(Chest. 2006;129:791-794.)
© 2006
American College of Chest Physicians
Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue With Initial Presentation in the Pleura*
Andrew Mitchell, MD;
Caroline Meunier, MD;
Denise Ouellette, MD and
Thomas Colby, MD
* From the Departments of Pathology (Drs. Mitchell and Meunier) and Surgery (Dr. Ouellette), Maisonneuve-Rosemont Hospital, Montreal, QC, Canada; and the Department of Pathology (Dr. Colby), Mayo Clinic Scottsdale, Scottsdale, AZ.
Correspondence to: Andrew Mitchell, MD, Department of Pathology, Maisonneuve-Rosemont Hospital, 5415 Blvd de L’Assomption, Montreal, QC, Canada H1T 2X1; e-mail: plaines{at}mac.com
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Abstract
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Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (EMZL/MALT-type) occurs in a wide variety of body sites; it is well recognized as a form of primary lung lymphoma. However, until recently, pleural presentation of this form of low-grade lymphoma has not been documented. A small series of case reports has brought to attention the potential for primary occurrence or initial presentation in the pleura of EMZL/MALT-type. In this report, we describe an additional patient with EMZL/MALT-type with initial pleural presentation and review the literature. Clinicians and pathologists dealing with lymphoproliferative disorders involving the pleura should be aware of this rare entity.
Key Words: extranodal • lymphoma • marginal • mucosa associated • mucosa-associated lymphoid tissue • pleura • pleural • zone
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Introduction
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Two forms of high-grade lymphoma involve the pleura as primary neoplasms: primary effusion lymphoma (PEL) and pyothorax-associated lymphoma (PAL).1 Both have well-characterized clinical and pathologic features. A small series of case reports234 has identified another type of lymphoma, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (EMZL/MALT-type), a low-grade neoplasm, as having similar potential for primary occurrence or initial presentation in the pleura. In this report, we describe a patient with pleural presentation of EMZL/MALT-type and review the literature.
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Case Presentation
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A 47-year-old man presented in the emergency department with fever and left-sided chest pain. A chest radiograph showed massive consolidation of the basal segments of the lower lobe of the left lung with an associated mild pleural effusion. IV antibiotics were begun, and the patient was admitted to hospital. A chest radiograph the following day showed marked progression of the pleural effusion. A thoracic drain was inserted into the left pleural cavity. Cultures of a sample of the withdrawn fluid failed to grow any infectious agents. Four days later, a CT scan of the thorax showed continued lower-lobe consolidation and a localized collection of fluid suggestive of empyema. Infiltrates were also noted in the posterior segment of the right upper lobe, interpreted as possible pneumonia (Fig 1
). Left thoracotomy with decortication of the pleural cavity was performed.
Pathologic examination of the decortication specimen showed the customary accumulations of fibrin, granulation tissue, and reactive fibrosis with associated inflammation. However, several fragments, representing a minority of the tissue removed, contained dense band-like infiltrates of small lymphocytes and plasmocytes with little to no cytologic atypia suggestive of low-grade lymphoma (Fig 2
). The immunohistochemical study of the infiltrate showed the small lymphocytes to be B lineage (CD20 positive) with the plasma cell population demonstrating IgM-
monoclonality. There was no expression of the markers CD5, CD23, CD10, BCL-6, and cyclin D1. These results were interpreted as consistent with either EMZL/MALT-type or lymphoplasmocytic lymphoma (LPL).
The patient’s medical history was essentially negative. He had felt well in the months preceding his current illness. He had stopped smoking 4 years prior to the current episode. There was no history of any lymphoproliferative disorder. Subsequent investigation revealed no laboratory evidence of anemia or lymphocytosis; the lactate dehydrogenase level was normal. HIV test results were negative. However, serum immunofixation revealed a monoclonal gammopathy, IgM- (which has persisted during 18 months of follow-up). A CT scan of the abdomen and pelvis showed no evidence of lymphadenopathy. A bone marrow biopsy of the iliac crest was morphologically normal, without evidence of a lymphomatous infiltrate; the marrow immunophenotype by flow cytometry was also normal. Polymerase chain reaction analysis of the bone marrow aspirate demonstrated absence of an Ig heavy chain rearrangement and the 14; 18 translocation.
