This case report describes a rare case of primary adrenal lymphoma presenting as Addison's disease in a 56-year-old male patient. Imaging and biopsy revealed bilateral adrenal involvement by mantle cell lymphoma. The patient was treated with R-CHOP chemotherapy and achieved complete remission, with restoration of adrenal function. Primary adrenal lymphoma is an uncommon and aggressive malignancy, but outcomes have improved with rituximab-containing regimens like R-CHOP. This case highlights an unusual presentation of primary adrenal lymphoma as Addison's disease from mantle cell histology.

1. Primary adrenal lymphoma presenting as
Addison's disease
Shruthi KP*, Vinayak M*, Nair IR**, Pavithran K*
* Department of Medical Oncology and hematology, ** Dept of Pathology,
Amrita Institute of medical Sciences, Amrita Viswavidhyapeetham, Kochi,
India-682041

2. INTRODUCTION:
 Malignant lymphoma primarily arising in the adrenal glands,
i.e., primary adrenal lymphoma (PAL), is an extremely rare
disease and accounts for less than 1% of all non-Hodgkin’s
lymphoma.
 As of 2013, less than 200 cases of PAL have been reported and
there are few case series and literature reviews.
 PAL occurs predominantly in elderly males, diffuse large B-cell
lymphoma (DLBCL) as the most common histology, frequent
bilateral adrenal involvement with adrenal insufficiency and a
tendency for patients to develop central nervous system
involvement. We report a case of primary adrenal lymphoma
of mantle cell type presenting as Addison’s disease. PAL of
mantle cell lymphoma of the adrenal has not been reported
previously.

3. CASE REPORT:
 A 56-year-old male patient, who was a known case of diabetes
mellitus, hypertension and chronic liver disease, presented with
complaints of generalized weakness and easy fatigability since one
month. On further enquiry, a history of progressive skin discoloration of
the face over 3 months was also elicited.
 Clinical examination: revealed stable vital parameters, bilateral
pitting edema and hyperpigmentation over the face, and trunk.
Systemic examination was non-contributory.
 Investigations: revealed normal renal and liver functions. Lactate
dehydrogenase (LDH) was 348 u/ l(normal140-280u/l). X-ray chest was
within normal limits.

4.  8am serum cortisol level was - 0.9μg/dl (normal7-28micrograms/dl).
 Contrast-enhanced computed tomography (CT) scan of the
abdomen revealed that the left adrenal gland was replaced by a
lobulated mass with soft tissue attenuation and the multiple nodules
in the right adrenal gland which was suggestive of malignancy. CT
scan of the chest was normal.
 Subsequent percutaneous CT-guided biopsy of the adrenal mass
reported as round cell neoplasm. Immunohistochemistry
demonstrated positivity for LCA, CD20, BCL2, CD5, and Cyclin D1
with a Ki 67 index of 90%.
 PET-CT scan showed involvement of both adrenals and a regional
nodal involvement.

5. PET/CT scan before
R-CHOP
PET/CT scan after 3
cycles of R-CHOP

6.  Bone marrow study was normal.
 These findings of radiological and pathological assessments
supported the diagnosis of primary adrenal lymphoma of the
mantle cell type.
 He was started on combination chemotherapy with Rituximab with
Cyclophosphamide, Adriamycin, Vincristine and Prednisolone(R-
CHOP). After 3rd cycle, reassessment showed complete metabolic
response by PET-CT scan.
 Autologous stem cell transplant was not offered in view of the
comorbidities. He completed 6 cycles of R-CHOP and is doing well
and in remission during the last 18 months of follow up. His adrenal
function also returned to normal and is off steroid replacement.

7. DISCUSSION:
 PAL is a rare type of extranodal lymphoma
 Unlike primary lymphomas arising in some extranodal sites like bone and breast,
PAL, usually bilateral, has been reported to have a male preponderance with a
median age of presentation being 65 years. The commonest type of PAL is
reported to be the DLBCL phenotype in about 70% cases.
 However our patient was younger (65years), presented with skin pigmentation
suggestive of adrenal insufficiency and the pathology was mantle cell type.
 Presentations of PAL are variable and depend upon tumor size, rapidity of
enlargement, and presence of adrenal insufficiency. Adrenocortical
insufficiency, observed in 50% of patients diagnosed with PAL, does not correlate
with size of the primary tumor.

8.  Bilateral adrenal masses can raise a suspicion for secondaries from
malignant tumors of the lung, breast, and colon, and infections,
primarily tuberculosis.
 Therapeutic modalities for adrenal lymphomas include
corticosteroid replacement, combination chemotherapy, surgery,
and surgery followed by chemotherapy and/radiotherapy
 Prognosis is generally poor.
 Only about 50% patients with PAL are reported to respond to
treatment with a median survival of 4 months in earlier reports.

9. Conclusion:
 The introduction of R-CHOP regimen have resulted in improved
survival.
 In patients achieving complete remission, significant prolongations of
overall survival and progression free survival are reported.
 PAL should be considered as a potential diagnosis in patients with
primary adrenal insufficiency who have elevated serum lactate
dehydrogenase and CT findings of an adrenal mass of variable
density with the adrenal glands retaining the adreniform shape. Use of
R-CHOP regimen results in improved survival.

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