Vol 11, No 3 (2020)
Review paper
Published online: 2020-09-29

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Primary adrenal lymphomas as interdisciplinary endocrinological and hematological problem: practical guidance on diagnosis and treatment

Joanna Szydełko1, Michał Litwińczuk2, Magdalena Szydełko3, Monika Podhorecka4, Agnieszka Szymczyk5
Hematologia 2020;11(3):125-165.

Abstract

Primary adrenal lymphomas (PAL) are rare neoplasms that should be considered in the differential diagnosis of adrenal insufficiency and incidentally detected focal lesions within the adrenal glands, especially those located bilaterally. PAL are tumors characterized by a rapid growth, an aggressive course and a poor prognosis. The disease usually develops in the elderly, most often in the 6.–7. decade of life, 2–7 times more often in men than in women. The etiopathogenesis of PAL is complex, multifactorial and still not fully understood.. The most commonly reported symptoms include fever of unknown origin, abdominal pain, fatigue, and weight loss. In the case of lesions localized on both sides, symptoms of adrenal insufficiency are relatively common. PAL may also be clinically silent and be detected as incidentalomas. The examination of choice in PAL diagnostics is computed tomography (CT) of the abdominal cavity aimed at the adrenal glands and combined with hormonal assessment. The gold standard in the diagnosis of PAL is percutaneous fine-needle aspiration biopsy under ultrasound or CT guidance, which allows for the final diagnosis. Histopathologically, the vast majority of cases are diffuse large B-cell lymphoma. It has been suggested to combine various methods of treatment, including immunochemotherapy/chemotherapy, surgery with subsequent immunochemotherapy/chemotherapy and/or radiotherapy. The purpose of the following systematic review was to perform an interdisciplinary analysis of the clinical picture, pathogenesis, diagnostic methods and current therapeutic strategies, taking into account the prognosis for individual PAL subtypes.

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