Primary bilateral adrenal non-Hodgkin's lymphoma is an extremely rare entity. Only 44 cases have been reported in the literature. The most common presenting symptoms are abdominal pain, fever, asthenia, constipation, weight loss or typical symptoms of adrenal insufficiency, hypertension, darkening of skin, orthostatic hypotension or an addisonian crisis.
Methods
The case is presented of a 57-year-old man suffering from primary bilateral adrenal lymphoma with symptoms of adrenal insufficiency syndrome associated with bilateral, stabbing lumbar pain and a palpable mass on the left side. Laboratory tests revealed a considerable increase in lactate dehydrogenase levels, adrenal insufficiency, and high corticotropin levels.
Results
Abdominal CT scan showed two large adrenal masses. A CT-guided fine needle aspiration biopsy revealed a large B-cell non-Hodgkin's lymphoma. Combination chemotherapy according to the CHOP protocol with cyclophosphamide, doxorubicin, vincristine and prednisolone was initiated, which caused a slight reduction in size of the two adrenal masses. The patient underwent a bilateral adrenalectomy with almost complete excision of the tumors. Replacement therapy with cortisone acetate was initiated postoperatively. Adjuvant combination chemotherapy according to the CHOP protocol was started but renal failure gradually emerged and the treatment could not be completed. The patient died seven months after surgery due to acute bronchial pneumonia and progression of disease.
Conclusions
Primary bilateral adrenal non-Hodgkin's lymphoma mainly affects adult men. Diagnosis is based on histological examination. Whether associated or not with radiotherapy, chemotherapy is the most recommended treatment. Surgery, where possible, seems to lead to an increase in survival rates, but it is not possible to draw any definite conclusions on its effectiveness as yet.
HsuC.W., HoC.L., SheuW.H., HarnH.J., ChaoT.Y.: 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, 1999.
WuH.C., ShihL.Y., ChenT.C., ChuS.H., TsaiC.C.: 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, 1999.
18.
EllisR.D., ReadD.: Bilateral adrenal non-Hodgkin's lymphoma with adrenal insufficiency. Postgrad Med J, 76: 508–509, 2000.
19.
MermershtainW., LielY., ZirkinH.J., LupuL., LantsbergS., CohenY.: Primary bilateral adrenal lymphoma relapsing as a solid cerebral mass after complete clinical remission: a case report. Am J Clin Oncol, 24: 583–585, 2001.
20.
ViswanathanV., MiddletonM.L.: Primary adrenal lymphoma: a case report. Clin Nucl Med, 26: 787–788, 2001.
21.
HahnJ.S., ChoiH.S., SuhC.O., LeeW.J.: A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement. Yonsei Med J, 43: 385–390, 2002.
22.
SchocketL.S., SyedN.A., FineS.L.: Primary adrenal lymphoma with choroidal metastases. Am J Ophthalmol, 134: 775–776, 2002.
23.
LuJ.Y., ChangC.C., ChangY.L.: Adrenal lymphoma and Addison's disease: report of a case. J Formos Med Assoc, 101: 854–858, 2002.
24.
BrinkI., SchneiderB., HoegerleS.: Enormous bilateral adrenal uptake of F-18 FDG caused by non-Hodgkin's lymphoma. Clin Nucl Med, 27: 739–740, 2002.
25.
TuminoS., LeottaM.L., BranciforteG., ManteroF., CalogeroA.E.: Bilateral adrenal non-Hodgkin lymphoma type B. J Endocrinol Invest, 26: 1120–1123, 2003.
GillettM., HaakS.: Not just another fall in the elderly. Bilateral adrenal lymphoma presenting with adrenal insufficiency causing weakness. Aust Fam Physician, 32: 248–250, 2003.
29.
BenchekrounA., QarroA., KasmaouiH., IkenA., MarzoukM., FaikM.: Le lymphome malin non hodgkinien bilatéral et primitif de la surrénale (à propos d'une observation avec revue de la littérature). Ann Chir, 128: 557–560, 2003.
30.
ZarT., KhanF., PetitW.Jr., BerneneJ.R.: Primary adrenal lymphoma presenting as adrenal insufficiency. A case report and review of literature. Conn Med, 68: 7–10, 2004.
31.
MantziosG., TsirigotisP., VeliouF., BoutsikakisI., PetrakiL., Kolovos PapageorgiouS., RobosY.: Primary adrenal lymphoma presenting as Addison's disease: case report and review of the literature. Ann Hematol, 83: 460–463, 2004.
32.
Hamid ZargarA., Ahmad LawayB., Alam BhatK., ShahA., AhmadM., Aejaz AzizS., Iftikhar BashirM., Iqbal WaniA., Hayat BhatM.: Adrenal insufficiency due to primary adrenal non-Hodgkin's lymphoma. Exp Clin Endocrinol Diabetes, 112: 462–464, 2004.
33.
KumarR., XiuY., MaviA., El-HaddadG., ZhuangH., AlaviA.: FDG-PET imaging in primary bilateral adrenal lymphoma: a case report and review of the literature. Clin Nucl Med, 30: 222–230, 2005.
34.
Diamanti-KandarakisE., ChatzismalisP., EconoumuF., LazaridesS., AndroulakiA., KouraklisG.: Primary adrenal lymphoma presented with adrenal insufficiency. Case report. Hormones (Athens), 3: 68–73, 2007.
35.
ChanJ.K.: Anaplastic large cell lymphoma: Redefining its morphologic spectrum and importance of recognition of the ALK positive subset. Adv Anat Pathol, 5: 281–313, 1999.
36.
ClavioM., RossiE., TruiniM., CarraraP., RavettiJ.L., SprianoM., VimercatiA.R., SantiniG., CanepaL., PierriI., CelestiL., MiglinoM., CastellanetaA., DamascoE., GobbiM.: Anaplastic large cell lymphoma: a clinicopathologic study of 53 patients. Leuk Lymphoma, 22: 319–327, 1996.
KimS.H., BrennanM.F., RussoP., BurtM.E., CoitD.G.: The role of surgery in the treatment of clinically isolated adrenal metastasis. Cancer, 82: 389–394, 1998.
39.
DahamiZ., DebbaghA., DakirM., HafianiM., JoualA., BennaniS., El MriniM., BenjellounS.: Lymphome primitif de la surrénale de phénotype B, diagnostiqué à la ponction-biopsie percutanée. Ann Urol (Paris), 35: 22–25, 2001.
40.
HaabF., DuclosJ.M., JulienJ., PlouinP.F.: Tumeur des deux surrénales: 12 cas consécutifs. Presse Med, 23: 511–514, 1994.
SinghD., KumarL., SharmaA., VijayaraghavanM., ThulkarS., TandonN.: Adrenal involvement in non-Hodgkin's lymphoma: four cases and review of literature. Leuk Lymphoma, 45: 789–794, 2004.
43.
MaugendreD., DerrienC., GruloisI., SimonG.P., GuilhemI., PoirierJ.Y., Le PriseP.Y., AllanicH.: Primary adrenal insufficiency. A case report and literature review. Ann Endocrinol, 59: 34–39, 1998.
44.
Wangj, SunN.C., RensloR., ChuangC.C., TabbarahH.J., BarajasL., FrenchS.W.: Clinically silent primary adrenal lymphoma: a case report and review of the literature. Am J Hematol, 58: 130–136, 1998.
