Large retroperitoneal teratomas involving adrenal gland are exceedingly rare.
A 29 year old male was found to have a large right renal /adrenal mass on CT scan whilst being worked up for hypertension in 2004. He was lost to follow up and represented in 2012 with abdominal discomfort. A repeat CT scan, showed this large and complex mass arising from the right kidney measuring 23 × 23 × 26 cm, encasing the dual renal arteries, causing right urinary tract obstruction. An elective right nephrectomy and removal of the mass was performed through laparotomy and median sternotomy, after consulting multi-disciplinary team.
The histology was suggestive of a mature teratoma with mucinous borderline proliferation with no secretory component arising from retroperitoneum. There was extensive perirenal fibrosis and necrosis. The right adrenal gland was compressed and trapped in the mass.
He had a complicated post-operative course and was hypotensive requiring vasopressors. Unexpectedly, he was found to have a low cortisol level of 276nmol/L at 0500 hrs. and 302nmol/L at 0800hrs on two separate occasions in the intensive care, which appeared to be low for his critical illness.
He received hydrocortisone replacement from 3rd post –operative day with improvement in his blood pressure.
Repeat cortisol was 205nmol/L with an ACTH level of 19.8pmol/L (RR 0-10). An outpatient short-synacthen test on two occasions found him to be glucocorticoid deficient. The adrenal antibodies were negative. The renin was 1.3ng/ml/hr. (1.2-2.8), Aldosterone 66 pmol/L (80-1040) with an aldosterone renin ratio of 1.8 (0-30). The 17-hydroxy progesterone was 10.2 nmol/L(<10.3) following synacthen stimulation making classic and non-classic congenital adrenal hyperplasia unlikely.
The tumor markers were negative: AFP 1IU/L (RR<8), HCG <2IU/L (RR <5).
We present a case of large retroperitoneal teratoma involving adrenal gland with persistent hypocortisolism..