Double-hit primary unilateral adrenal lymphoma with good outcome

Olivera Marković; Dragomir Marisavljević; Svetlana Jelić; Biljana Mihaljević; Taamara Martinović; Vesna Čemerikić

doi:10.2298/3401

Olivera Marković Clinical Hospital Center “Bežanijska kosa”, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Dragomir Marisavljević Clinical Hospital Center “Bežanijska kosa”, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Svetlana Jelić Clinical Hospital Center “Bežanijska kosa”, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Biljana Mihaljević Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Clinic of Hematology, Clinical Center of Serbia, Belgrade, Serbia
Taamara Martinović Faculty of Medicine, University of Belgrade, Belgrade, Serbia
Vesna Čemerikić Beolab, Belgrade, Serbia

DOI: https://doi.org/10.2298/3401

Keywords: lymphoma, non-hodkin, adrenal gland neoplasms, drug therapy, surgical procedures, operative, prognosis,

Abstract

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 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).

Author Biography

Olivera Marković, Clinical Hospital Center “Bežanijska kosa”, Belgrade, Serbia; Faculty of Medicine, University of Belgrade, Belgrade, Serbia

Department of Hematology

References

Kumar R, Xiu Y, Mavi A, El-Haddad G, Zhuang H, Alavi A. FDG-PET imaging in primary bilateral adrenal lymphoma: a case report and review of the literature. Clin Nucl Med 2005; 30(4): 222−30.

Kim YR, Kim JS, Min YH, Hyunyoon D, Shin H, Mun Y, et al. Prognostic factors in primary diffuse large B-cell lymphoma of adrenal gland treated with rituximab-CHOP chemotherapy from the Consortium for Improving Survival of Lymphoma (CISL). J Hematol Oncol 2012; 5(1): 49−51.

Yang Y, Li Q, Pan Y. Bilateral primary adrenal lymphoma. Br J Hematol 2010; 150(3): 250−4.

Singh D, Kumar L, Sharma A, Vijayaraghavan M, Thulkar S, Tan-don N. Adrenal Involvement in Non-Hodgkin's Lymphoma: Four Cases and Review of Literature. Leuk Lymphoma 2004; 45(4): 789−94.

Tomita N, Tokunaka M, Nakamura N, Takeuchi K, Koike J, Mo-tomura S, et al. Clinicopathological features of lympho-ma/leukemia patients carrying both BCL2 and MYC translocations. Haematologica 2009; 94(7): 935−43.

Grigg AP, Connors JM. Primary adrenal lymphoma. Clin Lym-phoma 2003; 4(3): 154−60.

Mozos A, Ye H, Chuang W, Chu J, Huang W, Chen H, et al. Most primary adrenal lymphomas are diffuse large B-cell lymphomas with non-germinal center B-cell phenotype, BCL6 gene rearrangement and poor prognosis. Mod Pathol 2009; 22(9): 1210−7.

Goldin LR, Landgren O. Autoimmunity and lymphomagenesis. Int J Cancer 2009; 124(7): 1497−502.

Lam KY, Lo CY. Metastatic tumours of the adrenal glands: a 30-year experience in a teaching hospital. Clin Endocrinol 2002; 56(1): 95−101.

Reddy SV, Prabhudesai S, Gnanasekaran B. Origin of primary adrenal lymphoma and predisposing factors for primary adrenal insufficiency in primary adrenal lymphoma. Indian J Endocrinol Metab 2011; 15(4): 350−1.

Spyroglou A, Schneider HJ, Mussack T, Reincke M, von Werder K, Beuschlein F. Primary adrenal lymphoma: 3 case reports with different outcomes. Exp Clin Endocrinol Diabetes 2011; 119(4): 208−13.

Lim KH, Chiou TY, Lin CJ, Hsieh RK. Rituximab in the treat-ment of primary bilateral adrenal lymphoma with adrenal cri-sis. Med Oncol 2008; 25(1): 107−9.

Savage KJ, Johnson NA, Ben-Neriah S, Connors JM, Sehn LH, Fa-rinha P, et al. MYC gene rearrangements are associated with a poor prognosis in diffuse large B-cell lymphoma patients treated with R-CHOP chemotherapy. Blood 2009; 114(17): 3533−7.

Johnson NA, Slack GW, Savage KJ, Connors JM, Ben-Neriah S, Rogic S, et al. Concurrent expression of MYC and BCL2 in diffuse large B-cell lymphoma treated with rituximab plus cyclophos-phamide, doxorubicin, vincristine, and prednisone. J Clin On-col 2012; 30(28): 3452−9.

Smith A, Eyvazzadeh D, Kavic SM. Laparoscopic adrenalectomy for unsuspected unilateral primary adrenal lymphoma. JSLS 2011; 15(3): 427−9.

Shirao S, Kuroda H, Kida M, Watanabe H, Matsunaga T, Niitsu Y, et al. Effective combined modality therapy for a patient with primary adrenal lymphoma. Rinsho Ketsueki 2006; 47(3): 204−9. (Japanese)

Horiguchi K, Hashimoto K, Hashizume M, Masuo T, Suto M, Okajo J, et al. Primary bilateral adrenal diffuse large B-cell lymphoma demonstrating adrenal failure. Intern Med 2010; 49(20): 2241−6.