An unfortunate case of Cushing’s syndrome.
M. Moriarty, A. Qureshi
Dept. of Diabetes and Endocrinology, Northwick Park Hospital
A 24 year old female was referred by her GP with a two month history of hair growth on her face and back, thin limbs and facial swelling. On examination she had a cushingoid appearance with moon face, posterior fat pad, thin limbs and easy bruising.
Her family history was remarkable for an aunt who had
Cushing's syndrome due to an adrenal adenoma and had curative adrenalectomy.
We proceeded to perform a low dose dexamethasone suppression test. The result was consistent with Cushing's syndrome, serum cortisol did not suppress and ACTH was undetectable. MRI adrenals showed a heterogeneous enhancing lesion in the right adrenal gland which was 10x9x9cm.
She was commenced on metyrapone to which she had good
symptomatic and biochemical response, while awaiting surgical excision.
Following open right adrenalectomy histology confirmed the presence of a 7cm
adrenal cortical carcinoma. She commenced adjuvant treatment with
mitotane for one year.
Unfortunately after 12 months she developed recurrent disease and had an excision of tumour from the superior pole of right kidney and the right hemidiaphragm. While she had adjuvant chemotherapy regrettably her condition deteriorated. She was unable to undergo radiotherapy and repeat imaging showed progressive metastatic disease despite treatment.
Adrenal carcinoma is a rare malignancy; incidence is approximately 1 to 2 cases per million population per year. The majority of cases present with symptoms of Cushing’s disease. Prognosis for most patients is regrettably poor. Though uncommon adrenal carcinoma should be considered particularly in adenoma larger than 4cm or increasing in size.