Adrenal lymphoma 'the great imitator'

Aung, Nwe Ni, Oo, Win Htun, Richardson, Tristan, Bujanova, Jana, Evans, Nick, Dharmasiri, Michelle, Fairburn, Katharine, Marigold, Richard, Jupp, Becky and Marigold, James (2021) Adrenal lymphoma 'the great imitator'. Endocrine Abstracts, 77, [LB34]. (doi:10.1530/endoabs.77.lb34).

Abstract

Introduction: adrenal lymphoma and its association with intravascular lymphoma is rare but needs consideration in cases presenting with bilateral adrenal masses and unexplained neurological symptoms. We present two recent cases presenting with multiple cerebral infarcts associated with primary adrenal lymphoma.

Case 1: a 67-year-old male was admitted to the stroke unit with symptoms of middle cerebral artery (MCA) stroke. MRI demonstrated multiple infarcts. Repeat imaging after the patient’s rapid neurological decline showed multiple new cortical infarcts. Carotid doppler, echocardiogram, and vasculitic screen were all normal. LDH was raised-2554u/l (reference range: 225-425); CTCAP demonstrated bilateral adrenal masses suggesting an infiltrative process. PET showed highly avid adrenal glands. EUS-guided biopsy confirmed the presence of high-grade diffuse large B cell lymphoma. R-CHOP chemotherapy was commenced with no further cerebral infarcts.

Case 2: a 74-year-old female was re-admitted under the stroke team with worsening neurological deficit (after recent admission for MCA stroke). Repeat CT and MRI scans revealed multiple cerebral infarcts. CT angiogram, doppler, echocardiogram, and vasculitic screen were negative.LDH was 363u/l (reference range: 90-225); CTCAP showed marked enlargement of both adrenals, with PET-CT demonstrating bilaterally enlarged and hyper-metabolic adrenal glands. US-guided biopsy revealed a high-grade lymphoma.

Discussion: 1. There is a well-documented association between adrenal lymphoma and CNS disease. 2. Cerebral intravascular lymphoma is a ‘great imitator’ and can present with various neurological presentations without the classical characteristics of lymphoma. 3. The radiological features of adrenal lymphoma include enlargement of the adrenals whilst retaining the normal adrenal contours and significant avidity on PET. 4. Intravascular lymphoma poses a significant diagnostic challenge due to the lack of expected features of lymphoma (lack of symptoms without significant rises in LDH, CRP, or ESR in all cases). The association with adrenal hyperplasia and PET-avidity should alert clinicians to the diagnosis.

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