Clinical features and anesthetic management of multiple endocrine neoplasia associated with pheochromocytoma.
OBJECTIVE
To investigate clinical features and anesthetic management of multiple endocrine neoplasia (MEN) associated with pheochromocytoma.


METHODS
Medical records of patients who were diagnosed as multiple endocrine neoplasia associated with pheochromocytoma in our hospital from April 1977 to April 2001 were reviewed retrospectively. The demographic data, clinical presentations, family history, biochemical examinations, type of MEN, sequence of different surgical procedures, anesthetic methods and hemodynamics during surgery were analyzed.


RESULTS
Thirteen cases of MEN associated with pheochromocytoma were investigated, accounting for 6% (13/213) of the pheochromocytoma patients admitted into our hospital. Nine of the 13 patients presented as type IIa MEN (Sipple syndrome), one as type IIb MEN, and three as mixed MEN. Four patients with type IIa MEN had a family history of similar disease. Five patients with other coexisting endocrine disorders first underwent excision of the pheochromocytomas, although only two had hypertensive symptoms at the time of admittance. Seven patients without histories of hypertension received surgical treatment for pheochromocytoma secondly. The excision of pheochromocytoma was performed under general anesthesia in 8 patients and epidural block in 4 patients. Marked hemodynamic fluctuation was recorded in 8 patients. No perioperative death was recorded.


CONCLUSIONS
Pheochromocytoma may be linked to other endocrine disorders during MEN, either as the main clinical presentation or most frequently as an occult tumor. Recognition of this feature of pheochromocytoma is of importance to the improvement of diagnosis and treatment both for pheochromocytoma and MEN.
Luo
Ailun
A
Department of Anesthesia, Peking Union Medical College Hospital, Beijing 100730, China. luoailun@public.pumch.ac.cn
Guo
Xiangyang
X
Ren
Hongzhi
H
Huang
Yuguang
Y
Ye
Tiehu
T
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D000315:Adrenalectomy; D000328:Adult; D000758:Anesthesia / Q000379:methods*; D005260:Female; D006439:Hemodynamics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000503:physiopathology / Q000601:surgery*; D010673:Pheochromocytoma / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies