A very rare case of extranodal B-cell non-Hodgkin lymphoma presenting with adrenal and heart involvement

Abstract

We report an extremely rare case of extranodal B-cell NHL: DLBCL (diffuse large B-cell non-Hodgkin lymphoma), stage IVE, presenting with heart and bilateral adrenal involvement. On admission, adrenal and thorax imaging identified large bilateral adrenal masses and a 4.6 cm mass in the right atrium wall. An adrenal biopsy revealed the presence of a DLBCL, with triple expression of bcl2, bcl6, C-MYC(+70%). Following six cycles of systemic immunochemotherapy with R-DA-EPOCH, and high methotrexate dose for CNS prophylaxis a marked decrease of lymphoma infiltration was observed. The selection of the appropriate treatment modality can lead to profound response and improve patient's outcome.

Keywords: Adrenal lymphoma; Cardiac lymphoma; Diffuse large b-cell non-hodgkin lymphoma; Non-hodgkin's lymphoma; PCL, primary cardiac lymphoma.

Fig. 1

A: Contrast-enhanced adrenal CT: comparisons of adrenal masses before and after treatment. i: bilateral ill defined adrenal masses of 6.7 cm on the right and of 3.7 cm on the left adrenal and ii: significant bilateral masses’ regression post-treatment. B: Cardiac MRI (balanced SSFP sequences): Comparisons of cardiac mass before and after treatment i: mass of 4.6 × 5.5 cm of reduced signal surrounding the right atrium and ii: significant mass regression post-treatment C: PET-CT scan. i: High accumulation of FDG in both the cardiac atrium (SUVmax 21.2) and adrenals tumors (SUVmax 20.5). ii: The abnormal uptake disappeared after 6 cycles of immunochemotherapy.

Fig. 2

Immunohistochemistry of adrenal neoplastic tissue establishing the diagnosis of a DLBCL. The neoplastic cells show positivity for CD20, bcl2, bcl6 and MUM1 whereas c-myc is positive in 70% of the neoplastic nuclei.