 had progression or relapse outside the orbit. Compared with the other subtypes, MALT lymphoma was more likely to present with local disease (P = 0.001), achieve complete remission (CR) (0.022), and be alive at last follow-up (0.197), and less likely to experience recurrence (P = 0.06). In conclusion, OALs in Koreans are characterized by a preponderance of primary lymphomas over systemic lymphomas, striking predominance of MALT type lymphomas, and young age of occurrence. Histologic subtype by WHO classification has a significant correlation with the final outcome, with the most favorable outcome associated with OALs of the MALT type.

Primary marginal zone B-cell lymphoma of the lymph node resembling plasmacytoma arising from a plasma cell variant of Castleman's disease. A clinicopathological and immunohistochemical study of seven patients.

Nodal marginal zone B-cell lymphomas (NMZBL) occasionally represent prominent plasma cell differentiation. Recently, we presented a patient with NMZBL who exhibited histological features that resembled plasmacytoma arising from a localized plasma cell variant of Castleman's disease. To further clarify the clinicopathological, immunohistochemical, and genotypical findings, we studied seven such patients. Clinically, these patients were characterized by localized disease and an indolent clinical course with a slowly growing bulky mass in the affected lymph node. Only one patient exhibited paraproteinemia. Histologically, the lesions were characterized by numerous evenly distributed germinal centers in extensive sheets of plasma cells. Various numbers of centrocyte-like (CCL) cells arranged in a marginal zone distribution pattern occupied the peripheral region of the lymph node. The majority of the lymphoid follicles had atrophic or regressive germinal centers. A few lymphoid follicles were colonized by CCL cells. Immunohistochemistry showed that all of the lesions contained a monoclonal plasma cell population. In three tumors, a number of the CCL cells had a similar light chain restriction pattern to that observed in plasma cells. Two of the four patients evaluated exhibited clonal bands for the IgH gene by polymerase chain reaction assay. Moreover, the presence of surface IgM+, IgD- and CD27+ CCL- cells suggests that these tumors are derived from memory B-lymphocytes.

Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin

Primary cutaneous B-cell lymphomas have been associated with Borrelia burgdorferi, the spirochete responsible for Lyme disease. Recently, cutaneous marginal zone B-cell lymphoma has been proposed as a distinct clinical-pathological entity. We report a case of primary cutaneous marginal zone lymphoma, associated with B burgdorferi infection. Polymerase chain reaction (PCR) amplification of the third complementarity determining region (CDR3) of the immunoglobulin heavy chain gene showed the presence of a monoclonal lymphoproliferation, therefore strengthening the histological diagnosis of a malignant process. B burgdorfer-specific hbb gene sequences were detected by PCR in the lymphoma tissue at diagnosis but not after antibiotic treatment. A nearly complete clinical and histological regression was observed after B burgdorferi eradication, with immunohistochemistry studies showing disappearance of plasma cell differentiation and a marked decline in the number of CD3+ T cells and Ki-67+ cells. Our case confirms the link between B burgdorferi and some cutaneous lymphomas. The disappearance of the microorganism accompanied by the unequivocal decrease of most indicators of active T- and B-cell immune response strongly supported a pathogenetic role for B burgdorferi in sustaining an antigen-driven development and growth of this cutaneous marginal zone lymphoma. Antibiotic therapy (analogous to Helicobacter pylori infection in gastric MALT lymphoma) might be helpful with the aim of averting or at least deferring the indication for more aggressive treatment.

Primary cutaneous marginal zone B-cell lymphoma: A report of 9 cases

Background: Primary cutaneous B-cell lymphoma is a heterogeneous group among which marginal zone B-cell lymphoma (MZL) appears to be the most common subtype. Objective: We analyze clinical presentation, histologic aspects, and outcome of patients with primary cutaneous MZL. Methods: All samples classified as primary cutaneous lymphoma over the past 10 years were reviewed, and cases of primary MZL were identified. Results: Nine cases of MZL were analyzed, all from the upper body region, with a predominance in elderly women. Histologic aspects included a dense, nodular, deep-seated infiltrate containing various proportions of small cells displaying a centrocyte-like, plasmacytoid or monocytoid appearance. Surface expression of CD5, CD10, and CD23 was negative. Long survival was noted but relapses in the skin, nodes, orbit, salivary glands, and breast were observed. Conclusion: MZL is the predominant primary cutaneous lymphoma of our study. It has distinctive histologic and clinical features as well as outcome. (J Am Acad Dermatol 1999;41:181-8.)