Abstract
Primary gastric mantle cell lymphoma is a rare form of gastointestinal tumour. Although peritoneal carcinomatosis accompanied by malignant ascites is relatively common, mantle cell lymphoma presenting with ascites is rare. Also, effusions involving pericardial and pleural cavities are uncommon during the course of lymphomas. We report the first case in which pericardial, pleural and peritoneal effusion of a primary gastric mantle cell lymphoma.
Introduction
Mantle cell lymphoma (MCL), a mature B-cell non-Hodgkin lymphoma (NHL), often shows aggressive behaviour. It is also characterised by the chromosomal translocation t(11;14)(q13;q32). Specific result of this translocation is an overexpression of the cyclin D1. Immunohistochemical CD5+, CD19+, CD20+, CD10−, CD23−, FMC-7+, bcl-2+ and cyclin D1+ are specific of MCL. 1 Common sites of involvement are lymph nodes, spleen, Waldeyer’s ring, bone marrow, blood and extranodal sites including the gastrointestinal tract.2,3 Primary gastric MCL represents 2.5%–7% of NHLs. 4 Involvement of the serosa may be the presenting feature in a wide and complex variety of lymphomas. 5 Although the frequency of pleural effusion is 20%–30% in lymphomas, the involvement of peritoneal and pericardial cavities is uncommon. 6 Treatment of MCL still remains a very controversial issue with very poor response rates.7,8 In this report, we describe the case of a primary gastric MCL with pericardial, pleural and peritoneal involvement.
Case
A 55-year-old female presented to our clinic with abdominal fullness, dyspnea and dyspepsia lasting for three months. There was no disease in her personal and family history. Also there was no history of smoking, illicit drug use or alcohol consumption. Physical examination revealed decreased breath sounds. Her abdomen was protuberant with positive fluid thrill. Laboratory analyses showed anaemia (haemoglobin 10.5 g/dl), with elevated ESR (42 mm/h). White blood cell count (WBC) and platelet count were normal (8.2 × 109/l and 235 × 109/l, respectively). Biochemical tests showed elevated lactate dehydrogenase (LDH) (688 u/l) and decreased albumin (2.8 g/dl). Other blood chemistry was normal. On thorax CT, pericardial and bilateral pleural effusion were detected (Figure 1(a)). Also, abdominal CT scan showed peritoneal mass, diffuse peritoneal thickening and ascites (Figure 1(b)). Analysis of the ascitic fluid showed an exudate with a WBC of 111.8 × 109/l and red blood cell count (RBC) of 105 × 109/l. Flow cytometry (FC) analysis was performed by using FACSCalibur flow cytometer (Becton-Dickinson, Erembodegem, Belgium). In the FC, CD5, CD19, CD20, CD22 and FMC7 found to be positive, while CD23 was negative (Figure 2). Also, analysis of the pleural fluid revealed an exudate with a WBC of 56.7 × 109/l and an RBC of 87 × 109/l and the following results on biochemical analysis: pleural total protein 3.0 g/dl (serum 5.4 g/dl), glucose 45 mg/dl (serum 80 mg/dl), LDH 980 u/l (serum 1350 µ/l) and albumin 1.9 g/dl (serum 2.8 g/dl). FC analysis of the pleural fluid showed the same results as described for the ascitic fluid. Bacterial and mycobacterial culture tests of fluids were reported as negative. Cytological studies revealed the presence of neoplastic lymphoid cells both in ascitic and pleural fluid samples. Gastroscopy identified a large ulcerated lesion in the gastric corpus. Biopsy of lesion was reported as gastric MCL (Figure 3). Fluorescent in situ hybridisation analysis showed the translocation t(11;14)(q13;q32), which is characteristic for MCL. No involvement was detected in bone marrow biopsy. The patient was diagnosed as stage IIIE MCL with high MIPI score.
9
Following chemotherapy (three-cycle R-CHOP), however, the patient died due to aggressive progression of MCL.
Transverse CT image of chest (a) showing pericardial (double arrow) and pleural involvement (arrow). Note bilateral pleural effusion. Transverse CT image of abdomen (b) demonstrating ascites, peritoneal mass (*) and diffuse peritoneal thickening (arrows) compatible with peritoneal involvement. On flow cytometric analysis of the ascitic fluid, lymphoma cells showed CD5, CD19, CD20, CD22 and FMC7 expression, while CD23 was negative. Histological picture of gastric mantle cell lymphoma. (a) light microscopy showing atypical lymphoid infiltration of the gastric mucosa (H&E, 100×); (b) atypical lymphoid cells showing immunoreactivity with CD20 (100×); (c) atypical lymphoid cells showing immunoreactivity with cyclinD1 (100×).
Discussion
Primary gastrointestinal MCLs are very uncommon, and they rarely involve stomach. Only few authors described the appearance of a primary gastric MCL.10,11 However, MCL is more likely to appear along with other gastrointestinal diseases (Chron’s disease, adenocarcinoma).4,12 We present a case where MCL was not associated with any other gastrointestinal diseases. The most important clinical problems in our case were ascites and pleural effusion. Also, pericardial involvement was detected by using thorax CT. Development of serosal effusion in the course of lymphomas, either primary or otherwise, is considered as one of the adverse factors affecting overall survival. Experience with pleural, pericardial and peritoneal effusion resulting from direct infiltration with MCL is limited. To our knowledge, such triple serosal involvement as the initial clinical manifestation of MCL as in our patient is uncommon. Recently, FC has increasingly become important in the immunophenotyping of serous fluids. It is a potentially useful tool for clinical practice with quick diagnosis. Additionally, the demonstration of monoclonal B cells or a lymphoid population with aberrant expression of surface markers may be the only way to confirm the malignant nature of an effusion. 13 MCL most commonly has the phenotype CD5+, CD23−, FMC7+. In our case CD5, CD19, CD20, CD22 and FMC7 found to be positive, while CD23 was negative, and multiple serosal involvement of gastric MCL was confirmed. Effective treatment of gastric MCL still remains a very controversial issue with poor response rates. 14 Currently, the role of maintenance/consolidation approaches is being tested as relapses continue to appear. Also, management of relapsed/refractory MCL requires an individualised treatment approach, incorporating factors such as functional status, response to prior therapies and disease biology. Bendamustine and/or high-dose cytarabine plus rituximab based chemotherapy represent the most common salvage therapy with an overall response rate of 70–80%.15,16 The presence of multiple serosal involvement in our case, who had high MIPI score at initial presentation, also may have contributed to the poor outcome. To the best of our knowledge, this is the first reported case of gastric MCL presenting with pleural, pericardial and peritoneal involvement.
Footnotes
Declaration of conflicting interests
None declared.
Funding
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.