Case Report
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An Unusual Cause of Bilateral Adrenal Incidentaloma: A Case Report of Primary Adrenal Lymphoma

Year 2023, , 287 - 292, 29.10.2023
https://doi.org/10.46310/tjim.1348432

Abstract

Primary adrenal lymphoma (PAL) is an extremely rare among the causes of adrenal incidentaloma. Most were diagnosed with adrenal insufficiency and B symptoms (unexplained weight loss, night sweats, fever). This article presented a 57-year-old woman who was investigated for bilateral adrenal masses found incidentally on computed tomography (CT). Physical examination and laboratory tests revealed no evidence of adrenal insufficiency or B symptoms. Only 24-hour urinary metanephrine and normetanephrine excretion were increased. Tumour F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET/CT) scan showed the greatest dimension was 14 cm in the left adrenal mass and the maximum standardized uptake value (SUV max) was 26.1 (relative to mean SUV in the normal liver parenchyma, which was 2). An adrenal biopsy was performed after taking adequate precautions against the possibility of a catecholamine crisis. Histopathology revealed high-grade B-cell lymphoma. Bone marrow involvement and brain metastasis were not observed. She received the R-EPOCH (rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) regimen and intrathecal methotrexate therapy as central nervous system prophylaxis. The patient responded well to treatment, and close clinical follow-up continues. PAL should always be considered when a bilateral adrenal mass is detected.

Supporting Institution

Hacettepe University School of Medicine

References

  • Fassnacht M, Tsagarakis S, Terzolo M, Tabarin A, Sahdev A, Newell-Price J, Pelsma I, Marina L, Lorenz K, Bancos I, Arlt W, Dekkers OM. European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2023 Jul 20;189(1):G1-G42. doi: 10.1093/ejendo/lvad066.
  • Yang Y, Xie W, Ren Y, Tian H, Chen T. A case report of primary adrenal lymphoma: A rare but aggressive and invasive disease. Medicine (Baltimore). 2020 Jul 10;99(28):e20938. doi: 10.1097/MD.0000000000020938. 3.
  • Khurana A, Kaur P, Chauhan AK, Kataria SP, Bansal N. Primary non Hodgkin's lymphoma of left adrenal gland - A rare presentation. J Clin Diagn Res. 2015 Apr;9(4):XD01-XD03. doi: 10.7860/JCDR/2015/8079.5745.
  • Wang J, Sun NC, Renslo R, Chuang CC, Tabbarah HJ, Barajas L, French SW. Clinically silent primary adrenal lymphoma: a case report and review of the literature. Am J Hematol. 1998 Jun;58(2):130-6. doi: 10.1002/(sici)1096-8652(199806)58:2<130::aid-ajh8>3.0.co;2-t.
  • Grønning K, Sharma A, Mastroianni MA, Karlsson BD, Husebye ES, Løvås K, Nermoen I. Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases. Endocrinol Diabetes Metab Case Rep. 2020 Mar 10;2020:19-0131. doi: 10.1530/EDM-19-0131.
  • Schreiber CS, Sakon JR, Simião FP, Tomarchio MP, Huayllas M, Pereira LC, Stella LC, Santomauro AC Jr, Bueno SS, Fraige FF. Primary adrenal lymphoma: a case series study. Ann Hematol. 2008 Oct;87(10):859-61. doi: 10.1007/s00277-008-0492-x.
  • Rashidi A, Fisher SI. Primary adrenal lymphoma: a systematic review. Ann Hematol. 2013 Dec;92(12):1583-93. doi: 10.1007/s00277-013-1812-3.
