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

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ClinicalRadiology (1990) 42, 63-64 Case Report: Non-Hodgkin's Lymphoma Confined to Adrenal Glands Presenting with Addison's Disease S. KHAN, N. RABY and M. MICHELL Department of Radiology, King's College Hospital, London the 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 REPORT 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 15.3mmol. 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 10cm x 7 cmx 15.8cm. The right adrenal measured 10.2cm 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 lymphoma is relatively common but even when present there are usually no signs or symptoms of hypoadrenalism. In one report, nearly 25% of patients with lymphoma had adrenal involvement at autopsy (Rosenberg et al., 1961). However, several retrospective studies have shown an incidence of only 1- 4% of adrenal masses demonstrated on CT at the time of presentation in patients with Non-Hodgkin's lymphoma (Glaser et al., 1983; Paling and Williamson, 1983). In one series, bilateral involvement was shown in two out of seven cases of adrenal lymphoma shown on CT scanning (Jafri et al., 1983). Hypoadrenalism due to lymphoma is unusual. Reported cases of bilateral adrenal masses with hypoad- renalism include granulomatous infiltration, chronic infection (Gibbet al., 1985), acute bilateral haemorrhage, metastatic infiltration and rarely lymphoma (Wheatley et al., 1985; Doppman et al., 1982). Several cases of 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 of lymphoma of the adrenals was only made at post-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 Trucut biopsy. The CT appearances of adrenal enlargement due to lymphoma are non-specific and may be mimicked by several other conditions (Moss et al., 1983). Secondary deposits from carcinoma of the lung, breast, thyroid, colon and melanoma are usually solid although there may be cystic components as well. Adenomas are bilateral in 10% of cases and cortical adenomas may have low attenuation values (less than 30 HU)due to their high lipid content. Primary adrenal neoplasms are even rarer but may have similar appearances to adenomas. Ten per cent of phaeochromocytomas are bilateral and may have cystic as well as solid components. Neuroblastomas may arise from the adrenal medulla but are usually diagnosed in children. Other primary tumours such as haemangio- mas, fibromas and lymphangiomas are extremely rare and usually incidental findings. In conclusion, in a patient with unilateral or bilateral adrenal enlargement even in the absence of lymphadeno- pathy the possibility of lymphoma should be considered. The patient may very rarely present with Addison's disease and the nature of the adrenal mass can be confirmed by CT guided needle biopsy. REFERENCES Doppman, JL, Gill, JR, Nienhuis, AW, Earll, JM & Long, JA, (1982). CT findings in Addison's disease. Journal of Computer Assisted Tomography, 6, 7594761.
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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

the

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.

REFERENCES Doppman, JL, Gill, JR, Nienhuis, AW, Earll, JM & Long, JA, (1982).

CT findings in Addison's disease. Journal of Computer Assisted Tomography, 6, 7594761.

64 CLINICAL RADIOLOGY

Foster, SC & Gauvin, GA (1983). A man with a large supra-renal mass. Urological Radiology, 5, 58-61.

Gibb, WRG, Ramsay, AD, McNeil, NI & Wrong, OM (1985) Bilateral adrenal masses. British Medical Journal, 291, 203 204.

Glaser, HS, Lee, JKT, Balge, DM, Mauro, MA, Griffith, R & Sagel, SS (1983). Non-Hodgkin's lymphoma; computed tomographic demon- stration of unusual extranodal involvement. Radiology, 149, 211-217.

Jafri, SZH, Francis, IR, Glaser, GM, Bree, RL & Amendloa, MA, (1983). CT detection of adrenal lymphoma. Journal of Computer Assisted Tomography, 7, 254-256.

Moss, AA, Gamsu, G and Genant, HK (1983). Computed Tomography of the Body, pp. 837-876. Saunders, London.

Paling, M & Williamson, BRJ (1983). Adrenal involvement in Non- Hodgkin's lymphoma. American Journal of Roentgenology, 141, 303-305.

Rosenberg, SA, Diamond, HD, Jaslowitz, B & Craver, LR (1961). Lymphoma, a review of 11,269 cases. Medicine (Baltimore), 40, 31-84.

Shea, TC & Spark, R (1985). Non-Hodgkin's lymphoma limited to the adrenal glands with adrenal insuffÉciency. American Journal of Medicine, 78, 7! 1-714.

Sparagana, M (1970). Addison's disease due to reticulum cell sarcoma apparently confined to the adrenals. Journal of the American Geriatrics Society, 18, 550-554.

Vicks, BS, Perusek, M, Johnson, J & Tio, F (1987). Primary adrenal lymphoma: CT and sonographic appearances. Journal of Clinical Ultrasound, 15, 1355 1359.

Wheatley, T, Gallagher, S & Dixon, AK (1985). Adrenal insufficiency and bilateral adrenal enlargement. Demonstration by computed tomography. Postgraduate Medical Journal, 61,435 438.


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