Please use this identifier to cite or link to this item: https://doi.org/10.1016/j.anndiagpath.2016.05.005
Title: Primary cardiac diffuse large B-cell lymphoma in immunocompetent patients: clinical, histologic, immunophenotypic, and genotypic features of 3 cases
Authors: Soon, Gwyneth 
Ow, Guan Wei 
Chan, Hian Li 
Ng, Siok Bian 
Wang, Shi 
Keywords: Science & Technology
Life Sciences & Biomedicine
Pathology
Cardiac
Large B-cell lymphoma
Immunocompetent
RITUXIMAB PLUS CYCLOPHOSPHAMIDE
POOR-PROGNOSIS
SURVIVAL
MYC
VINCRISTINE
DOXORUBICIN
PREDNISONE
EXPRESSION
HEART
BCL2
Issue Date: 1-Oct-2016
Publisher: ELSEVIER SCIENCE INC
Citation: Soon, Gwyneth, Ow, Guan Wei, Chan, Hian Li, Ng, Siok Bian, Wang, Shi (2016-10-01). Primary cardiac diffuse large B-cell lymphoma in immunocompetent patients: clinical, histologic, immunophenotypic, and genotypic features of 3 cases. ANNALS OF DIAGNOSTIC PATHOLOGY 24 : 40-46. ScholarBank@NUS Repository. https://doi.org/10.1016/j.anndiagpath.2016.05.005
Abstract: Primary cardiac lymphoma (PCL) is a rare extranodal lymphoma that involves only the heart and/or pericardium. Primary cardiac lymphoma is much less common in immunocompetent patients compared with those who are immunosuppressed. Patients with PCL have variable clinical manifestations that may lead to misdiagnosis and delay in treatment. Modern radiologic imaging now allows for earlier detection of these tumors. This study describes the clinical, histologic/cytologic, immunophenotypic, and molecular genetic findings for 3 immunocompetent patients with primary cardiac diffuse large B-cell lymphoma. All 3 patients had different initial clinical presentations. The neoplastic cells in all 3 cases were large in size, morphologically resembling diffuse large B-cell lymphoma. Neoplastic cells in 2 cases had non–germinal center (GC)-like (non–GC-like) and 1 case had GC-like immunophenotype. Neoplastic cells in all 3 cases showed C-MYC and BCL2 immunohistochemical protein coexpression. Neoplastic cells in 1 case showed double-hit MYC and BCL2 gene rearrangements, whereas another 1 case showed MYC gene rearrangement without BCL2 gene rearrangement. Epstein-Barr virus–encoded RNA was negative in the neoplastic cells in all 3 cases. All 3 patients received rituximab-based chemotherapy. Two patients subsequently had disease relapse at other extranodal sites at 10 and 24 months, respectively, whereas 1 patient was alive without disease at 9 months after diagnosis. If there is sufficient diagnostic tissue in these rare tumors, molecular studies should ideally be performed for prognostication and further patient management.
Source Title: ANNALS OF DIAGNOSTIC PATHOLOGY
URI: https://scholarbank.nus.edu.sg/handle/10635/206595
ISSN: 10929134
15328198
DOI: 10.1016/j.anndiagpath.2016.05.005
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