‘Adrenal rush’ in a patient with Neurofibromatosis-1

Figures & data

Table 1. Workup of to evaluate secondary causes of hypertension in our patient.

Investigations	Results
Blood/serum:
Thyroid function tests	Normal
Aldosterone	Normal
Renin	Normal
Free metanephrine	656 pg/ml (N < 57)
Free normetanephrine	1099 pg/ml (N < 148)
24-hour urine:
Dopamine	551 mcg/24-hour (N: 52–480)
Epinephrine	99 mcg/24-hour (N: 2–24)
Norepinephrine	327 mcg/24-hour (N: 15–100)
Metanephrine	15,203 mcg/24-hour (N: 36–190)
Normetanephrine	22,909 mcg/24-hour (N: 35–482)
Free cortisol	209 mcg/24-hour (N: 4–50)
Imaging:
Renal doppler ultrasound	No renal artery stenosis.

Figure 1. MRI abdomen/pelvis without contrast demonstrating bilateral solid slightly heterogeneous adrenal gland masses (yellow arrows).

Figure 2. PET scan showing intense FDG (fluorodeoxyglucose) localization within the enlarged right adrenal gland (yellow arrow).

Table 2. National institutes of health 1988 consensus development conference diagnostic criteria for Neurofibromatosis −1.

Diagnostic criteria (Patient is required to meet two or more of the criteria):
Six or more café au-lait macules >5 mm in prepubertal individuals and >15 mm in post-pubertal individuals Two or more neurofibromas of any type or one plexiform neurofibroma Freckling in axillary or inguinal regions Optic glioma Two or more iris hamartomas (Lisch nodules) Bony dysplasia or pseudo-arthrosis A first-degree relative with NF-1

Table 3. Conditions associated with Neurofibromatosis-1.

ASSOCIATED CONDITIONS
Vascular: Renal artery stenosis Coarctation of aorta Cervical AV fistulas Intra-cranial vascular aneurysms Congenital valvular abnormalities
Endocrine: Pheochromocytoma Pancreatic neuroendocrine tumors
Oncological: Gastrointestinal stromal tumors Rhabdomyosarcoma Leukemia Breast cancer