Phaechromocytoma is a rare
catecholamine secreting
tumor, which occasionally presents as a life threatening crisis in association with surgery and
anesthesia. We report a 58-year-old women with known Recklinghausen's disease who was admitted for elective resection of a pancreas tail
cystadenoma. A
cystadenocarcinoma was taken into account differential diagnostically. No clinical symtoms or signs pointing to a
hormone active
tumor were found preoperatively. After opening of the abdomen and palpation of the
tumor, a
hypertensive crisis occurred accompanied by considerable
tachycardia, leading to the tentative diagnosis of a phaeochromocytoma in connection to the known
phacomatosis. The
hypertensive crisis was treated with
nitroglycerin and
esmolol. The putative
tumor of the pancreas represented itself as an adrenal
tumor without relationship to the pancreas. Following
ligature of the suprarenal vein,
antihypertensive therapy could be finished. For stabilization of blood pressure a
noradrenaline application was necessary in descending dosage over a period of two days. The further postoperative course was without complications. The results of the urine
catecholamine measurements and histological examinations confirmed the intraoperative diagnosis. An unidentified phaeochromocytoma is a vital threat for patients during surgery and
anesthesia. Phaeochromocytomas are observed in patients suffering from Recklinghausen's disease (and other
phacomatoses) in an above average incidence. Therefore, such a
tumor should be excluded in these patients before elective surgery even if the patient does not show symptoms (asymptomatic phaeochromocytomas occur). The determination of
catecholamines in 24 hour urine collections is an easy and specific diagnostic procedure and should be used in patients suffering from
phacomatoses before elective surgery.