Subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2A.
OBJECTIVE
To describe our surgical technique for, and results of, subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia (MEN) type 2.


METHODS
Retrospective study.


METHODS
Teaching hospital, The Netherlands.


METHODS
6 patients (four women and 2 men, mean age 35 years, range 31-46) with MEN type 2 who presented between 1993 and 1996.


METHODS
Cortical sparing adrenalectomy (n =4) together with contralateral total adrenalectomy in bilateral disease (n = 2).


METHODS
Morbidity, mortality, adrenal function postoperatively, and recurrence.


RESULTS
Cortical sparing adrenalectomy leaving adequate adrenal reserve was possible in all cases. There was no operative morbidity or mortality. Mean follow-up was 40 months (range 1347). One patient developed a recurrent phaeochromocytoma 24 months after subtotal adrenalectomy.


CONCLUSIONS
Subtotal adrenalectomy with preservation of adequate adrenal cortical reserve was feasible in all cases. Long term follow-up is necessary to establish its definitive place in the treatment of familial phaeochromocytoma.
de Graaf
J S
JS
Department of Surgery, University Hospital Utrecht, The Netherlands.
Lips
C J
CJ
Rütter
J E
JE
van Vroonhoven
T J
TJ
D000302:Adrenal Cortex / Q000502:physiology; D000310:Adrenal Gland Neoplasms / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D018813:Multiple Endocrine Neoplasia Type 2a / Q000601:surgery*; D009364:Neoplasm Recurrence, Local; D010673:Pheochromocytoma / Q000601:surgery*; D013997:Time Factors