JRSM >
Volume 96, Number 10 >
Pp. 503-504
Southend Hospital, Prittlewell Chase, Westcliff-on-Sea, Essex SS0 0RY, UK
Correspondence to: Dr J F Blaikley, Flat 7, Capitol Court, 124 School Lane, Manchester M20 6LB, UK E-mail: jblaikley{at}yahoo.co.uk
Lymphomas commonly metastasize to the adrenal glands,1 but they seldom cause adrenal insufficiency.2
CASE HISTORY
A woman of 74 was admitted for investigation of nausea, dizziness exacerbated by head movements, anorexia and progressive weight loss. Eight months earlier, cutaneous T cell lymphoma (CTCL) had been diagnosed, without extracutaneous spread as judged by CT scans of chest and abdomen. After radiotherapy, the disease had remitted. She now had postural hypotension (blood pressure 139/73 mmHg lying, 120/88 mmHg standing) and cutaneous nodules were noted over both upper limbs consistent with recurrence of CTCL. There was slight hyponatraemia (Na+ 134 mmol/L) but potassium was normal (4.3 mmol/L). All other electrolytes, liver function tests, and the full blood count were normal. However, as her symptoms rapidly got worse, the serum sodium fell to 119 mmol/L and the serum potassium rose to 5.0 mmol/L. The 9 am cortisol was low at 121 nmol/L and a short Synacthen (tetracosactide) test showed adrenal insufficiency (cortisol 117 nmol/L 30 minutes after 250mg tetracosactide). Hydrocortisone 50 mg three times daily was prescribed and over the next five days the patient made a remarkable recovery, with the sodium rising to 136 mmol/L and the potassium falling to 4.1 mmol/L. An MRI scan of the brain was normal but a CT scan of the abdomen showed bilateral suprarenal masses (Figure 1). Eight days later the patient suddenly lost consciousness. An addisonian crisis was suspected but there was no response to intravenous hydrocortisone. A CT scan of the brain then revealed a frontal haemorrhage, from which she died five days later. At necropsy the tumour was seen to have infiltrated both kidneys and both adrenal glands. Deposits were also seen in the liver and in one paraaortic lymph node. The pituitary gland was macroscopically normal. On histological examination the cutaneous plaques were positive for T cell markers (CD3; CD43) and negative for B cell markers (CD20; CD79a). Staining for CD30 and ALK protein was also negative. The histology of the tumour infiltrating the adrenal glands matched that of the cutaneous lymphoma.
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COMMENT
For any tumour to cause adrenal insufficiency it has to destroy over 90% of the glandular tissue in both adrenal glands.3 This is a rare event with lymphomas: at the Mayo Clinic only 5 such cases were seen in 18 years.2 However, lymphomas do commonly metastasize to the adrenals.3 The lymphoma in this case was classified as a CD30-ve CTCL (EORTC4) or peripheral T cell lymphoma, type unspecified (WHO5). Although one subtype of CTCL, mycosis fungoides, has been associated with adrenal insufficiency we have found no previous cases with CD30-ve CTCL. None of the histological sections taken at necropsy showed the tumour invading the vessel walls, making an alternative diagnosis of lymphomatous vasculitis unlikely. Systemic anaplastic large-cell carcinoma, one of the main differential diagnoses, was ruled out since the lymphoma was ALK negative and negative for the t(2:5) translocation in the TCR receptor, and no tumour cells were found in the vascular endothelium.
The importance of CD30 for prognosis has only lately been realized.6 It is a type 1 membrane glycoprotein and belongs to the tumour necrosis factor receptor family. CD30+ve CTCL is indolent whereas CD30-ve CTCL is aggressive. This is reflected in their 5-year survival rates of 90% and 15%, respectively.
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