Endocrine Abstracts

An 80-year-old patient was referred due to an incidental finding of a right adrenal mass during investigation of Iron Deficiency Anaemia (Ferritin 11.7 μg/l). CT scan of abdomen and pelvis revealed a 10×5 cm ill-defined heterogeneous mass within the right supra-renal space with areas of enhancement in the arterial phase imaging without calcification. Clinically, the patient lacked signs of endocrinopathy, such as (catecholamine or corticosteroid excess). However, the history was positive for drenching night sweats, fatigue and abdominal discomfort for several months. The initial investigations were unremarkable. Plasma metanephrine 10 ng/l (<100). Normetanephrines 57 ng/l (<170), Overnight dexamethasone suppression 0900 h Cortisol-19 nmol/l (0–50), Urine steroid profile showed normal total cortisol metabolite output, ACTH 19 ng/l (<50), Renin 21 mU/l (5-60), Aldosterone 283 pmol/l (100-800), Renin aldosterone ratio <80, Androstenedione <0.5 nmol/l (2-5.4), Total testosterone 0.1 nmol/L (0.1-1.42), SHBG 91 nmol/l (17.3-125), DHEA <0.1 μmol/l (0.9-2.1), 17b Oestradiol 48 pmol/l (114-332),17 hydroxyprogesterone <1, FSH 55.3 iu/l (3.5-12.5) and LH 31 iu/l (2.4-12.6). A dedicated CT adrenal revealed a large right adrenal mass 10.4 cm×5 cm with pre-contrast density of 40 HU, Portal venous phase density approximately 80–100 HU and delayed post-contrast density approximately 60 HU. The case was discussed in the Adrenal MDT and differentials included lymphoma, Sarcoma or inflammatory condition. Patient underwent a CT guided biopsy which showed diffuse staining of monoclonal lymphoplasmacytic infiltrate. Immunohistochemistry revealed positive CD20 B-Cell markers in addition to other B cell marker (BCL2 and IgM) with low proliferation index and no staining for CD10, CD5 and BCL6. These features indicated extra-nodal marginal zone lymphoma. Staging PET CT displayed moderate to intense uptake (SUVmax 8) in the right adrenal mass with no active lymphoproliferative disease elsewhere. Patient was referred to haematology MDT and subsequently started on Rituximab monotherapy for adrenal marginal zone lymphoma. At the point of writing, patient remained under oncology follow up. Primary adrenal lymphoma (PAL) is rare (<1%) of all extranodal lymphomas. While diffuse large B cell lymphoma is more prevalent and associated with a poor prognosis, marginal zone lymphoma, a low grade B cell neoplasm can rarely occur presenting as a relatively indolent condition. The diagnosis is usually challenging and pathological examination is the only approach to establish the diagnosis. The role of surgery for the management remains controversial. Immunochemotherapy typically employing Rituximab, is the standard treatment for symptomatic patients with extra nodal Marginal Zone Lymphoma of various origins.