  • Mozos A, Ye H, Chuang WY, Chu JS, Huang WT, Chen HK, Hsu YH, Bacon CM, Du MQ, Campo E, Chuang SS. 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 Sep;22(9):1210-7. doi: 10.1038/modpathol.2009.87.
  • Ohsawa M, Tomita Y, Hashimoto M, Yasunaga Y, Kanno H, Aozasa K. Malignant lymphoma of the adrenal gland: its possible correlation with the Epstein-Barr virus. Mod Pathol. 1996 May;9(5):534-43.
  • Wang Y, Ren Y, Ma L, Li J, Zhu Y, Zhao L, Tian H, Chen T. Clinical features of 50 patients with primary adrenal lymphoma. Front Endocrinol (Lausanne). 2020 Sep 24;11:595. doi: 10.3389/fendo.2020.00595.
  • Simpson WG, Babbar P, Payne LF. Bilateral primary adrenal non-Hodgkin's lymphoma without adrenal insufficiency. Urol Ann. 2015 Apr-Jun;7(2):259-61. doi: 10.4103/0974-7796.152942.
  • Kim YR, Kim JS, Min YH, Hyunyoon D, Shin HJ, Mun YC, Park Y, Do YR, Jeong SH, Park JS, Oh SY, Lee S, Park EK, Jang JS, Lee WS, Lee HW, Eom H, Ahn JS, Jeong JH, Baek SK, Kim SJ, Kim WS, Suh C. 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 Aug 13;5:49. doi: 10.1186/1756-8722-5-49.
  • Ekhzaimy A, Mujamammi A. Bilateral primary adrenal lymphoma with adrenal insufficiency. BMJ Case Rep. 2016 Oct 26;2016:bcr2016217417. doi: 10.1136/bcr-2016-217417.
  • Laurent C, Casasnovas O, Martin L, Chauchet A, Ghesquieres H, Aussedat G, Fornecker LM, Bologna S, Borot S, Laurent K, Bouillet B, Verges B, Petit JM. Adrenal lymphoma: presentation, management and prognosis. QJM. 2017 Feb 1;110(2):103-109. doi: 10.1093/qjmed/hcw174.
  • Coiffier B, Lepage E, Briere J, Herbrecht R, Tilly H, Bouabdallah R, Morel P, Van Den Neste E, Salles G, Gaulard P, Reyes F, Lederlin P, Gisselbrecht C. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med. 2002 Jan 24;346(4):235-42. doi: 10.1056/NEJMoa011795.
  • Kanemasa Y, Shimoyama T, Sasaki Y, Tamura M, Sawada T, Omuro Y, Hishima T, Maeda Y. Central nervous system relapse in patients with diffuse large B cell lymphoma: analysis of the risk factors and proposal of a new prognostic model. Ann Hematol. 2016 Oct;95(10):1661-9. doi: 10.1007/s00277-016-2744-5.
  • Lin Z, Chen X, Liu L, Zeng H, Li Z, Xu B. The role of central nervous system (CNS) prophylaxis in preventing DLBCL patients from CNS relapse: A network meta-analysis. Crit Rev Oncol Hematol. 2022 Aug;176:103756. doi: 10.1016/j.critrevonc.2022.103756.
  • Li S, Wang Z, Wu Z, Zhuang H, Xu Y. Clinical characteristics and outcomes of primary adrenal diffuse large B cell lymphoma in a large contemporary cohort: a SEER-based analysis. Ann Hematol. 2019 Sep;98(9):2111-9. doi: 10.1007/s00277-019-03740-9.
  • Pinar IE, Ozkocaman V, Ersal T, Ayhan EY, Gursoy V, Yalcin C, Orhan B, Candar O, Ozkalemkas F. Comparison of international prognostic indices and validation study for patients with diffuse large B-cell lymphoma in the rituximab era. Int J Hematol Oncol. 2023;33(2):57-65. doi: 10.4999/uhod.236953.
  • Somasundaram H, Boyer PN, Casey J, Wong M, Shenoy V. Primary adrenal lymphoma as a rare cause of primary adrenal insufficiency: Challenges in management and a review of the literature. AACE Clin Case Rep. 2022 May 20;8(5):199-203. doi: 10.1016/j.aace.2022.05.003.
Year 2023, , 287 - 292, 29.10.2023
https://doi.org/10.46310/tjim.1348432

