Abstract
A primary pleural marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) is a very rare eventuality. Here, we report a rare case of MALT lymphoma arising in the pleura and update the literature on this topic. A 74-year-old female was hospitalized for persistent cough and weakness. A chest radiograph and total-body CT scan showed only large right-sided pleural effusion, and the coexistence of pleural thickening. Video-assisted thoracoscopic exploration and a talc pleurodesis were performed and microscopic and immunohistochemical findings showed that the tumor was a pleural MALT lymphoma. The patient received immunotherapy with Rituximab and obtained a good response that lasted 2 years. To the best of our knowledge, only seven cases of primary pleural MALT lymphoma have been documented until recently, mostly from Japan with a mean age for all patients of 60.5 years. The pathogenesis of MALT lymphomas remains unclear, although a possible chronic antigenic stimulation by microbial pathogens and/or autoantigens has been hypothesized. Surgical resection was performed in most cases, and some patients received postoperative chemotherapy or immunotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.
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Swerdlow SH, Campo E, Lee Harris N, Jaffe ES, Pileri SA, Stein H, Thiele J, Vadirman JW. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In: Isaacson PG, Chott A, Nakamura S, Muller-Hermelink HK, Harris NL, Swerdlow SH, editors. World Health Organization classification of tumours of haematopoietic and lymphoid tissues. 2008. p. 214–9.
Kodama K, Yokose T, Takahashi K, et al. Low-grade lymphoma of mucosa-associated lymphoid tissue in the lung: a report of a case with pleural dissemination. Lung Cancer. 1999;24:175–8.
Ahmad H, Pawade J, Falk S, et al. Primary pleural lymphomas. Thorax. 2003;58:908–9.
Hirai S, Hamanaka Y, Mitsui N, et al. Primary malignant lymphoma arising in the pleura without preceding longstanding pyothorax. Ann Thorac Cardiovasc Surg. 2004;10:297–300.
Mitchell A, Meunier C, Ouelette D, Colby T. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue with initial presentation in the pleura. Chest. 2006;129:791–4.
Gomyo H, Kajimoto K, Maeda A, Mizuno I, Funada Y, Fukui E, Hanioka K, Ogura M, Murayama T. t(14;18)(q32;q21)-bearing pleural MALT lymphoma with IgM paraproteinemia: value of detection of specific cytogenetic abnormalities in the differential diagnosis of MALT lymphoma, lymphoplasmacytic lymphoma. Hematology. 2007;12(4):315–8.
Kawahara K, Sasada S, Nagano T, Suzuki H, Kobayashi M, Matsui K, Takata K, Yoshino T, Michida T, Iwasaki T. Pleural MALT lymphoma diagnosed on thoracoscopic resection under local anesthesia using an insulation-tipped diathermic knife. Pathol Int. 2008;58(4):253–6.
King RM, Pairolero PC, Trastek VF, et al. Primary chest wall tumors: factors affecting survival. Ann Thorac Surg. 1986;41:597–601.
Iuchi K, Ichimiya A, Akashi A, et al. Non-Hodgkin's lymphoma of the pleural cavity developing from long-standing pyothorax. Cancer. 1987;60(8):1771–5.
Swerdlow SH, Campo E, Lee Harris N, Jaffe ES, Pileri SA, Stein H, Thiele J, Vadirman JW. Primary effusion lymphoma. In: Said J, Cesarman E, editors. World Health Organization classification of tumours of haematopoietic and lymphoid tissues. 2008. p. 260–1.
Bende RJ, van Maldegem F, van Noesel CJ. Chronic inflammatory disease, lymphoid tissue neogenesis and extranodal marginal zone B-cell lymphomas. Haematologica. 2009;94(8):1109–23.
Inagaki H. Mucosa-associated lymphoid tissue lymphoma: molecular pathogenesis and clinicopathological significance. Pathol Int. 2007;57(8):474–84.
Isaacson PG, Du MQ. MALT lymphoma: from morphology to molecules. Nat Rev Cancer. 2004;4(8):644–53.
Streubel B, Simonitsch-Klupp I, Müllauer L, Lamprecht A, Huber D, Siebert R, Stolte M, Trautinger F, Lukas J, Püspök A, Formanek M, Assanasen T, Müller-Hermelink HK, Cerroni L, Raderer M, Chott A. Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites. Leukemia. 2004;18(10):1722–6.
Stefanovic A, Lossos IS. Extranodal marginal zone lymphoma of the ocular adnexa. Blood. 2009;114(3):501–10.
Suarez F, Lortholary O, Hermine O, Lecuit M. Infection-associated lymphomas derived from marginal zone B cells: a model of antigen-driven lymphoproliferation. Blood. 2006;107(8):3034–44.
Swerdlow SH, Campo E, Lee Harris N, Jaffe ES, Pileri SA, Stein H, Thiele J, Vadirman JW. Introduction and overview of the classification of the lymphoid neoplasms. In: Jaffe ES, Harris NL, Stein H, Campo E, Pileri SA, Swerdlow SH, editors. World Health Organization classification of tumours of haematopoietic and lymphoid tissues. 2008. p. 166.
Swerdlow SH, Campo E, Lee Harris N, Jaffe ES, Pileri SA, Stein H, Thiele J, Vadirman JW. Splenic B-cell marginal zone lymphoma. In: Isaacson PG, Piris MA, Berger F, Swerdlow SH, Thieblemont C, Pittaluga S, Harris NL, editors. World Health Organization classification of tumours of haematopoietic and lymphoid tissues. 2008. p. 185–7.
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Motta, G., Conticello, C., Amato, G. et al. Pleuric presentation of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue: a case report and a review of the literature. Int J Hematol 92, 369–373 (2010). https://doi.org/10.1007/s12185-010-0645-2
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DOI: https://doi.org/10.1007/s12185-010-0645-2