Z029

 

Case of possible non-secretory metastatic Phaeochromocytoma

 

Z Banu, B Khoo, E Karra, D Patel, Royal Free London NHS Foundation Trust

 

A 46-year-old gentleman was diagnosed with right sided (6cm) sporadic phaeochromocytoma in 2010 at the age of 36. He had two-year history of palpitations, sweating, hypertension and multiple syncopal episodes. He underwent right adrenalectomy with partial nephrectomy at a separate London teaching unit. Histology showed completely excised phaeochromocytoma, positive for SDH staining with negative blood genetic analysis in 2011.He has been under surveillance at Royal Free since January 2017 with monitoring of 24 hours urinary metanephrines. He is currently not on any medication.

 

He had no significant past medical history of note. There was a family history of hypertension in both parents, and his mother had stroke at the age of 66. His father had MI in his 40s and died of heart failure at the age of 63. His older brother has lymphoma.

 

His BP was 145/86mmHg, heart rate 73/minute and BMI 26. There were no symptoms suggestive of adrenal hormone excess. He has had normal 24-hour urinary metanephrine since 2012.

 

 Biochemical profile (09/2019)

Result

Normal Range

24 Hr Urine Metadrenaline

0.43

0-1.2

24 Hr Urine Normetadrenaline

1.18

0-3.3

24 Hr Urine 3-Methoxytyramine

0.69

0-2.5

Free T4 Plasma

14.4 pmol

12-22

Thyroid Stimulating Hormone Serum

1.41 munit/L

0.3-4.2

Cortisol at 9am

252 nmol/L

172-497

 

He developed right abdominal pain and had been diagnosed with acute appendicitis. He underwent laparoscopic appendicectomy in June 2019 at Barnet Hospital with no surgical complication. A CT scan revealed incidental finding of left retroperitoneal mass in left suprarenal area, measuring 32mm.

 

Ga68 Dotatate PET CT showed lobulated retroperitoneal masses in the left suprarenal region and left para-aortic space at the level of the left renal vein which demonstrated low grade Dotatate uptake (less than background liver).  These may represent recurrent phaeochromocytoma, a paragangliomas or malignant/metastatic lymphadenopathy. A subsequent FDG PET CT demonstrated increased FDG activity in cluster of nodes centred to left of coeliac axis.

 

The adrenal MDT discussed management of an enlarging nodal mass possibly invading left renal vein, which is thought to be likely metastatic from previous phaeochromocytoma. Surgical management would require open resection with left nephrectomy. CT guided biopsy has been arranged prior to surgical intervention to achieve histological diagnosis. These results would be available for the meeting.