Abstract

References

  • Fassnacht M, Tsagarakis S, Terzolo M, Tabarin A, Sahdev A, Newell-Price J, Pelsma I, Marina L, Lorenz K, Bancos I, Arlt W, Dekkers OM. European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2023 Jul 20;189(1):G1-G42. doi: 10.1093/ejendo/lvad066.
  • Yang Y, Xie W, Ren Y, Tian H, Chen T. A case report of primary adrenal lymphoma: A rare but aggressive and invasive disease. Medicine (Baltimore). 2020 Jul 10;99(28):e20938. doi: 10.1097/MD.0000000000020938. 3.
  • Khurana A, Kaur P, Chauhan AK, Kataria SP, Bansal N. Primary non Hodgkin's lymphoma of left adrenal gland - A rare presentation. J Clin Diagn Res. 2015 Apr;9(4):XD01-XD03. doi: 10.7860/JCDR/2015/8079.5745.
  • Wang J, Sun NC, Renslo R, Chuang CC, Tabbarah HJ, Barajas L, French SW. Clinically silent primary adrenal lymphoma: a case report and review of the literature. Am J Hematol. 1998 Jun;58(2):130-6. doi: 10.1002/(sici)1096-8652(199806)58:2<130::aid-ajh8>3.0.co;2-t.
  • Grønning K, Sharma A, Mastroianni MA, Karlsson BD, Husebye ES, Løvås K, Nermoen I. Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases. Endocrinol Diabetes Metab Case Rep. 2020 Mar 10;2020:19-0131. doi: 10.1530/EDM-19-0131.
  • Schreiber CS, Sakon JR, Simião FP, Tomarchio MP, Huayllas M, Pereira LC, Stella LC, Santomauro AC Jr, Bueno SS, Fraige FF. Primary adrenal lymphoma: a case series study. Ann Hematol. 2008 Oct;87(10):859-61. doi: 10.1007/s00277-008-0492-x.
  • Rashidi A, Fisher SI. Primary adrenal lymphoma: a systematic review. Ann Hematol. 2013 Dec;92(12):1583-93. doi: 10.1007/s00277-013-1812-3.
  • Mozos A, Ye H, Chuang WY, Chu JS, Huang WT, Chen HK, Hsu YH, Bacon CM, Du MQ, Campo E, Chuang SS. 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 Sep;22(9):1210-7. doi: 10.1038/modpathol.2009.87.
  • Ohsawa M, Tomita Y, Hashimoto M, Yasunaga Y, Kanno H, Aozasa K. Malignant lymphoma of the adrenal gland: its possible correlation with the Epstein-Barr virus. Mod Pathol. 1996 May;9(5):534-43.
  • Wang Y, Ren Y, Ma L, Li J, Zhu Y, Zhao L, Tian H, Chen T. Clinical features of 50 patients with primary adrenal lymphoma. Front Endocrinol (Lausanne). 2020 Sep 24;11:595. doi: 10.3389/fendo.2020.00595.
  • Simpson WG, Babbar P, Payne LF. Bilateral primary adrenal non-Hodgkin's lymphoma without adrenal insufficiency. Urol Ann. 2015 Apr-Jun;7(2):259-61. doi: 10.4103/0974-7796.152942.
  • Kim YR, Kim JS, Min YH, Hyunyoon D, Shin HJ, Mun YC, Park Y, Do YR, Jeong SH, Park JS, Oh SY, Lee S, Park EK, Jang JS, Lee WS, Lee HW, Eom H, Ahn JS, Jeong JH, Baek SK, Kim SJ, Kim WS, Suh C. 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 Aug 13;5:49. doi: 10.1186/1756-8722-5-49.
  • Ekhzaimy A, Mujamammi A. Bilateral primary adrenal lymphoma with adrenal insufficiency. BMJ Case Rep. 2016 Oct 26;2016:bcr2016217417. doi: 10.1136/bcr-2016-217417.
  • Laurent C, Casasnovas O, Martin L, Chauchet A, Ghesquieres H, Aussedat G, Fornecker LM, Bologna S, Borot S, Laurent K, Bouillet B, Verges B, Petit JM. Adrenal lymphoma: presentation, management and prognosis. QJM. 2017 Feb 1;110(2):103-109. doi: 10.1093/qjmed/hcw174.
  • Coiffier B, Lepage E, Briere J, Herbrecht R, Tilly H, Bouabdallah R, Morel P, Van Den Neste E, Salles G, Gaulard P, Reyes F, Lederlin P, Gisselbrecht C. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med. 2002 Jan 24;346(4):235-42. doi: 10.1056/NEJMoa011795.
  • Kanemasa Y, Shimoyama T, Sasaki Y, Tamura M, Sawada T, Omuro Y, Hishima T, Maeda Y. Central nervous system relapse in patients with diffuse large B cell lymphoma: analysis of the risk factors and proposal of a new prognostic model. Ann Hematol. 2016 Oct;95(10):1661-9. doi: 10.1007/s00277-016-2744-5.
  • Lin Z, Chen X, Liu L, Zeng H, Li Z, Xu B. The role of central nervous system (CNS) prophylaxis in preventing DLBCL patients from CNS relapse: A network meta-analysis. Crit Rev Oncol Hematol. 2022 Aug;176:103756. doi: 10.1016/j.critrevonc.2022.103756.
  • Li S, Wang Z, Wu Z, Zhuang H, Xu Y. Clinical characteristics and outcomes of primary adrenal diffuse large B cell lymphoma in a large contemporary cohort: a SEER-based analysis. Ann Hematol. 2019 Sep;98(9):2111-9. doi: 10.1007/s00277-019-03740-9.
  • Pinar IE, Ozkocaman V, Ersal T, Ayhan EY, Gursoy V, Yalcin C, Orhan B, Candar O, Ozkalemkas F. Comparison of international prognostic indices and validation study for patients with diffuse large B-cell lymphoma in the rituximab era. Int J Hematol Oncol. 2023;33(2):57-65. doi: 10.4999/uhod.236953.
  • Somasundaram H, Boyer PN, Casey J, Wong M, Shenoy V. Primary adrenal lymphoma as a rare cause of primary adrenal insufficiency: Challenges in management and a review of the literature. AACE Clin Case Rep. 2022 May 20;8(5):199-203. doi: 10.1016/j.aace.2022.05.003.
There are 20 citations in total.

Details

Primary Language English
Subjects Clinical Oncology
Journal Section Case Reports
Authors

Mert Tokatlı 0000-0003-4914-7903

Neslihan Nisa Gecici This is me 0009-0003-5315-8362

Meral Ilgaz Ergin This is me 0000-0002-4514-1937

Umit Yavuz Malkan This is me 0000-0001-5444-4895

Oguz Abdullah Uyaroglu This is me 0000-0003-0440-2026

Publication Date October 29, 2023
Submission Date August 23, 2023
Acceptance Date September 19, 2023
Published in Issue Year 2023

Cite

EndNote Tokatlı M, Gecici NN, Ergin MI, Malkan UY, Uyaroglu OA (October 1, 2023) An Unusual Cause of Bilateral Adrenal Incidentaloma: A Case Report of Primary Adrenal Lymphoma. Turkish Journal of Internal Medicine 5 4 287–292.

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