Incidence of marginal zone lymphoma in the United States, 2001-2009 with a focus on primary anatomic site.

The aetiology of marginal zone lymphoma (MZL) is purported to differ by anatomic site. While this is supported by clinical series of single MZL sites, no population-based study has comprehensively assessed incidence patterns across sites. To gain insight into disease aetiology, we assessed MZL incidence by site using data from 18 U.S. Surveillance, Epidemiology and End Results (SEER) Program population-based registries. We calculated age-adjusted incidence rates (IRs) by sex, race, and calendar year. During 2001-2009, 4,081 (IR = 5·7/1,000,000 person-years) and 8,821 (IR = 12·3) individuals were diagnosed with nodal MZL and extranodal MZL, respectively. The most common extranodal sites were stomach (IR = 3·8), spleen (IR = 1·6), eye/adnexa (IR = 1·4), and lung, skin, and salivary glands (IRs = 0·9-1·0). We observed distinct age-specific patterns by MZL site, with IRs increasing steeply at younger ages and less prominently after mid-life at several sites, except skin. Gender and racial/ethnic disparities were also apparent across sites. Between 2001-2005 and 2006-2009, MZL IRs decreased significantly for gastric (-15%) and soft tissue (-28%) sites, whereas IRs increased significantly for lung (18%), skin (43%), and kidney/renal pelvis (116%). In combination, our findings support the contention that MZL is characterized by aetiological heterogeneity across sites and susceptibility is probably influenced by intrinsic characteristics and environmental exposures.

Splenic marginal zone lymphoma: a population-based study on the 2001-2008 incidence and survival in the United States.

The epidemiology of splenic marginal zone lymphoma (SMZL) in the United States has not been addressed. Eight years of data (2001-2008) from 17 registries of the Surveillance, Epidemiology and End Results (SEER) program were used for this study. Of the 116,411 cases of non-Hodgkin lymphoma (NHL) in the registries, 763 (0.6%) were SMZL. The overall annual age-adjusted incidence was 0.13 per 100,000 persons per year. The annual percent change in age-adjusted incidence was 4.81% overall (p < 0.05), and significantly increasing trends were found for patients who were white, male or aged 70 years and older (p < 0.05). The relative 5-year overall survival rate for patients with SMZL was 81% (95% confidence interval 75-86%). The incidence of SMZL was highest among whites, males and older patients. A steadily increasing trend in incidence was observed for SMZL. The relative 5-year overall survival rate was high.

[Historical development and current concepts on B-cell lymphomas of the marginal extraganglionar site of lymphoid tissue associated with MALT lymphoma. A tribute to Dennis H Wright and Peter G Isaacson]. / Linfoma de céllulas B de la zona marginal extraganglionar del tejido linfoide asociado a mucosas (linfoma MALT). Evolución histórica y conceptos actuales. Un tributo a Dennis H. Wright y Peter G. Isaacson.

Significant advances in the understanding of marginal zone lymphoma since the first description in 1983 by Peter Isaacson and Dennis Wright have been noted. MALT lymphomas are a subgroup of low-grade B-cell lymphomas that arise from extranodal sites, comprising 7-8% of all B-cell lymphomas and displaying distinct clinicopathological characteristics. MALT lymphomas remain localized in the primary site for long periods of time and seldom disseminate unto other organs. These type of lymphomas infrequently arise in native MALT, but instead arise in MALT acquired in the course of chronic inflammatory disorders, such as Sjögren's syndrome and Helicobacter pylori infection. Eradication of H. pylori produces a clinical regression of the lymphoma in about 75% of cases. The histological hallmarks of MALT lymphoma include neoplastic centrocyte-like B cells, cells resembling monocytoid cells and the presence of lymphoepithelial lesions. The gastrointestinal tract, particularly the stomach, include two-thirds of cases; however MALT lymphomas also occur in other organs such as salivary glands, lung, thyroid, ocular adnexa, breast and skin. Genetic studies have identified three chromosomal translocations specifically associated with MALT lymphomas that include: t(1l:18)(q21;q21), t(1;14)(p22;q32), and t(14;18)(q32;q21). Although these translocations involve different genes, they appear to share a common oncogenic pathway involving NFkappaB.

