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Primary adrenal lymphoma presenting as Addison’s disease: case report and review of the literature

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Abstract

Primary adrenal lymphoma (PAL) is an extremely rare entity and adrenal insufficiency is a common complication. Bilateral enlargement of adrenal glands should raise the suspicion of lymphoma, especially in patients with clinical or laboratory features of adrenal insufficiency. Most of these cases are highly aggressive tumors and should be treated with multiagent chemotherapy. The role of bilateral adrenalectomy and/or radiotherapy cannot be estimated. Our patient with PAL was an 80-year-old man who presented with clinical and laboratory features of adrenal insufficiency. Combination chemotherapy plus rituximab was unsuccessful and our patient died from progressive disease.

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References

  1. Isaacson PG, Norton AJ (1994) Extranodal lymphomas. Churchill Livingstone, Edinburgh

  2. Lack EE, Gruhn JG (1996) Adrenal glands. In: Damianov I, Linder J (eds) Andersons Pathology, 10th edn. Mosby, St. Louis

  3. Lack EE (1997) Tumors of the adrenal gland and extra-adrenal paraganglia. Atlas of tumor pathology, fascicle 19. Third series. Armed Forces Institute of Pathology, Washington DC, p 182

  4. Falchook S, Allard JC (1991) CT of primary adrenal lymphoma. J Comput Assist Tomogr 15:1048

    CAS  PubMed  Google Scholar 

  5. Kung AWC, Pun KK, Lam K, Wang C, Leung CY (1990) Addisonian crisis as presenting feature in malignancies. Cancer 65:177–179

    CAS  PubMed  Google Scholar 

  6. Gondoh M, Sakamoto H, Fujii A, Masuda Y, Watanabe S, Takano Y, Yanagisawa A, Katoh Y (1987) A case of primary non-Hodgkin lymphoma of the left adrenal gland. Rinsho Hoshasen 32:155–158

    CAS  PubMed  Google Scholar 

  7. Huminer D, Garty M, Lapidot M, Leiba S, Borohov H, Rosenfeld JB (1988) Lymphoma presenting with adrenal insufficiency. Adrenal enlargement on computed tomographic scanning as a clue to diagnosis. Am J Med 84:169–172

    CAS  PubMed  Google Scholar 

  8. Ito Y, Yamaha M, Koide T, Sakai S, Seko A, Watanabe K (1988) A case of bilateral adrenal non-Hodgkin lymphoma. Hinyokika Kiyo 34:137–140

    CAS  PubMed  Google Scholar 

  9. Robertz-Vaupel GM, Glasmacher AG, Vogel J, Vaupel HA, Koster O, Pizzulli L (1990) Primary bilateral, adrenal, highly malignant B-cell lymphoma with lymphoblastic mengiosis. Klin Wochenschr 68:1076–1081

    CAS  PubMed  Google Scholar 

  10. Sasagawa I, Nishimori H, Futagawa S, Sadamori N, Jo T, Kawachi T, Hayashibara T, Tokunaga S, Itoyama T, Tsukasaki K (1991) Primary bilateral adrenal lymphomas in 3 cases. Rinsho Ketsueki 32:1492–1497

    CAS  PubMed  Google Scholar 

  11. Casolo P, Frassoldati A, Giaco M, Lazzaretti MG, Mosca D, Piccinini L (1991) A case of primary non-Hodgkin lymphoma of the adrenal gland. Minerva Med 82:781–785

    CAS  PubMed  Google Scholar 

  12. Dobnig H, Silly H, Ohlinger W, Neubauer M, Leb G, Krejs GJ (1992) Successful treatment of primary adrenal insufficiency due to malignant non-Hodgkin’s lymphoma. Clin Invest 70:938–941

    CAS  Google Scholar 

  13. Al-Fiar FZ, Pantalony D, Shepherd F (1997) Primary bilateral adrenal lymphoma. Leuk Lymphoma 27:543–549

    CAS  PubMed  Google Scholar 

  14. Wang J, Sun NCJ, Renslo R, Chuang CC, Tabbarah HJ, Barajas L, French SW (1998) Clinically silent primary adrenal lymphoma: a case report and review of the literature. Am J Hematol 58:130–136

