Category: Adrenal Disorders
Bilateral primary adrenal lymphomas (PAL) are very rare, accounting for less than 1% of Non-Hodgkin's lymphoma. We present a case of 66 year old male with bilateral primary adrenal lymphoma with rapid progressive course.
CASE PRESENTATION: A 66 year old male who presented to the hospital with three months of failure to thrive. His medical history was significant for coronary artery disease and atrial fibrillation. CT abdomen revealed 11 cm mass in left and 8cm on the right adrenal gland. The masses were described as large and lobular with metastasis to be high on the differential per the radiologist. As compared to a CAT scan of his abdomen 10 years ago that revealed normal adrenal glands. LDH level was found to be elevated at 410 units/L (Normal:94-250).After a complete biochemical work up indicated nonsecretory masses, the patient underwent core biopsy of the left adrenal mass. Unfortunately, the patient sustained rapid clinical deterioration due to development of an aspiration pneumonia. Goals of care discussed with patient and family and they chose comfort measures only. The patient passed away shortly after. Results of the biopsy revealed diffuse large B cell lymphoma, germinal center phenotype .Cytogenetic testing was positive for BCL- 6 rearrangement and negative for c-MYC, BCL- 2.
Results : -
PAL non-Hodgkin's lymphoma should be considered in the differential diagnosis of adrenal masses specially if bilateral and large masses . MRI findings for adrenal lymphoma are characterized by low signal intensity on T-1 and high signal intensity on T2 . An elevated LDH is characteristic of lymphoma. Optimal management is debatable because
Conclusion : Primary adrenal lymphoma is rare and management decisions are based on small case series .R-CHOP treatment has shown to have promising results in a few case reports. However larger studies should be carried out for validation of this treatment modality. Predictors of poor prognosis are reported to be advanced age, large tumor, elevated LDH, bilateral disease.
Aboorva K Sudhakar– Endocrine Fellow, Baystate Health, Massachusetts
Muhammad Khan– Resident, UMass- Baystate Medical center, Connecticut
Canan Gunay– Endocrine Fellow, Baystate Health
Neha Malhotra– Attending Physician, Baystate Health, springfield, Massachusetts
Baystate Health, Massachusetts
She received her medical degree from Coimbatore Medical College, India and completed her residency at Lahey Clinic, Burlington, MA.
UMass- Baystate Medical center, Connecticut
Internal medicine resident at UMass- Baystate Medical Center
Dr Canan Gunay received her medical degree from Kocaeli University Medical School, Turkey and completed her residency at St Vincent Medical Center, CT. She is currently a second year fellow at Baystate Medical Center, MA.