Linfoma de céllulas B de la zona marginal extraganglionar del tejido linfoide asociado a mucosas (linfoma MALT): evolución histórica y conceptos actuales: un tributo a Dennis H. Wright y Peter G. Isaacson / Historical development and current concepts on B-cell lymphomas of the marginal extraganglionar site of lymphoid tissue associated with MALT lymphoma: a tribute to Dennis H Wright and Peter G Isaacson

Se han realizado avances importantes en el entendimiento del linfoma de la zona marginal, (linfoma MALT), desde la primera descripción en 1983 por Peter Isaacson y Dennis Wright. Los linfomas MALT son un subgrupo de neoplasias de bajo grado que representan entre el 7 y el 8 % de todos los linfomas B, que se originan en sitios extraganglionares y presentan características clínico-patológicas propias. Se mantienen localizados por largos periodos de tiempo y sólo ocasionalmente se diseminan a otros órganos. Rara vez se originan de un MALT normal y aparecen en el MALT adquirido en el curso de alteraciones inflamatorias crónicas como en el síndrome de Sjögren o la infección por Helicobacter pylori. La erradicación de H. pylori puede producir regresión clínica del linfoma en un 75 % de los casos. Histológicamente presenta células B neoplásicas centrocitoides, células monocitoides y lesiones linfoepiteliales. El aparato digestivo, particularmente el estómago, está afectado en las dos terceras partes de los casos. Sin embargo, puede presentarse en otros órganos como glándulas salivales, pulmón, tiroides, anexos oculares y piel. Estudios genéticos han identificado tres traslocaciones cromosómicas específicas que son: t(11:18)(q21;q21), t(1;14)(p22;q32), y t(14;18)(q32;q21). A pesar de que estas traslocaciones afectan diferentes genes, comparten una vía oncogénica común que afecta al NFκB.

Significant advances in the understanding of marginal zone lymphoma since the first description in 1983 by Peter Isaacson and Dennis Wright have been noted. MALT lymphomas are a subgroup of low-grade B-cell lymphomas that arise from extranodal sites, comprising 7-8% of all B-cell lymphomas and displaying distinct clinicopathological characteristics. MALT lymphomas remain localized in the primary site for long periods of time and seldom disseminate unto other organs. These type of lymphomas infrequently arise in native MALT, but instead arise in MALT acquired in the course of chronic inflammatory disorders, such as Sjõgren's syndrome and Helicobacter pylori infection. Eradication of H. pylori produces a clinical regression of the lymphoma in about 75% of cases. The histological hallmarks of MALT lymphoma include neoplastic centrocyte-like B cells, cells resembling monocytoid cells and the presence of lymphoepithelial lesions. The gastrointestinal tract, particularly the stomach, include two-thirds of cases; however MALT lymphomas also occur in other organs such as salivary glands, lung, thyroid, ocular adnexa, breast and skin. Genetic studies have identified three chromosomal translocations specifically associated with MALT lymphomas that include: t(1l:18)(q21;q21), t(1;14)(p22;q32), and t(14;18)(q32;q21). Although these translocations involve different genes, they appear to share a common oncogenic pathway involving NFκB.

A cirurgia no tratamento do linfoma gástrico primário do tipo Malt / Surgery in the treatment of primary gastric lymphoma of MALT

O autor relata um estudo retrospectivo de 21 prontuários de pacientes portadores de linfoma gástrico primário do tipo MALT submetidos a tratamento cirúrgico exclusivo ou associado à radioterapia e/ou quimioterapia, durante o período de 1982 a 1996 no Hospital do câncer - Ministério da Saúde...O tratamento adjuvante foi preconizado em 10 pacientes e consistiu em radioterapia em três pacientes no estágio IE, quimioterapia em cinco casos, quatro no estágio IE e um no IIE1. A associação de radioterapia e quimioterapia foi realizada em dois pacientes no estágio IE. O seguimento variou de 3 a 132 meses e evidenciou que dois pacientes faleceram por evolução da doença e um por complicações decorrentes da quimioterapia. Considera-se que o linfoma é uma doença pouco frequente, com diagnóstico baseado na endoscopia digestiva alta e que a cirurgia é essencial para o estagiamento e tratamento da doença.