Primary Adrenal Lymphoma

In an attempt to investigate the clinical characters, laboratory features, and outcome of primary adrenal lymphoma, we analyzed the medical records of four patients with primary adrenal lymphoma and reviewed the literature. Three patients who accepted surgical excision died in 3 months; one patient died after 2 cycles of chemotherapy. The prognosis of primary adrenal lymphoma is very poor and early diagnosis and intervention may dramatically affect the clinical outcome. Secondary involvement of the adrenal glands by malignant lymphoma has been reported to occur in as many as 25% of the patients with the disease. Nevertheless, primary adrenal lymphoma is extremely uncommon and to the best of our knowledge only 83 cases have been reported worldwide. Because of the non-specific symptoms (absence of tumor cells in the peripheral blood), and the relatively acute clinical course, the correct diagnosis is very difficult to establish. We describe four patients and review the medical literature in order to define this clinical entity more clearly. Four patients aged 28–74 years (3 males and 1 female) are reported here. Symptoms included loin pain, weight loss, intermittent fever and abdominal discomfort. Physical examination revealed that blood pressures were normal. No lymphadenopathy was noted. All patients revealed a firm, slightly tender, and fixed mass on abdominal. Serum biochemistry showed almost normal levels of sodium, potassium, hepatatic enzymes, and creatinine. C-reactive protein (CRP) was 1.36–1.46 mg/dl (normal. , 0.8 mg/dl) and lactic acid dehydrogenase (LDH) was 485.0– 1609.5 u/l (normal.40 , 133 u/l). The serum cortisol levels were normal or slightly high. Ultrasonography revealed large bilateral masses with distinct borders in the region of both adrenal glands, and no normal adrenal glands could be identified. The size of the masses measured from 4.2 to 16.4 cm in diameter. The echos of these masses were low, and not much vascularity was evident on CDFI. Computer tomography (CT) showed large bilateral masses in the adrenal regions. Magnetic resonance images (MRI) of the masses showed complex density with obvious vascularity. Three patients agreed to tumor excision, and one received CHOP chemotherapy. Pathological examination showed that the normal structure of the adrenal had disappeared and was substituted by a diffuse, infiltration of lymphoid cells (T-cell 2 cases, B-cell 2 cases). Immunohistochemical studies showed CD20þ , CD3þ , CD68þ , CD45ROþ , Vimentinþ , Syn2 , CgA2 , Keratin2 . But three patients who had accepted surgical excision died within 3 months, one patient die after 2 cycles of chemotherapy. Primary adrenal malignant lymphoma (PAL) is extremely rare, and only a few case reports have been published in the medical literature [1–4]. According to the literature, the clinical picture is not specific. The presenting symptoms of primary adrenal NHL, such as abdominal discomfort, lumbar pain, fever, night sweats, nausea, vomiting, lethargy, fatigue and weight loss, may be related to the lymphoma itself or to adrenal insufficiency [5,6]. About 90–95% of adrenal issue must be destroyed before adrenal insufficiency is manifest. For this reason, although solid tumor metastases to the adrenal glands are common, they rarely produce symptoms of adrenal insufficiency. The median age at presentation of PAL is in the mid 60s with a male to female ratio of 2:1 and a unilateral lymphoma to bilateral lymphoma ratio of 1:3 [1]. It appears that there is no correlation between the size of the tumor and adrenal dysfunction. On examination, no lymphadenopathy is noted and hepatosplenomegaly may be detected. Usually, some masses can be palpated in the upper quadrant. Laboratory investigations reveal almost normal serum biochemistry, liver enzymes and renal function. The serum LDH level may be high although the relationship between serum LDH levels and adrenal tumors has not previously been appreciated [4], and some associated with adrenal insufficiency. Ultrasonography revealed large unilateral or bilateral masses in the region of the adrenal glands; normal adrenal glands could not be identified. Nevertheless the border of