Pheochromocytoma-induced hypertensive encephalopathy revealing MEN-IIa syndrome in a 13-year old boy. Implications for screening procedures and surgery.
In the multiple endocrine neoplasia (MEN) type II syndrome, pheochromocytomas become manifest at a later age than medullary thyroid carcinomas (MTC) do. The present report concerns a 13-year-old boy, belonging to a MEN-IIA kindred, who was admitted because of convulsive seizures related to hypertensive encephalopathy. A pheochromocytoma was suspected immediately and appropriate medical therapy was initiated. A right adrenal pheochromocytoma was removed, as well as a pheochromocytoma of an accessory right adrenal gland. Today, three years later our patient is still asymptomatic and the results of the thyroid C-cell provocative tests remain normal. This case clearly justifies the conclusion that periodic investigation of MEN-II family members to detect both medullary thyroid carcinoma and pheochromocytoma should begin early in life, as the latter may be the initial life-threatening expression of the disease. Long-term follow-up of patients treated with unilateral adrenalectomy will permit better definition of the risk of contralateral recurrence in such cases.
Jadoul
M
M
Department of Internal Medicine, Utrecht, University Hospital, The Netherlands.
Leo
J R
JR
Berends
M J
MJ
Ooms
E C
EC
Buurke
E J
EJ
Vasen
H F
HF
Seelen
P J
PJ
Lips
C J
CJ
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000235:genetics / Q000601:surgery; D000315:Adrenalectomy; D005820:Genetic Testing; D006801:Humans; D007427:Intracranial Pressure; D008297:Male; D009377:Multiple Endocrine Neoplasia / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology; D010375:Pedigree; D010673:Pheochromocytoma / Q000150:complications* / Q000235:genetics / Q000601:surgery