- H Kahal, specialist trainee 3 in diabetes and endocrinology,
- E Cooper, foundation year 2,
- R Sriraman, specialist registrar in general medicine, diabetes, and endocrinology,
- D V Coppini, consultant physician, honorary senior lecturer in diabetes and endocrinology
- 1Department of Diabetes and Endocrinology, Poole Hospital NHS Foundation Trust, Poole BH15 2JB
- Correspondence to: H Kahal hassoon011{at}yahoo.com
A woman presented to surgeons with abdominal discomfort in 1990. She underwent abdominal computed tomography, which showed a large incidental right suprarenal mass (fig 1⇓). Follow-up scans suggested a benign stable adenoma, measuring 49×60 mm, and the decision was made not to operate. An endocrine opinion was not sought. Her medical history included hypertension.
Fig 1 Abdominal computed tomography scan showing a suprarenal mass (arrow)
In June 2006, at the age of 69, she re-presented with peritonitis, and a laparotomy showed a thickened wall cyst at the duodeno-jejunal junction. During elective excision of the cyst in April 2007, it was difficult to achieve haemodynamic stability. An unplanned resection of the suprarenal mass was performed concomitantly. The cyst stained strongly positive for CD117. Figure 2⇓ shows the patient’s clinical features.
Fig 2 The patient’s clinical features
Questions
1 What is the patient’s underlying condition?
2 What is the cause of her hypertension?
3 What is the incidental finding in this case, as suggested by the cyst’s histology?
Answers
Short answers
1 The presence of numerous neurofibromas and café au lait macules (fig 3⇓) is diagnostic for neurofibromatosis type 1.
Fig 3 The patient’s clinical features. The black arrow points to a neurofibroma and the pink arrow points to a café au lait macule
2 Phaeochromocytoma, which has an overall incidence of <1% in patients with neurofibromatosis …
Sign in
Personal subscribers, sign in here:
Article access
Article access for 1 day
Purchase this article for £20 $30 €32*
The PDF version can be downloaded as your personal record

CiteULike
Connotea
Del.icio.us
Digg
Facebook
Reddit
Technorati
Twitter
Stumbleupon
Rapid responses
Latest Responses
The decline in the breast cancer incidence is 1.2% and it is not significant.
Published 10 February 2012
'twas ever thus
Published 10 February 2012
The value of historic human remains
Published 10 February 2012
In Praise of British Literature
Published 10 February 2012
Is real shared decision making possible?
Published 10 February 2012
Most responses
Does anyone understand the government’s plan for the NHS? (17 responses)
Published 17 Jan 2012
Bad medicine: medical nutrition (15 responses)
Published 18 Jan 2012
Shared decision making: really putting patients at the centre of healthcare (7 responses)
Published 27 Jan 2012
Why legislation is necessary for my health reforms (7 responses)
Published 1 Feb 2012
Search for evidence goes on (5 responses)
Published 17 Jan 2012