title: Subtotal adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2A.
abstract: 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.
authors.affiliation: Department of Surgery, University Hospital Utrecht, The Netherlands.
authors.forename: J S
authors.initials: JS
authors.lastname: de Graaf
authors.forename: C J
authors.initials: CJ
authors.lastname: Lips
authors.forename: J E
authors.initials: JE
authors.lastname: Rütter
authors.forename: T J
authors.initials: TJ
authors.lastname: van Vroonhoven
mesh_terms: 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