    Article  CAS  PubMed  Google Scholar 

  15. Sasagawa I, Sadamori N, Itoyama T, Tsukasaki K, Nakamura H, Tomonaga M, Kishikawa M (1995) Primary adrenal lymphoma with chromosomal abnormalities. Acta Haematol 94:156

    CAS  PubMed  Google Scholar 

  16. Salvatore JR, Robin RS (1999) Primary bilateral adrenal lymphoma. Leuk Lymphoma 34:111–117

    CAS  PubMed  Google Scholar 

  17. Chu P, Costa J, Lachman MF (1996) Angiotropic large cell lymphoma presenting as primary adrenal insufficiency. Hum Pathol 27:209–211

    CAS  PubMed  Google Scholar 

  18. Hsu CW, Ho CL, Sheu WHH, Harn HJ, Chao TY (1999) Adrenal insufficiency caused by primary aggressive non-Hodgkin’s lymphoma of bilateral adrenal glands: report of a case and literature review. Ann Hematol 78:151–154

    Article  CAS  PubMed  Google Scholar 

  19. Lee KS, Chung YS, Park KH, Kim HS, Kim HM (1999) A case of primary bilateral adrenal lymphoma with partial adrenal insufficiency. Yonsei Med J 40:297–300

    CAS  PubMed  Google Scholar 

  20. Takai K, Hiragino T, Isoyama R, Takahashi M, Naito K (1999) A case of primary adrenal lymphoma diagnosed from percutaneous needle biopsy. Urol Int 62:31–33

    Article  CAS  PubMed  Google Scholar 

  21. Wu HC, Shih LY, Chen TC, Chu SH, Tsai CC (1999) A patient with bilateral primary adrenal lymphoma, presenting with fever of unknown origin and achieving long-term disease-free survival after resection and chemotherapy. Ann Hematol 78:289–292

    Article  CAS  PubMed  Google Scholar 

  22. Cavanna L, Civardi G, Vallisa D, Berte R (1999) Primary adrenal non-Hodgkin’s lymphoma associated with autoimmune hemolytic anemia: a case diagnosed by ultrasound-guided fine needle biopsy. Ann Ital Med Int 14:298–301

    CAS  PubMed  Google Scholar 

  23. Yamamoto E, Ozaki N, Nakagawa M, Kimoto M (1999) Primary bilateral adrenal lymphoma associated with idiopathic thrombocytopenic purpura. Leuk Lymphoma 35:403–408

    Google Scholar 

  24. Kuyama A, Takeushi M, Munemasa M, Tsutsui K, Aga N, Goda Y, Kanetada K (2000) Successful treatment of primary adrenal non-Hodgkin’s lymphoma associated with adrenal insufficiency. Leuk Lymphoma 38:203–205

    CAS  PubMed  Google Scholar 

  25. Ellis RD, Read D (2000) Bilateral adrenal non-Hodgkin’s lymphoma with adrenal insufficiency. Postgrad Med J 76:508–509

    Article  CAS  PubMed  Google Scholar 

  26. Frankel WL, Shapiro P, Weidner N (2000) Primary anaplastic large cell lymphoma of the adrenal gland. Ann Diagn Pathol 4:158–164

    CAS  PubMed  Google Scholar 

  27. Mermershtain W, Liel Y, Zirkin HJ, Lupu L, Lantsberg S, Cohen Y (2001) Primary bilateral adrenal lymphoma relapsing as a solid cerebral mass after complete clinical remission: a case report. Am J Clin Oncol 24:583–585

    Article  CAS  PubMed  Google Scholar 

  28. Hahn JS, Choi HS, Suh CO, Lee WJ (2002) A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement. Yonsei Med J 43:385–390

    PubMed  Google Scholar 

  29. Miyamura T, Obama K, Takahira H, Uike N, Kozuru M (1993) A case of primary non-Hodgkin’s lymphoma of the adrenal gland presenting with Addison’s disease. Rinsho Ketsueki 34:882–884

    CAS  PubMed  Google Scholar 

  30. Levaltier X, Troussard X, Fournier L,Reznik Y, Reman O, Mahoudeau J, Leporrier M (1994) Primary adrenal lymphoma. Report of a case. Presse Med 23:372–374

