A Giant Retroperitoneal Teratoma Post-Operative Adrenal Insufficiency

Presentation Number: SAT-0761
Date of Presentation: June 21st, 2014

Tripti Joshi* and Shamasunder H Acharya
John Hunter Hospital, New Lambton, Australia

Abstract

TITLE: A Giant Retroperitoneal Teratoma and Post-Operative Adrenal Insufficiency.

Background: Large retroperitoneal teratomas involving adrenal gland are exceedingly rare.

Clinical case: A 29 year old male with a background of schizophrenia 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 appears to be low for his critical illness.

He received hydrocortisone replacement from 3rdpost –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 8 am cortisol was 102 nmol/L with an ACTH level of 5.6pmol/L (RR 0-10) at 0 min followed by a stimulated 60 min level of 206nmol/L. 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).

Conclusion: We present a case of large retroperitoneal teratoma involving adrenal gland with post- operative persistent hypocortisolism. Teratomas are not known to be secretory to cause adrenal suppression.

Teratomas are a type of non-seminomatous germ cell neoplasms, arising from pluripotent cells that can differentiate into ectoderm, mesoderm, and endoderm (1). Retroperitoneal teratomas are the least common of the extra-gonadal tumors and involvement of adrenal gland is exceedingly rare. Majority of retroperitoneal teratomas involving adrenal gland are asymptomatic, benign and present as an incidental finding and 6% of retroperitoneal teratomas are malignant. Surgical excision is recommended for symptomatic relief and malignant potential (2).

 

Nothing to Disclose: TJ, SHA