Endgames Picture Quiz

A woman with a suprarenal mass and hypertension

BMJ 2009; 338 doi: http://dx.doi.org/10.1136/bmj.b333 (Published 05 March 2009) Cite this as: BMJ 2009;338:b333
  1. H Kahal, specialist trainee 3 in diabetes and endocrinology,
  2. E Cooper, foundation year 2,
  3. R Sriraman, specialist registrar in general medicine, diabetes, and endocrinology,
  4. D V Coppini, consultant physician, honorary senior lecturer in diabetes and endocrinology
  1. 1Department of Diabetes and Endocrinology, Poole Hospital NHS Foundation Trust, Poole BH15 2JB
  1. 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 …

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