title: Pheochromocytoma management, outcomes and the role of cortical preservation.
abstract: OBJECTIVE
  To evaluate the management and outcome of children with pheochromocytoma and determine the role of cortex preservation in cases of bilateral disease.
  
  
  METHODS
  Retrospective review of children, below 12 y of age, with pheochromocytoma managed between November 2003 and December 2012 was done.
  
  
  RESULTS
  Twelve patients, nine boys and three girls with median age 9 y were enrolled. Eleven (92 %) had adrenal tumors and in one it was extra-adrenal. Five (42 %) had bilateral disease. Ten presented with hypertension, one with headache and one with abdominal pain and fever. All were stabilized pre-operatively with alpha and beta blockers and volume expansion. Six children with unilateral disease underwent total adrenalectomy. Out of five with bilateral disease, one child underwent bilateral total adrenalectomy and was later started on hormone replacement. Remaining four underwent total adrenalectomy on one side and partial on the other side. Post-operatively all became symptom free and normotensive and were off medications within 1 mo. Two children developed recurrence 1 mo post-operatively, one with an initial unilateral pheochromocytoma and one with paraganglionoma. At the last follow up, 10 out of 12(83 %) were disease free while two with recurrence are still awaiting surgery.
  
  
  CONCLUSIONS
  Surgical resection of pheochromocytoma is effective treatment to achieve cure and prolong survival. Cortex preservation should be done in bilateral disease as risk of recurrence in such cases seems to be of lesser significance as compared to the morbidity and mortality of adrenal insufficiency and consequent lifelong hormone replacement.
authors.affiliation: Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India.
authors.forename: Alisha
authors.initials: A
authors.lastname: Gupta
authors.forename: Sandeep
authors.initials: S
authors.lastname: Agarwala
authors.forename: Nikhil
authors.initials: N
authors.lastname: Tandon
authors.forename: M
authors.initials: M
authors.lastname: Srinivas
authors.forename: Minu
authors.initials: M
authors.lastname: Bajpai
authors.forename: Devendra Kumar
authors.initials: DK
authors.lastname: Gupta
authors.forename: Arun Kumar
authors.initials: AK
authors.lastname: Gupta
authors.forename: Chandersekhar
authors.initials: C
authors.lastname: Bal
authors.forename: Rakesh
authors.initials: R
authors.lastname: Kumar
authors.forename: Veereshwar
authors.initials: V
authors.lastname: Bhatnagar
mesh_terms: D000302:Adrenal Cortex / Q000502:physiology; D000310:Adrenal Gland Neoplasms / Q000601:surgery*; D000315:Adrenalectomy; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D010673:Pheochromocytoma / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome