[Type II multiple endocrine neoplasms (MEN IIB): an unusual cause of constipation. A plea for early thyroidectomy].
We present a 5 year old girl who was diagnosed to have multiple endocrine neoplasia syndrome type IIB (MEN IIB). She presented with serious constipation and had the distinct features of MEN IIB. 100% of the patients with MEN IIB develop medullary thyreoïd carcinoma (MTC). MTC in MEN IIB is known to behave in a very aggressive way. Microscopically MTC was already present in this patient despite of normal calcitonin stimulation tests. Accordingly we plead for a more radical management relating to thyreoïdectomy in MEN IIB.
Post
E D
ED
Wilhelmina Kinderziekenhuis, Universiteitskliniek voor kinderen en jeugdigen, afd. alg. Pediatrie, Utrecht.
Wauters
E A
EA
Huber
J
J
Williams
E D
ED
Lips
C J
CJ
D002675:Child, Preschool; D003248:Constipation / Q000209:etiology*; D005260:Female; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000150:complications*; D013964:Thyroid Neoplasms / Q000473:pathology* / Q000601:surgery; D013965:Thyroidectomy
Multiple endocriene neoplasieën type II (MEN IIB): een ongewone oorzaak van obstipatie. Een pleidooi voor vroege thyreoïdectomie.