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Pediatric and adolescent pheochromocytoma: clinical presentation and outcome of surgery

Anjali Mishra1, Prateek Kumar Mehrotra, Gaurav Agarwal

  • 1Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, UP, India. Correspondence to: Dr Anjali Mishra, Additional Professor, Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226 014, India. anjali@sgpgi.ac.in.

Indian Pediatrics
|May 15, 2014
PubMed

Insights

A study of pediatric pheochromocytoma surgery in India found familial cases and persistent hypertension. Long-term follow-up and thorough history are crucial for diagnosing familial pheochromocytoma.

Area of Science:

  • Pediatric Surgery
  • Endocrinology
  • Oncology

Background:

  • Pheochromocytoma is a rare neuroendocrine tumor.
  • Pediatric pheochromocytoma presents unique diagnostic and management challenges.

Purpose of the Study:

  • To detail the clinical presentation and surgical outcomes of pediatric pheochromocytoma.
  • To identify key factors in managing this rare tumor in children.

Main Methods:

  • Retrospective review of clinical records for 24 pediatric patients operated between 1990 and 2011.
  • Diagnosis of familial disease confirmed through clinical examination and follow-up.

Main Results:

  • Familial, bilateral, and extra-adrenal pheochromocytomas were observed in 20.8%, 20.8%, and 12.5% of cases, respectively.
  • Malignant pheochromocytoma occurred in 4.2% of patients.
  • Median follow-up was 36 months, with 12.5% experiencing persistent hypertension and a similar proportion mortality.

Conclusions:

  • Good patient history and extended follow-up are vital for identifying familial pheochromocytoma in children.
  • Genetic screening should be considered, especially in the absence of routine testing.
Abstract

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