Primary mediastinal (thymic) large B cell lymphoma with aberrant expression of CD3: a case report with review of the literature.
We report the first case of primary mediastinal large B cell lymphoma (PMBL) with aberrant expression of CD3. PMBL is a subtype of diffuse large B cell lymphoma (DLBCL) and usually presents with bulky mediastinal lesions. Lineage ambiguity/infidelity is uncommon in DLBCL but has been described in sporadic case reports/series. A literature search identifies 13 additional cases of DLBCL expressing CD3, with the majority displaying cytoplasmic expression. Of the 14 total cases, 6 are pyothorax-associated lymphoma, 4 are conventional DLBCL, 2 are plasmablastic lymphoma, one is primary effusion lymphoma and one is PMBL. Two cases show genotypic ambiguity/infidelity with dual clonal IG and TCR gene rearrangements in addition to ambiguous immunophenotypes. Of the 13 cases tested for EBV status, 11 are positive, suggesting an important role of EBV in promoting lineage ambiguity/infidelity. A low threshold for testing EBV status is advocated in DLBCL with phenotypic ambiguity along with panels of immunohistochemical and molecular studies.
Department of Pathology, Duke University Medical Center, DUMC Box 3712, M-345 Davison Bldg Green Zone, Duke Hospital South, Durham, NC 27710, USA. endi.wang@duke.edu
Endi
E
Wang
Maggie
M
Stoecker
D000328:Adult; D001706:Biopsy; D017252:CD3 Complex / Q000378:metabolism*; D005260:Female; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology*; D008479:Mediastinal Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology*; D011859:Radiography; D013953:Thymus Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology
D017252:CD3 Complex