Primary adrenal lymphoma with paraneoplastic syndrome

Abstract

Context: The adrenal gland is a common site for neoplastic diseases and primary adrenal lymphoma (PAL) is a rare tumor with around 120 cases reported so far.

Case report: We present a rare case of 76-year-old male who presented with headache, confusion, inappropriate body movements and abdominal pain. Adrenal biopsy revealed PAL and he has had an excellent neurologic outcome to date with chemotherapy and involved field radiation.

Conclusion: The majority of cases of PAL are B cell lymphomas with diffuse large cell in 70% of cases. Clinical symptoms are variable and patients may present with abdominal pain, fever, anorexia, weight loss, fatigue or symptoms of adrenal insufficiency. Therapeutic modalities for PAL include surgery, chemotherapy and radiotherapy and corticosteroid replacement. With this case report, we hope to raise awareness about this rare disease and to include lymphoma in the differential of adrenal masses.

Keywords: Diffuse large B cell lymphoma; Encephalopathy; Paraneoplastic syndrome; Primary adrenal lymphoma.

Figure 1

Computed tomographic scan of abdomen showing decrease in the size of left adrenal mass with treatment

Figure 2

(a) Adrenal biopsy showing neoplastic cells with nuclear pleomorphism and prominent nucleoli with a relatively dark cytoplasm. (b) The cells show diffuse strong staining for B-cell marker CD20 and a very high proliferation rate on the Ki-67 stain (c)