
Hematology Specialist Association 18. National Congress
More infoThe adrenal glands do not contain lymphoid tissue, and primary adrenal lymphoma (PAL) is extremely rare, accounting for less than 1% of all non-Hodgkin lymphomas and 3% of primary extranodal lymphomas [1, 2]. PAL is primarily bilateral. Approximately 250 cases have been described in the literature to date, with most published articles on PAL being case series with only a limited number of patients.
Case Report74-year-old male patıent with known type 2 dm diagnosis, the patient was admitted to our hospital emergency department with complaints of nausea, fatigue, and drowsiness and was followed up in the endocrinology department. laboratory parameters revealed tsh: 0.02 t4: 0.58 Acth: 32.3 Cortisol 7.05 Na: 124 mmol/l K: 4.6 mmol/l. the patient was first given corticosteroids and then levothyroxine replacements in endocrine follow-ups. contrast-enhanced pituitary and brain mrıs revealed a suspected microadenoma in the left posterior adenohypophysis and suspicious inflammation findings in both optic nerve sheaths. pet ct showed a lesion measuring 41 × 31 mm (suvmax: 19.8) in the right adrenal gland and approximately 40 × 35 mm (suvmax: 21.07) in the left adrenal gland. low-level increased fdg uptake was observed in the th4 vertebra, l4 vertebra and left femur proximal diaphyseal region. the patient underwent a right adrenal gland biopsy and it was found to be non-hodgkin lymphoma, diffuse large b cell lymphoma (germinal center phenotype) cd 20 +, cd10 +, bcl 6 +, bcl 2+, cmyc: 50% +. mild lymphocytosis was observed in the bone marrow aspiration biopsy. DA-R-EPOCH treatment was applıed to the patıent, who was conscıous, orıented, co-operated, general condıtıon and good oral ıntake under cortıcosteroıd and levothyroxıne treatment ın the follow-up performance and was externed as no complıcatıons were observed.
DiscussionPAL is extremely rare, primary adrenal DLBCL (PA-DLBCL) is of a non-germinal center B cell (nonGCB) phenotype. PAL usually has no excretory endocrine function and the symptoms are due to the pressure effect of the mass, whereas adrenal insufficiency usually exists.The most common manifestations were B symptoms, which include unexplained fever, weight loss, night sweats (68%), vague abdominal pain (42%), and fatigue (36%), some of which were present in the current patient. There is no correlation between tumor size and adrenal insufficiency. Generally, obvious clinical manifestations of adrenal insufficiency tend to appear when > 90% of the adrenal gland is damaged . It can improve with the destruction of the lymphomatous tissue at the end of the chemotherapy.