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Erschienen in: The Indian Journal of Pediatrics 8/2014

01.08.2014 | Original Article

Pheochromocytoma Management, Outcomes and the Role of Cortical Preservation

verfasst von: Alisha Gupta, Sandeep Agarwala, Nikhil Tandon, M. Srinivas, Minu Bajpai, Devendra Kumar Gupta, Arun Kumar Gupta, Chandersekhar Bal, Rakesh Kumar, Veereshwar Bhatnagar

Erschienen in: Indian Journal of Pediatrics | Ausgabe 8/2014

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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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Metadaten
Titel
Pheochromocytoma Management, Outcomes and the Role of Cortical Preservation
verfasst von
Alisha Gupta
Sandeep Agarwala
Nikhil Tandon
M. Srinivas
Minu Bajpai
Devendra Kumar Gupta
Arun Kumar Gupta
Chandersekhar Bal
Rakesh Kumar
Veereshwar Bhatnagar
Publikationsdatum
01.08.2014
Verlag
Springer India
Erschienen in
Indian Journal of Pediatrics / Ausgabe 8/2014
Print ISSN: 0019-5456
Elektronische ISSN: 0973-7693
DOI
https://doi.org/10.1007/s12098-013-1283-5

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