Our patient is a 52-year-old Malay woman who presented with a three-week history of intermittent pyrexia. This was associated with an unintentional weight loss of 10 kg over three months. She has a past medical history of asthma, hypertension, and hyperlipidaemia.
There were no specific localizing symptoms including cough, sputum, haemoptysis, dysuria, abdominal pain, or diarrhoea. There was also no history of travel.
Parameters revealed pyrexia of 40 degrees Celsius and sinus tachycardia of 113 beats per minutes but normal blood pressure. Physical examination was unremarkable with normal heart and breath sounds. Abdomen was soft, nontender with no palpable masses. There was no cervical, axillary, or inguinal lymphadenopathy. There were no neurological deficits and stigmata of liver disease.
Initial investigations showed microcytic hypochromic anaemia with a haemoglobin of 9.8 g/dL and mean cell volume of 75.2 fL. Renal function was unremarkable, but there was a raised lactate dehydrogenase (LDH) level of 2140 U/L. Other blood results are summarised in Table 1.
A computed tomography scan of the thorax abdomen and pelvis was performed, and it showed bilateral homogenous adrenal masses measuring 3.7 x 5.4 x 6.4 cm on the left and 5.5 x 2.7 x 5.9 cm on the right (Figures 1 and 2). There was also a prominent left supraclavicular lymph node measuring 0.8 cm and a few subcentimetre mediastinal lymph nodes measuring up to 0.8 cm.
Early morning cortisol was subsequently found to be low at 242 nmol/L and short Synacthen test with 250 mcg of tetracosactide did not show adequate cortisol response (Table 1). Acid Fast Bacilli stain and culture alongside with Polymerase Chain Reaction for Tuberculosis (TB PCR) of the patient was negative.
A left adrenal biopsy was performed, and histology revealed sheets of large lymphoid cells with a prominent intravascular growth pattern. The neoplastic cells were positive for CD20, CD79A, MUM1, BCL6, and BCL2. There was no expression for CD10, consistent with a nongerminal centre B-cell origin. MYC was positive in 70% of tumour cells and Ki-67 proliferation index was 90%. CD5 was positive; Cyclin D1 and SOX11 were negative, which also excludes mantle cell lymphoma. Bone marrow biopsy did not show any marrow involvement (Figure 6).
The patient was reviewed by a Hematologist, and the decision was made to manage her as for Stage 4 Diffuse Large B-Cell Lymphoma (DLBCL) in view of organ involvement. Her Eastern Cooperative Oncology Group (ECOG) status was zero, and International Prognostic Index (IPI) was one because of raised lactate dehydrogenase. She was treated with six cycles of R-CHOP and four cycles of intrathecal methotrexate chemotherapy.
Remarkably, repeat computer tomography scan after four cycles of R-CHOP chemotherapy showed interval regression of bilateral adrenal masses with nearly normal adrenal glands (Figure 3). The left enlarged supraclavicular and mediastinal nodes remained stable in size.
A Positron Emission Tomography (PET) scan six weeks after completion of both R-CHOP and intrathecal methotrexate revealed no fluorodeoxyglucose (FDG) update in the chest, abdomen, or pelvis and the enlarged left supraclavicular lymph node showed mild-FDG avidity that was smaller in size compared to previous computed tomography scans. This was discussed in a multidisciplinary tumour board, and it was decided to consider this as a reactive lymph node (please see Figures 4 and 5).
She remained in complete remission five months after chemotherapy and have since returned to work. However, repeat Synacthen test showed persistent adrenal insufficiency, and she remained on hydrocortisone replacement therapy. She is being monitored closely by a Hematologist and an Endocrinologist.