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Pheochromocytoma in a Child without Hypertension: A Contribution to the "Rule of 10s"

Prasanta Kumar Tripathy1, Kaumudee Pattnaik2, Manjushree Nayak2

  • 1Department of Pediatric Surgery, SCB Medical College, Cuttack, Odisha, India.

Indian Journal of Medical and Paediatric Oncology : Official Journal of Indian Society of Medical & Paediatric Oncology
|May 5, 2017
PubMed

Insights

Pheochromocytoma (PCC) is a rare adrenal tumor. This case highlights a nonhypertensive PCC in a 14-month-old, contributing to the "rule of 10s" in pediatric oncology.

Area of Science:

  • Pediatric Oncology
  • Endocrinology
  • Neuroendocrinology

Background:

  • Pheochromocytoma (PCC) is a neuroendocrine tumor of the adrenal medulla.
  • While well-defined in adults, pediatric PCC diagnosis and treatment present unique challenges.
  • Children represent only 10% of reported PCC cases, typically diagnosed around age 11.

Observation:

  • A 14-month-old female presented with PCC, an unusually young age at diagnosis.
  • Unlike typical presentations, this child was not hypertensive.
  • This case aligns with the "rule of 10s" for pediatric pheochromocytoma.

Findings:

  • PCC can occur in very young children.
  • Nonhypertensive presentation is possible in pediatric PCC.
  • This case adds to the understanding of atypical pediatric PCC.

Implications:

  • Highlights the need for considering PCC in hypertensive and normotensive children.
  • Emphasizes the importance of early diagnosis in pediatric neuroendocrine tumors.
  • Contributes to the limited data on childhood pheochromocytoma, especially atypical cases.

Keywords:
Adrenal medullachromaffin cellsparagangliomapheochromocytoma

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