Annals of Hematology

, Volume 83, Issue 7, pp 460–463 | Cite as

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

  • George Mantzios
  • Panagiotis Tsirigotis
  • Filio Veliou
  • Iosif Boutsikakis
  • Lillian Petraki
  • John Kolovos
  • Sotirios Papageorgiou
  • Yannis Robos
Case Report

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.

Keywords

Adrenal lymphoma Addison’s disease Extranodal lymphoma 

References

  1. 1.
    Isaacson PG, Norton AJ (1994) Extranodal lymphomas. Churchill Livingstone, EdinburghGoogle Scholar
  2. 2.
    Lack EE, Gruhn JG (1996) Adrenal glands. In: Damianov I, Linder J (eds) Andersons Pathology, 10th edn. Mosby, St. LouisGoogle Scholar
  3. 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 182Google Scholar
  4. 4.
    Falchook S, Allard JC (1991) CT of primary adrenal lymphoma. J Comput Assist Tomogr 15:1048PubMedGoogle Scholar
  5. 5.
    Kung AWC, Pun KK, Lam K, Wang C, Leung CY (1990) Addisonian crisis as presenting feature in malignancies. Cancer 65:177–179PubMedGoogle Scholar
  6. 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 32:155–158PubMedGoogle Scholar
  7. 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 84:169–172PubMedGoogle Scholar
  8. 8.
    Ito Y, Yamaha M, Koide T, Sakai S, Seko A, Watanabe K (1988) A case of bilateral adrenal non-Hodgkin lymphoma. Hinyokika Kiyo 34:137–140PubMedGoogle Scholar
  9. 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 68:1076–1081PubMedGoogle Scholar
  10. 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 32:1492–1497PubMedGoogle Scholar
  11. 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 82:781–785PubMedGoogle Scholar
  12. 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 70:938–941Google Scholar
  13. 13.
    Al-Fiar FZ, Pantalony D, Shepherd F (1997) Primary bilateral adrenal lymphoma. Leuk Lymphoma 27:543–549PubMedGoogle Scholar
  14. 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 58:130–136CrossRefPubMedGoogle Scholar
  15. 15.
    Sasagawa I, Sadamori N, Itoyama T, Tsukasaki K, Nakamura H, Tomonaga M, Kishikawa M (1995) Primary adrenal lymphoma with chromosomal abnormalities. Acta Haematol 94:156PubMedGoogle Scholar
  16. 16.
    Salvatore JR, Robin RS (1999) Primary bilateral adrenal lymphoma. Leuk Lymphoma 34:111–117PubMedGoogle Scholar
  17. 17.
    Chu P, Costa J, Lachman MF (1996) Angiotropic large cell lymphoma presenting as primary adrenal insufficiency. Hum Pathol 27:209–211PubMedGoogle Scholar
  18. 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 78:151–154CrossRefPubMedGoogle Scholar
  19. 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 40:297–300PubMedGoogle Scholar
  20. 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 62:31–33CrossRefPubMedGoogle Scholar
  21. 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 78:289–292CrossRefPubMedGoogle Scholar
  22. 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 14:298–301PubMedGoogle Scholar
  23. 23.
    Yamamoto E, Ozaki N, Nakagawa M, Kimoto M (1999) Primary bilateral adrenal lymphoma associated with idiopathic thrombocytopenic purpura. Leuk Lymphoma 35:403–408Google Scholar
  24. 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 38:203–205PubMedGoogle Scholar
  25. 25.
    Ellis RD, Read D (2000) Bilateral adrenal non-Hodgkin’s lymphoma with adrenal insufficiency. Postgrad Med J 76:508–509CrossRefPubMedGoogle Scholar
  26. 26.
    Frankel WL, Shapiro P, Weidner N (2000) Primary anaplastic large cell lymphoma of the adrenal gland. Ann Diagn Pathol 4:158–164PubMedGoogle Scholar
  27. 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 24:583–585CrossRefPubMedGoogle Scholar
  28. 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 43:385–390PubMedGoogle Scholar
  29. 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 34:882–884PubMedGoogle Scholar
  30. 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 23:372–374PubMedGoogle Scholar
  31. 31.
    Mizusawa H, Okaneya T, Yoneyama T, Taguchi I (1995) Primary malignant lymphoma of the adrenal gland: a case report. Hinyokika Kiyo 41:991–994PubMedGoogle Scholar
  32. 32.
    Efremidis SC, Harsoulis F, Douma S, Zafiriadou E, Zamboulis C, Kouri A (1996) Adrenal insufficiency with enlarged adrenals. Abdom Imaging 21:168–171CrossRefPubMedGoogle Scholar
  33. 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 59:34–39Google Scholar
  34. 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 9:1102–1105PubMedGoogle Scholar
  35. 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 42:710–712PubMedGoogle Scholar
  36. 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 43:851–856PubMedGoogle Scholar
  37. 37.
    Fujikata S, Tanji N, Aoki K, Ohoka H, Hojo N, Yokoyama M (2002) Extramedullary plasmacytoma arising from an adrenal gland. Urology 60:514CrossRefGoogle Scholar
  38. 38.
    Schocket LS, Syed NA, Fine SL (2002) Primary adrenal lymphoma with choroidal metastases. Am J Opthalmol 134:775–776CrossRefGoogle Scholar
  39. 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 19:524–526PubMedGoogle Scholar
  40. 40.
    Bakkali H, el Omari-Alaoui H, Elghazi el A, Errihani H, Benjaafar N, Elgueddari BK (2002) Bilateral primary adrenal lymphoma. Prog Urol 12:1279–1283PubMedGoogle Scholar
  41. 41.
    Lu JY, Chang CC, Chang YL (2002) Adrenal lymphoma and Addison’s disease: report of a case. J Formos Med Assoc 101:854–858PubMedGoogle Scholar
  42. 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 66:67–71PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • George Mantzios
    • 1
  • Panagiotis Tsirigotis
    • 1
  • Filio Veliou
    • 1
  • Iosif Boutsikakis
    • 2
  • Lillian Petraki
    • 3
  • John Kolovos
    • 4
  • Sotirios Papageorgiou
    • 1
  • Yannis Robos
    • 1
  1. 1.Hematology DepartmentMetropolitan HospitalPiraeusGreece
  2. 2.Internal Medicine DepartmentMetropolitan HospitalPiraeusGreece
  3. 3.Histopathology DepartmentMetropolitan HospitalPiraeusGreece
  4. 4.Radiology DepartmentMetropolitan HospitalPiraeusGreece

Personalised recommendations