R003

An unusual presentation of adrenocortical carcinoma. 

Fleur Talbot, James Ahlquist and Tom Kurzawinski,

Endocrine Unit, Southend Hospital, and Endocrine Surgery, University College London Hospital. 

Abstract:

Adrenocortical carcinoma (ACC) is a rare malignancy, usually presenting with abdominal mass, or signs of virilisation or Cushing’s syndrome.  Here we present a case of ACC with unusual features in the initial presentation. 

A 62 year old woman presented to general surgery with right upper quadrant pain.  She had recently had post-menopausal bleeding, attributed to uterine polyps.  A CT abdomen scan revealed a 12x8cm adrenal mass, and a biopsy was arranged directly.  Following biopsy she was referred to endocrinology; there was a 6 month history of worsening hirsutism, low mood, easy bruising and palpitations.  There were no signs of skin hyperpigmentation.  She had volatile blood pressure, notable facial hirsutism and plethora with thin skin.  Serum testosterone was 7.2 nmol/L, androstenedione >35 nmol/L, DHAS 26.2 umol/L, oestrogen was elevated at 195 pmol/L, ACTH <5 ng/L and 24 hour urinary free cortisol (386 nmol/day) & failure of dexamethasone suppression (9am cortisol 777 nmol/L) confirmed cortisol excess.  Review of the CT abdomen revealed endometrial hyperplasia.  FDG-PET demonstrated an FDG avid 9x13cm lesion.  A 15cm tumour was excised, and renal vein thrombosis was noted.  Histology was consistent with a primary adrenal cortical carcinoma, with infiltration but not breach of the capsule.  After surgery she was treated with dexamethasone.  Her post-operative investigations showed low or undetectable cortisol, androgens & oestradiol; there has been no further endometrial bleeding.  She has been treated with mitotane; recent blood tests and CT scan shows no sign of recurrence at 6 months. 

Discussion

This case highlights the importance of multidisciplinary working; it was fortunate that the patient did not have a phaeochromocytoma, as this had not been excluded by urinary catecholamines prior to the biopsy that might have precipitated a crisis.  The presenting feature of hirsutism was key to diagnosis.  It is likely that the excessive tumour androgens caused the recurrent endometrial hyperplasia, from peripheral conversion to oestrogens by aromatase.  This patient also demonstrates that the prognosis in ACC is not always poor, though the prognosis remains guarded.