ESA-SRB-AOTA 2019

Primary adrenal lymphoma and adrenal insufficiency (#619)

Ashish Munsif 1
  1. Endocrinology, Nepean Hospital, Sydney, NSW, Australia

Background: Primary adrenal lymphoma (PAL) is a rare, aggressive malignancy that can cause adrenal insufficiency with bilateral lesions. Fewer than 200 cases of this condition have been reported worldwide, of which a small proportion are unilateral [1, 2].  

 

Clinical case: A 66 year old gentleman with a background of hypertension, diverticular disease and obesity presents to emergency with nonspecific abdominal pain during an interstate motorbike trip. Examination does not reveal any cushingoid features or hypertension. Abdominal computed tomography imaging demonstrates a 72x64x68mm ill-defined left adrenal mass suspicious for primary adrenal cortical carcinoma. Due to intractable pain despite oral analgesia, the patient represents to emergency a few days later and is admitted for pain management. Functional testing for adrenal insufficiency, subclinical Cushing’s syndrome, phaeochromocytoma and hyperaldosteronism are requested during this admission, all of which are ultimately negative. Mass related pain is hypothesised to be the primary cause for the symptoms, with haemorrhage into the mass a less likely differential. Repeat computed tomography and PET imaging reveals an enlarging mass with intense uptake (SUVmax 36.9) and development of extensive lymphadenopathy, including para-aortic and left internal mammary uptake (SUVmax 41.9). A left adrenal mass biopsy is subsequently performed that identifies an aggressive, triple hit, Diffuse Large B Cell Lymphoma (DLBCL). The patient is currently undergoing R-CHOP chemotherapy with mild improvement.

 

Conclusion:

  1. PAL typically present with bilateral adrenal lesions and have concurrent adrenal insufficiency.
  2. The most common subtype is Non-Hodgkin’s Lymphoma, specifically DLBCL.
  3. The presence of a unilateral lesion and adrenal preservation make this case extremely rare
  4. PAL can mimic endocrine pathologies and therefore should be considered as a differential for adrenal lesions, especially if these are bilateral with associated adrenal insufficiency
  5. Biopsy of the suspected lesion following exclusion of a functioning mass (particularly phaeochromocytoma) greatly aids with the diagnostic dilemma
  1. Rashidi, A., & Fisher, S. I. (2013). Primary adrenal lymphoma: a systematic review. Annals of Hematology, 92(12), 1583-1593.
  2. Zhang, M., Zhao, S., Hu, Y., & Yao, J. (2019). Correlations between MDCT features and clinicopathological findings of primary adrenal lymphoma. European Journal of Radiology.