In the absence of any evidence of lymphoma outside the pleural space, the diagnosis of primary pleural EMZL/MALT-type was favored over LPL. The patient was discharged from hospital without treatment but with a plan for regular follow-up (he is currently asymptomatic). A thoracic CT scan 8 months later showed persistence, without progression, of the pulmonary infiltrates noted at presentation. It is unknown if these infiltrates represent pulmonary EMZL/MALT-type or organized pneumonia. If the former, this may explain the persistent IgM- monoclonal gammopathy. This question remains unanswered as the patient, presently feeling perfectly well, has refused lung biopsy.
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Discussion
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The above case illustrates an example of EMZL/MALT-type with initial pleural presentation. The distinction between this form of low-grade lymphoma and LPL cannot be made purely on the morphologic and immunohistochemical characteristics of the tumor cells; clinical extent of disease is also necessary for correct classification. Both are small B-cell proliferations with a similar immunohistochemical profile. Although plasma-cell or and/or plasmacytoid differentiation is invariably present in LPL, certain cases of EMZL/MALT-type also share this feature. LPL is characterized by infiltration of the bone marrow, lymph nodes, and spleen, all of which were uninvolved in our patient, with an associated serum monoclonal protein in the majority of cases.5 In contrast, EMZL/MALT-type primarily involves and/or is first diagnosed in extranodal tissues. It is also increasingly recognized as a cause of Waldenstrom macroglobulinemia,6 a syndrome characterized by serum monoclonal gammopathy, usually of IgM type, in the setting of a neoplasm of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells.5 In consideration of the clinical context, the diagnosis of EMZL/MALT-type is therefore appropriate in this case, although one must acknowledge that the IgM monoclonality of the plasma cell component may have led other pathologists, on strictly morphologic and immunohistochemical grounds, to favor LPL.
The clinical presentation of acute pleuritis with probable bronchopneumonia and the identification of low-grade lymphoma intimately admixed with the inflammatory pleural exudate raises the question of inflammation as an etiologic factor. Although chronic pyothorax is strongly associated—albeit after a many-year latency period—with the development of high-grade pleural lymphoma in the Japanese population (see below), a parallel association between acute pyothorax and pleural lymphoma has not been described. This represents a singular feature of the current case.
Initial pleural presentation of EMZL/MALT-type appears to be a rare clinicopathologic entity. A literature search using PubMed with the key words pleura, pleural, lymphoma, primary, low grade, marginal zone, MALT, and MALToma found only four other documented cases.234 The pertinent features of those cases and of the present one are summarized in Table 1
.
All patients have been middle-aged to elderly men without prior known chronic pleural disease. One patient had an IgM serum monoclonal gammopathy2; it is the only case in which concurrent lymphoma was confirmed histologically in the adjacent lung. The authors2 note that it is also the first known description of pleural extension from primary lung EMZL/MALT-type. Without histologic confirmation, we cannot conclude if there is lung parenchymal involvement in our patient, but the persistence of the IgM monoclonal gammopathy suggests this may be so.
No consistent pattern of presentation or etiologic factor can be identified in this small series. A search for Epstein-Barr virus (EBV) was not conducted in these cases, but given its low presence in gastric EMZL/MALT-type (8 to 12% of cases),7 it is highly unlikely to be an etiologic agent. The clinical course of all of these cases would appear to be indolent, in keeping with the behavior of this form of lymphoma in general.
Other Primary Pleural Lymphomas
Two forms of primary pleural lymphoma have been previously described; both have characteristic clinical and pathologic features.15 Table 2
summarizes their major features, which are contrasted with those of pleural EMZL/MALT-type.
