Pheochromocytoma Management,Outcomes and the Role of Cortical Preservation |
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Authors: | Alisha Gupta Sandeep Agarwala Nikhil Tandon M. Srinivas Minu Bajpai Devendra Kumar Gupta Arun Kumar Gupta Chandersekhar Bal Rakesh Kumar Veereshwar Bhatnagar |
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Affiliation: | 1. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, 110029, India 2. Department of Endocrinology, All India Institute of Medical Sciences, New Delhi, India 3. Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India 4. Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
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Abstract: | Objectives 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 enroled. 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. |
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