    CAS  PubMed  Google Scholar 

  31. Mizusawa H, Okaneya T, Yoneyama T, Taguchi I (1995) Primary malignant lymphoma of the adrenal gland: a case report. Hinyokika Kiyo 41:991–994

    CAS  PubMed  Google Scholar 

  32. Efremidis SC, Harsoulis F, Douma S, Zafiriadou E, Zamboulis C, Kouri A (1996) Adrenal insufficiency with enlarged adrenals. Abdom Imaging 21:168–171

    Article  CAS  PubMed  Google Scholar 

  33. Maugendre D, Derrien C, Grulois I, Simon JP, Guilhem I, Poirier JY, Le Prise PY, Allannic H (1998) Primary adrenal lymphoma with latent adrenal insufficiency: a case report and literature review. Ann Endocrinol 59:34–39

    CAS  Google Scholar 

  34. Tazi K, Achour A, Koutani A, Ibn Attya A, Hachimi M, Lakrissa A (1999) Primary non-Hodgkin’s malignant lymphoma of the adrenal gland. Case report and review of the literature. Prog Urol 9:1102–1105

    CAS  PubMed  Google Scholar 

  35. Uehara E, Tasaka T, Matsuhashi Y, Suemitsu I, Tamura T, Kuwajima M, Nagai M (2001) Adrenal gland lymphoma accompanied by renal cell carcinoma. Rinsho Ketsueki 42:710–712

    CAS  PubMed  Google Scholar 

  36. Toubai T, Akama H, Takagawa M, Ishida S, Kasai M, Tanaka J, Imamura M (2002) Primary adrenal lymphoma: a case report and literature review in Japan. Rinsho Ketsueki 43:851–856

    PubMed  Google Scholar 

  37. Fujikata S, Tanji N, Aoki K, Ohoka H, Hojo N, Yokoyama M (2002) Extramedullary plasmacytoma arising from an adrenal gland. Urology 60:514

    Article  Google Scholar 

  38. Schocket LS, Syed NA, Fine SL (2002) Primary adrenal lymphoma with choroidal metastases. Am J Opthalmol 134:775–776

    Article  Google Scholar 

  39. Alama Zaragoza MA, Robles Iniesta A, Roca Adelantado I, Sales Maicas MA, Navar de Leon MC, Roman Sanchez P (2002) Bilateral primary adrenal lymphoma: an unusual presentation. An Med Interna 19:524–526

    PubMed  Google Scholar 

  40. Bakkali H, el Omari-Alaoui H, Elghazi el A, Errihani H, Benjaafar N, Elgueddari BK (2002) Bilateral primary adrenal lymphoma. Prog Urol 12:1279–1283

    PubMed  Google Scholar 

  41. Lu JY, Chang CC, Chang YL (2002) Adrenal lymphoma and Addison’s disease: report of a case. J Formos Med Assoc 101:854–858

    PubMed  Google Scholar 

  42. Wang FF, Su CC, Chang YH, Pan CC, Tang KT, Jap TS, Lin HD, Won JG (2003) Primary adrenal lymphoma manifesting as adrenal incidentaloma. J Chin Med Assoc 66:67–71

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Hematology Department, Metropolitan Hospital, Ethn. Makariou-9 & El. Venizelou-1, Neo-Faliro, 18547, Piraeus, Greece

    George Mantzios, Panagiotis Tsirigotis, Filio Veliou, Sotirios Papageorgiou & Yannis Robos

  2. Internal Medicine Department, Metropolitan Hospital, Piraeus, Greece

    Iosif Boutsikakis

  3. Histopathology Department, Metropolitan Hospital, Piraeus, Greece

    Lillian Petraki

  4. Radiology Department, Metropolitan Hospital, Piraeus, Greece

    John Kolovos

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  1. George Mantzios
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  2. Panagiotis Tsirigotis
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Correspondence to Panagiotis Tsirigotis.

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Mantzios, G., Tsirigotis, P., Veliou, F. et al. Primary adrenal lymphoma presenting as Addison’s disease: case report and review of the literature. Ann Hematol 83, 460–463 (2004). https://doi.org/10.1007/s00277-003-0838-3

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  • DOI: https://doi.org/10.1007/s00277-003-0838-3

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