Abstract
Primary cardiac lymphoma (PCL) comprises rare cardiac tumors and exhibits rapid growth and poor prognosis. We report the case of a 65-year-old man with PCL associated with unstable hemodynamics caused by tricuspid valve obstruction. Generally, chemotherapy is the first choice of treatment for patients with PCL. This patient required emergency tumor reduction as he was at risk of having acute hemodynamic failure caused by tricuspid valve obstruction. Therefore, he underwent a 2-staged treatment: urgent surgery to avoid sudden death by tricuspid valve obstruction as well as pulmonary embolism during chemotherapy, followed by early chemotherapy. Pathological findings showed diffuse large B-cell lymphoma, and rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine, prednisolone (R-CHOP) therapy was initiated to treat any residual tumor infiltrating the myocardial wall. The patient showed a marked clinical improvement. We conclude that surgical tumor reduction and early chemotherapy might be an effective treatment for PCL patients with hemodynamic compromise.
<Learning objective: We experienced a case of cardiac lymphoma associated with acute hemodynamic failure caused by tricuspid valve obstruction. The impacted tumor was urgently resected to avoid cardiogenic shock and sudden death, although chemotherapy, not surgery, is generally the first choice of treatment for cardiac lymphoma. The residual tumor showed a good response to the early postoperative chemotherapy. This case report suggests the utility of 2-stage treatment for cardiac lymphoma with hemodynamic failure.>
Keywords: Cardiac tumors, Malignant lymphoma, Primary cardiac lymphoma, Tricuspid valve, Chemotherapy
Introduction
Primary cardiac lymphoma (PCL) comprises only 1.0–1.6% of primary cardiac tumors but it presents with rapid growth and poor prognosis [1], [2]. Here, we report a case of PCL that necessitated surgical intervention when it resulted in a mechanical obstruction of the tricuspid valve causing progressive acute right-sided heart failure. The patient underwent a 2-stage treatment: tumor resection to improve hemodynamics and early chemotherapy. The treatment strategy is discussed herein.
Case report
A 65-year-old male patient was referred to our hospital because of rapidly progressive dyspnea. He was hypotensive (76/58 mmHg) and tachycardic (107 beats/min). Although the heart sound was normal, a dilated jugular vein and hepatomegaly were noted. Transthoracic echocardiography revealed pericardial effusion and collapsed right ventricle (Fig. 1A). Computed tomography revealed a large mass in the right atrium (RA) and right ventricle (RV) (Fig. 1B). The patient was at risk of experiencing severe hemodynamic failure due to tricuspid valve obstruction although his hemodynamics improved slightly after emergency pericardiocentesis. Although the right coronary artery was involved, coronary angiography showed that it was patent without significant stenosis. Fluorodeoxyglucose-position emission tomography showed marked uptake with a maximum standardized uptake value of 19.3 in the RA, RV, and lymph node (Fig. 1C). Cytological examination of the pericardial effusion showed small cells with a high nuclear-to-cytoplasmic ratio, indicating the presence of atypical lymphoid cells, suggesting PCL, although other cardiac tumors such as sarcoma could not be excluded.
Fig. 1.
Preoperative images. (A) Preoperative transthoracic echocardiogram, pericardial effusion (arrow) and collapsed right ventricle are shown. (B) Preoperative computed tomograms showing a large heterogeneous mass (arrow head) involving the right atrium (RA). (C) High uptake of fluorodeoxyglucose (FDG) in the RA, RV, and mediastinal lymph node on FDG-position emission tomography. LA, left atrium; LV, left ventricle; RV, right ventricle.
Thus, an urgent surgery was planned: (1) to prevent right-sided heart failure induced by the tumor that occupied the RA and tricuspid valve, and (2) to prevent tricuspid valve obstruction or pulmonary embolism during chemotherapy.
He underwent the surgery on 5 days after admission. Intraoperative transesophageal echocardiography showed that the tricuspid valve was nearly completely obstructed by a tumor with a peak velocity of 1.8 m/s (Fig. 2A and B and Supplemental video 1). A cardiopulmonary bypass was established, a whitish mass occupying the RA and tricuspid valve was observed (Fig. 3A and B and Supplemental video 2). The tricuspid valve leaflet was displaced but not invaded.
Fig. 2.
Intraoperative transesophageal echocardiograms. (A) Preoperatively, the tumor occupied the right atrium (RA: circle) and the displaced tricuspid valve (arrow). The right ventricle (RV) wall was thick (arrow head). (B) Marked tricuspid stenosis was noted. (C) Postoperatively, tricuspid valve function was restored but with thickened RV wall. LV, left ventricle.
Fig. 3.
Surgical findings and pathological examination. (A) The tumor occupying the whole cavity of the right atrium (RA). (B) The white fragile tumor of the RA. (C) The histopathological examination showed diffuse growth of atypical cells with large, irregular, or round nuclei and a small cytoplasmic area (hematoxylin and eosin staining 20×). (D) The immunohistochemical staining. Atypical cells are positive for bcl-2 (20×). RV, right ventricle.
