Primary Adrenal Lymphoma with Paraneoplastic Syndrome
This was a case report of 76-year-old male patient who was transferred to our facility with a 3 weeks history of headache, confusion, agitation, difficulty with speech, inappropriate body movements, anorexia and abdominal pain. On presentation, he had a temperature of 38.4°C, cognitive impairment and generalized weakness. His complete blood count, comprehensive metabolic panel, thyroid stimulating hormone, sedimentation rate, ammonia, vitamin B12 levels and anti-nuclear antibody test were negative. Patient was started on vancomycin and piperacillin/tazobactam for his fevers. Magnetic resonance imaging (MRI) of the brain showed tiny hyper-intensities in the right cerebellum on diffusion weighted sequence. Cerebrospinal fluid (CSF) analysis showed a normal cell count and glucose with elevated protein of 102 mg/dl (normal 15-45 mg/dl) and negative cultures. Electroencephalogram showed moderate to severe diffuse encephalopathic process in both hemispheres. His cultures remained negative. As no obvious source of his fever was found, a computed tomography (CT) of chest, abdomen and pelvis was done to rule out occult infection. CT of abdomen revealed 8.6 cm × 6.8 cm × 13 cm left adrenal mass displacing the left kidney laterally []. Adrenal hormone levels were normal and adrenal insufficiency was not demonstrated. Enzyme-linked immunosorbent assay immunoassay was positive for tau protein (1617 pg/ml) in CSF while the Creutzfeldt Jakob 14-3-3 test was ambiguous. Paraneoplastic antibody work-up showed N-type calcium channel antibody, though his CSF antineuronal nuclear antibodies, purkinje cell cytoplasmic antibodies, antiglial nuclear antibody and amphiphysin antibodies were negative.\nBiopsy of the adrenal gland revealed diffuse large B cell lymphoma positive for CD20 and Ki-67 stain showing a proliferative index >90%. Immunostains for cyclin D-1, CD10, S-100, chromogranin A, pankeratin and calretinin were negative []. Chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab (CHOP-R) was administered followed by involved field radiation therapy. He had resolution of his neurological symptoms and repeat positron emission tomography (PET)-CT scan revealed a residual mass measuring 3.2 cm × 2 cm in the left suprarenal area without hypermetabolic activity [].