Double-hit primary unilateral adrenal lymphoma with good outcome

Olivera Markovic, Dragomir Marisavljevic, Svetlana Jelic, Biljana Mihaljevic, Tamara Martinovic, Vesna Cemerikic
2014 Vojnosanitetski Pregled  
Introduction. Primary adrenal non-Hodgkin's lymphoma (NHL) is a rare neoplasm with poor prognosis. On the other side, double-hit lymphomas with BCL2 and MYC translocation are characterized by advanced disease stage, extranodal and central nervous system involvements at presentation or disease progression. Case report. We reported a 73-year-old male patient with double-hit primary adrenal lymphoma and preserved adrenal function, showing a favorable clinical course. Computed tomography of abdomen
more » ... mography of abdomen showed a 9 7 cm mass of the left adrenal gland. Laparatomy with left adrenalectomy was done and histological examination revealed diagnosis of a diffuse large B-cell NHL (DLBCL), non-GCB subtype. The patient was treated with 6 cycles of R-CHOP chemotherapy with reduced doses of doxorubicin because of the decreased left verticle ejection fraction. The patient was followed up regularly for 20 months with no evidence of tumor recurrence despite the inherently poor prognostic profile and double-hit phenotype of the disease. Conclusion. R-CHOP chemotherapy in combination with adrenalectomy can be an effective first-line regimen for primary adrenal DLBCL, despite the inherently poor prognostic profile (non-GCB subtype, bulky disease, elevated lactate dehydrogenase and double-hit phenotype of the disease). Uvod. Primarni adrenalni neho kinski limfom izuzetno je redak oblik limfoma i odlikuje se lošom prognozom. Sa druge strane, double-hit limfomi sa BCL2 i MYC translokacijom karakterišu se uznapredovalom boleš u u vreme postavljanja dijagnoze, esto prisutnom ekstranodalnom lokalizacijom bolesti i lošom prognozom. Prikaz bolesnika. Prikazali smo bolesnika starog 73 godine sa double-hit primarnim limfomom nadbubrega sa o uvanom adrenalnom funkcijom i povoljnim klini kim tokom. Kompjuterizovanom tomografijom abdomena registrovana je tumorska promena leve adrenalne žlezde, veli ine 9 7 cm, dok nije utvr eno prisustvo bolesti na drugim lokalizacijama. U injeno je kompletno hirurško ukljanjanje tumorske promene i na osnovu histološkog pregleda postavljena je dijagnoza difuznog B krupno elijskog limfoma, (DLBCL) non-GCB podtipa. Nakon hirurškog le enja primenjen je R-CHOP protokol sa redukovanim dozama adriablastina zbog smanjene ejekcione frakcije leve komore. I pored veoma lošeg prognosti kog profila bolesti (non-GCB tip, bulky bolesti, visokih vrednosti laktat dehidrogenaze, visokog komorbiditetnog skora i double-hit fenotipa) bolesnik je ve 20 meseci u kompletnoj remisiji. Conclusion. Imunohemioterapija (R-CHOP) u kombinaciji sa hirurškim le enjem je efikasna prva linija terapije kod bolesnika sa primarnim adrenalnim difuznim B-krupno elijskim limfomom i pored nepovoljnog prognosti kog profila i double-hit fenotipa bolesti. Klju ne re i: limfom, nehodžkinov; nadbubrežne žlezde, neoplazme; le enje lekovima; hirurgija, operativne procedure; prognoza. Strana 690 VOJNOSANITETSKI PREGLED Volumen 71, Broj 7 Markovi O, et al. Vojnosanit Pregl 2014; 71(7): 689-692.
doi:10.2298/vsp1407689m pmid:25109118 fatcat:jemrxudauvhu3a4qp3tijzeubu