Case report: Non-Hodgkin's lymphoma confined to the adrenal glands presenting with Addison's disease

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Clinical Radiology (1990) 42, 63-64

Case Report: Non-Hodgkin's Lymphoma Confined to Adrenal Glands Presenting with Addison's Disease S. K H A N , N. R A B Y and M. M I C H E L L

Department o f Radiology, King's College Hospital, London

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We report an unusual case of Addison's disease which is unique in being diagnosed ante-mortem as being due to Non-Hodgkin's lymphoma of the adrenal glands. Both ante- and postmortem examinations showed the lymphoma to be entirely confined to the adrenals.

CASE R E P O R T

A 59-year-old male presented with a 6 month history of nausea, vomiting and lethargy with night sweats and weight loss. On examin- ation, he had increased palmar crease pigmentation and palpable, bilateral upper abdominal masses. The blood pressure was 110/70 mmHg; haemoglobin 10.4 g/1 (normochromic, normocytic) and a urea of 15.3 mmol. A short synacthen test confirmed the clinical diagnosis of primary hypoadrenalism.

Abdominal ultrasound (US) and computed tomography (CT) scans demonstrated bilateral adrenal masses, hepatosplenomegaly but no evidence ofintra-abdominal or mediastinal lymphadenopathy (Fig. 1). On CT scanning, the left adrenal mass measured 10 cm x 7 cmx 15.8 cm. The right adrenal measured 10.2 cm x 6.4 cm x 9.6 cm. The masses were of homogeneous attenuation with no cystic component. The mean Hounsfield Unit number was 38 pre-contrast and 65 post-contrast (100 ml of intravenous iohexo1350 mg iodine/ml). Trucut biopsy of the left adrenal mass was performed under CT guidance. Histology of this tissue showed a high grade B cell centroblastic Non-Hodgkin's lymphoma. Bone marrow aspiration and trephine did not show marrow involve- ment. The patient was rehydrated and started on replacement mineralo- corticosteroids and chemotherapy but died 10 days later of drug- induced aplasia with invasive aspergillosis. At post-mortem, there was no evidence of lymphoma within the liver, spleen, kidneys or sampled abdominal lymph nodes.

DISCUSSION

Adrenal involvement with l ymphoma is relatively common but even when present there are usually no signs or symptoms o f hypoadrenal ism. In one report , nearly 25% of patients with l ymphoma had adrenal involvement at autopsy (Rosenberg et al., 1961). However, several retrospective studies have shown an incidence o f only 1- 4% of adrenal masses demonstra ted on CT at the time o f presentation in patients with N o n - H o d g k i n ' s l y m p h o m a (Glaser et al., 1983; Paling and Williamson, 1983). In one series, bilateral involvement was shown in two out o f seven cases o f adrenal l ymphoma shown on CT scanning (Jafri et al., 1983).

Hypoadrenal ism due to l y m p h o m a is unusual. Reported cases o f bilateral adrenal masses with hypoad- renalism include g r anu loma tous infiltration, chronic infection (G ibbe t al., 1985), acute bilateral haemorrhage, metastatic infiltration and rarely l y m p h o m a (Wheatley et al., 1985; D o p p m a n et al., 1982). Several cases o f lymphoma confined to the adrenal glands have also been

Correspondence to: Dr S. Khan, Department of Radiology, King's College Hospital, London SE5 9RS.

Fig. 1 - CT scan showing bilateral adrenal masses of soft tissue density.

reported (Foster and Gauvin, 1983; Shea and Spark, 1985; Vicks et al., 1987). Only in one previously reported case has the patient presented with the Addison ' s disease (Sparagana, 1970) and the diagnosis o f l ymphoma o f the adrenals was only made at pos t -mortem. In our case the patient presented with Addison ' s disease and hypo- adrenalism was confirmed biochemicalty. CT and US confirmed adrenal masses and the histological diagnosis was made using CT guided Trucu t biopsy.

The CT appearances o f adrenal enlargement due to l ymphoma are non-specific and ma y be mimicked by several other conditions (Moss et al., 1983). Secondary deposits f rom carc inoma o f the lung, breast, thyroid, colon and melanoma are usually solid a l though there may be cystic components as well. Adenomas are bilateral in 10% of cases and cortical adenomas may have low at tenuat ion values (less than 30 H U ) d u e to their high lipid content. Pr imary adrenal neoplasms are even rarer but may have similar appearances to adenomas. Ten per cent o f phaeochromocy tomas are bilateral and may have cystic as well as solid components . Neuroblas tomas may arise f rom the adrenal medulla but are usually diagnosed in children. Other pr imary tumours such as haemangio- mas, fibromas and lymphangiomas are extremely rare and usually incidental findings.

In conclusion, in a pat ient with unilateral or bilateral adrenal enlargement even in the absence o f lymphadeno- pa thy the possibility o f l y m p h o m a should be considered. The patient may very rarely present with Addison ' s disease and the nature o f the adrenal mass can be confirmed by CT guided needle biopsy.

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64 CLINICAL RADIOLOGY

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