PEL, sometimes referred to as body cavity-based lymphoma, and PAL are both high-grade B-cell lymphomas of which numerous examples are recorded. Rare deviations from the high-grade B-cell subtype have been described in both forms.18
PEL presents as lymphomatous effusions without identifiable tumor masses. The pericardial and peritoneal cavities may be involved in addition to the pleura. Human herpes virus 8/Kaposi sarcoma herpes virus infection is always present. HIV infection is present in most cases; EBV infection is present in half of cases.1
The vast majority of cases of PAL have occurred in the Japanese population, often many decades after the development of tuberculosis-related pyothorax or treatment of pulmonary tuberculosis by artificial pneumothorax, the latter at one time a common treatment of the disease in that country.9 "Western" cases of PAL are exceedingly rare.10 EBV infection of tumor cells is detected in all cases. Both PEL and PAL are clinically aggressive. Survival in PEL is measured in months1; survival in PAL was 21.6% after 5 years in one large series.9
In summary, EMZL/MALT-type can present as a primary pleural neoplasm or present initially in the pleura via extension from an adjacent pulmonary primary site. Although lacking, as yet, recognized characteristic oncogenic influences as seen in PEL and PAL, pleural presentation of EMZL/MALT-type may be considered, as witnessed by the small but growing number of case reports, a clinicopathologic entity with which clinicians and pathologists dealing with pleural disease should be aware.
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Footnotes
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This work was performed at Maisonneuve-Rosemont Hospital, Montreal, Quebec, and Mayo Clinic Scottsdale, Scottsdale, AZ.
Abbreviations: EBV = Epstein-Barr virus; EMZL/MALT-type = extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue; LPL = lymphoplasmocytic lymphoma; PAL = pyothorax-associated lymphoma; PEL = primary effusion lymphoma
Received for publication May 5, 2005.
Accepted for publication June 30, 2005.
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References
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- Alexandrakis, MG, Passam, FH, Despina, SK, et al (2004) Pleural effusions in hematologic malignancies. Chest 125,1546-1555[Abstract/Free Full Text]
- 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;24,175-178[CrossRef][ISI][Medline]
- Ahmad, H, Pawade, J, Falk, S, et al Primary pleural lymphomas. Thorax 2003;58,908-909[Abstract/Free Full Text]
- 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[Medline]
- Isaacson, PG, Muller-Hermelink, HK, Piris, MA, et al Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). Jaffe, ES Harris, NL Stein, Het al eds. WHO classification tumors of haematopoietic and lymphoid tissues. 2001,157-160 IARC Press. Lyon, France:
- Valdez, RV, Finn, WG, Ross, CW, et al Waldenstrom macroglobulinemia caused by extranodal marginal zone B-cell lymphoma. Am J Clin Pathol 2001;116,683-690[Medline]
- Ben, Rejeb A, Kchir, NK, Laabidi, B, et al Detection of Epstein-Barr virus in malt type gastric malignant lymphoma using in situ hybridization. Clin Exp Pathol 1999;47,101-105[Medline]
- Ibuka, T, Fukayama, M, Hayashi, Y, et al Pyothorax-associated pleural lymphoma: a case evolving from T-cell-rich lymphoid infiltration to overt B-cell lymphoma in association with Epstein-Barr virus. Cancer 1994;73,738-744[CrossRef][ISI][Medline]
- Nakatsuka, S, Yao, M, Hoshida, Y, et al Pyothorax-associated lymphoma: a review of 106 cases. J Clin Oncol 2002;20,4255-4260[Abstract/Free Full Text]
- Androulaki, A, Drakos, E, Hatzianastassiou, S, et al Pyothorax- associated lymphoma (PAL): a western case with marked angiocentricity and review of the literature. Histopathology 2004;44,69-76[CrossRef][ISI][Medline]
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Abstract
Introduction