Supplementary Videos 1 and 2 related to this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jccase.2016.02.005.
Because a large portion of the RA and RV wall was invaded by the tumor and complete resection of the tumor could lead to cardiac rupture, only the portion that obstructed the RV inflow tract was resected. Histopathological examination showed diffuse growth of atypical cells with large, irregular, or round nuclei and small cytoplasmic areas (Fig. 3C). Immunohistochemistry showed that the atypical cells expressed Bcl-2 (Fig. 3D), CD20, and MUM1, suggesting a diagnosis of diffuse large B-cell lymphoma (DLBCL), a subtype of PCL.
As the postoperative course was uneventful, we decided to administer early chemotherapy consisting of 360 mg/m2 rituximab, 710 mg/m2 cyclophosphamide, 47 mg/m2 doxorubicin, 1.1 mg/m2 vincristine, and 47 mg/m2 prednisolone (R-CHOP) at 12 days after surgery. Following chemotherapy, the size of the tumor invading the RV wall was markedly reduced and the RV wall became isoechoic showing normal thickness on echocardiography (Fig. 2C). After receiving six cycles of R-CHOP including the initial early cycle, the patient showed a complete response, without recurrence or symptoms of heart failure. To date, he has been alive for 12 months with no sign of recurrence.
Discussion
Unlike malignant lymphoma arising in other regions, PCL uniquely causes cardiovascular complications including heart failure, cardiac tamponade, arrhythmia, and embolism [2], [3]. Generally, R-CHOP is an effective treatment for patients with good-prognosis DLBCL [4]. However, this patient had to undergo emergency tumor reduction, as he had acute hemodynamic failure due to tricuspid valve obstruction, even in DLBCL cases. Additionally, the destruction of the tumor by effective chemotherapy could lead to tricuspid obstruction or pulmonary embolism, potentially resulting in sudden death [5].
There are also two further factors to consider. The first is the considerable risk of hematogenous dissemination of tumor cells, and recurrence, which may be caused by the cardiopulmonary bypass [6]. In our case, however, the need to prevent acute circulatory failure took priority over this consideration. Therefore, the second stage of the treatment, the postoperative chemotherapy, was administered as soon as possible after tumor resection. Secondly, given an alternative scenario in which the tumor was not PCL, we would have considered employing a collagen gel droplet-embedded culture drug sensitive test to identify an effective anticancer drug [7].
Based on these considerations, we planned a 2-stage strategy involving reduction surgery to restore stable hemodynamics, followed by chemotherapy. This report demonstrated the efficacy of combined treatment with surgery and chemotherapy for PCL associated with cardiovascular complications.
Conflict of interest
No potential conflict of interest relevant to this case was reported.
References
- 1.Wold L.E., Lie J.T. Cardiac myxomas: a clinicopathologic profile. Am J Pathol. 1980;101:219–240. [PMC free article] [PubMed] [Google Scholar]
- 2.Butany J., Nair V., Naseemuddin A., Nair G.M., Catton C., Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005;6:219–228. doi: 10.1016/S1470-2045(05)70093-0. [DOI] [PubMed] [Google Scholar]
- 3.Patel J., Melly L., Sheppard M.N. Primary cardiac lymphoma: B- and T-cell cases at a specialist UK centre. Ann Oncol. 2010;21:1041–1045. doi: 10.1093/annonc/mdp424. [DOI] [PubMed] [Google Scholar]
- 4.Pfreundschuh M., Trumper L., Osterborg A., Pettengell R., Trneny M., Imrie K., Ma D., Gill D., Walewski J., Zinzani P.L., Stahel R., Kvaloy S., Shpilberg O., Jaeger U., Hansen M. CHOP-like chemotherapy plus rituximab versus CHOP-like chemotherapy alone in young patients with good-prognosis diffuse large-B-cell lymphoma: a randomised controlled trial by the MabThera International Trial (MInT) Group. Lancet Oncol. 2006;7:379–391. doi: 10.1016/S1470-2045(06)70664-7. [DOI] [PubMed] [Google Scholar]
- 5.Molajo A.O., McWilliam L., Ward C., Rahman A. Cardiac lymphoma: an unusual case of myocardial perforation – clinical, echocardiographic, haemodynamic and pathological features. Eur Heart J. 1987;8:549–552. doi: 10.1093/oxfordjournals.eurheartj.a062317. [DOI] [PubMed] [Google Scholar]
- 6.Akchurin R.S., Davidov M.I., Partigulov S.A., Brand J.B., Shiriaev A.A., Lepilin M.G., Dolgov I.M. Cardiopulmonary bypass and cell-saver technique in combined oncologic and cardiovascular surgery. Artif Organs. 1997;21:763–765. doi: 10.1111/j.1525-1594.1997.tb03738.x. [DOI] [PubMed] [Google Scholar]
- 7.Kobayashi H. Development of a new in vitro chemosensitivity test using collagen gel droplet embedded culture and image analysis for clinical usefulness. Recent Results Cancer Res. 2003;161:48–61. doi: 10.1007/978-3-642-19022-3_5. [DOI] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.