Open AccessSubtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2A
Author(s) -
de Graaf J. S.,
Lips C. J. M.,
Rütter J. E.,
van Vroonhoven T. J. M. V.
Publication year - 1999
Publication title -
european journal of surgery
Language(s) - English
Resource type - Journals
eISSN - 1741-9271
pISSN - 1102-4151
DOI - 10.1080/110241599750006415
Subject(s) - medicine , multiple endocrine neoplasia , pheochromocytoma , adrenalectomy , surgery , multiple endocrine neoplasia type 2 , endocrine system , bilateral adrenalectomy , general surgery , hormone , germline mutation , mutation , biochemistry , chemistry , gene
Objective: To describe our surgical technique for, and results of, subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia (MEN) type 2. Design: Retrospective study. Setting: Teaching hospital, The Netherlands. Subjects: 6 patients (four women and 2 men, mean age 35 years, range 31–46) with MEN type 2 who presented between 1993 and 1996. Interventions: Cortical sparing adrenalectomy ( n = 4) together with contralateral total adrenalectomy in bilateral disease ( n = 2). Main outcome measures: 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 13–47). 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. Copyright © 1999 Taylor and Francis Ltd.