Key result
Fli-1 positivity mimics angiosarcoma in primary cardiac diffuse large B-cell lymphoma.
Why the study?
Primary cardiac lymphoma is extremely rare and carries a high mortality rate unless recognized and treated in time, making accurate tissue pathology crucial for planning treatment.
Case Report (n=1)
No
Requires expanded IHC panels including CD20 in Fli-1-positive cardiac masses to avert angiosarcoma misdiagnosis; leaves open standardized diagnostic algorithms for rare primary cardiac lymphomas.
BACKGROUND: Primary cardiac lymphoma is an extra-nodal non-Hodgkin's lymphoma, which usually responds well to chemotherapy. The disease has high mortality rate unless it is recognized and treated in time. Tissue pathology is crucially the diagnosis gold standard for treatment plan. This is a case report of an elderly female who presented with a huge right-sided cardiac tumour obstructing tricuspid flow. CASE SUMMARY: An 81-year-old Asian female presented with clinical right-sided heart failure. Echocardiogram showed a large mass compressing and obliterating the right atrium. Trans-jugular tissue biopsy was performed. Initial pathology report was consistent with an angiosarcoma, based on an expression of Fli-1 (Friend leukaemia virus integration 1) from immunohistochemical staining. She died shortly after refusal to surgery. Autopsy was performed with diagnosis change to a diffuse large B-cell lymphoma (DLBCL) after tissue pathology. DISCUSSION: Primary cardiac lymphoma is extremely rare. Adequate tissue and proper immunohistochemical staining are mandatory for treatment plan. Besides an angiosarcoma, DLBCL should be considered in the differential diagnosis of Fli-1 positive tissue cardiac mass.
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Tanking et al. (2020) conducted a case report in Primary cardiac diffuse large B-cell lymphoma (DLBCL) initially misdiagnosed as angiosarcoma (n=1). Trans-jugular cardiac biopsy and immunohistochemical staining (Fli-1, ERG, CD20, Ki67) vs. Autopsy tissue pathology re-investigation was evaluated on Histopathological diagnosis of cardiac mass. Fli-1 positivity on initial biopsy without CD20 staining led to misdiagnosis of cardiac angiosarcoma in an 81-year-old female with primary cardiac diffuse large B-cell lymphoma confirmed at autopsy.
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