O-05 A RARE CAUSE OF BILATERAL GIANT ADRENAL MASS: PRIMARY ADRENAL LYMPHOMA
Authors: Ünal Fatma Tuğçe Şah , Emral Rıfat 
Published: January 2025

Introduction

Primary adrenal lymphoma (PAL) is an extremely rare form of non-Hodgkin lymphoma, representing less than 1% of cases. It is usually bilateral, unlike secondary adrenal lymphoma, which is often unilateral. Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of PAL, making up about 70% of cases. PAL primarily affects older patients and lacks distinct nodular lesions, making diagnosis difficult; pathological examination is essential for confirmation.

Clinical Case

A 62-year-old male patient with no known comorbidities presented with symptoms of night sweats, weight loss (approximately 10 kg over the past month), and general weakness persisting for the past two months. Physical examination revealed decreased breath sounds in the bilateral lower lung fields and a dullness to percussion over Traube's space on abdominal examination. No other pathological findings were noted. Vital signs were in normal range. Laboratory evaluation showed normocytic anemia, thrombocytopenia, and hypercalcemia with suppressed parathyroid hormone levels. Additionally, lactate dehydrogenase levels were elevated (see Table 1). A thoracic, abdominal, and pelvic computed tomography (CT) scan was performed to assess for malignancy. The CT scan revealed a solid mass lesion measuring approximately 11 x 18 x 10 cm with an average density of 50 HU, exhibiting non-homogeneous contrast enhancement. This lesion is located in the bilateral adrenal regions and show confluent extension anterior to the abdominal aorta (see Figure 1). Subsequently, the patient underwent hormonal analysis to assess adrenal function and an 18F-FDG PET/CT scan was planned. Hormonal analysis indicated that the adrenal mass was non-functional, with preserved adrenal gland function (see Table 2). The PET/CT scan showed intense pathological activity accumulation in the adrenal mass lesion (SUVmax: 25.9) and diffuse increased activity in the bone marrow. Following this, both bone marrow and adrenal mass biopsies were performed. The bone marrow biopsy did not reveal infiltrative pathology. However, the biopsy of the adrenal mass was reported as aggressive mature large B-cell lymphoma. Primary bilateral adrenal lymphoma was diagnosed. The patient was evaluated by hematology and subsequently initiated on R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy. Following six cycles of R-CHOP, the suprarenal mass is completely resolved. However, during follow-up one year later, the disease relapsed with a cranial mass.

Conclusion

PAL is a rare extranodal non-Hodgkin's lymphoma, predominantly affecting males around 70 years old. It usually presents with large bilateral adrenal masses and can cause primary adrenal insufficiency (PAI) in up to 70% of cases. Diagnosis is confirmed by histology after imaging. Treatment typically involves a combination of surgery, chemotherapy, and/or radiotherapy, with PAI requiring hormone replacement therapy.Figure 1:Imaging of the Tumor Lesion Before and After Chemotherapya/b) Pre/Post-Chemotherapy PET/CT Imaging, c/d) Pre/Post-Chemotherapy CT Imaging  Table 1:Patient’s laboratory parameters  Table 2:Patient’s adrenal function assessment(Values marked with an asterisk (*) were measured in a 24-hour urine collection, while the other values were measured in plasma.) (DHEAS: Dehydroepiandrosterone sulfate)