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Neoplastic causes of abnormal puberty.

Susanne Wendt1, John Shelso, Karen Wright

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Neoplasm-related precocious puberty (PP) is a rare childhood cancer symptom. Early consideration of neoplastic causes is crucial for timely cancer diagnosis and treatment, as PP can precede malignancy by months or years.

Keywords:
adrenocortical carcinomabrain tumorgonadal tumorhepatoblastomaparaneoplastic endocrinopathyparaneoplastic precocious puberty

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Area of Science:

  • Pediatric Endocrinology
  • Pediatric Oncology
  • Cancer Etiology

Background:

  • Neoplasm-related precocious puberty (PP) is an uncommon presentation of childhood cancer.
  • Evaluating suspected PP is complex, often overlooking potential malignancy.
  • Cancer is frequently not considered in the initial work-up of PP.

Purpose of the Study:

  • To characterize clinicopathologic features of patients with PP at a pediatric cancer center.
  • To review literature on neoplasm-associated PP.
  • To develop a diagnostic algorithm for evaluating PP in children.

Main Methods:

  • Retrospective review of patients with neoplasm and PP at St. Jude Children's Research Hospital (1975-2011).
  • Analysis of demographic, clinical, endocrine, and neoplasm-related data.
  • Literature review to supplement case series findings.

Main Results:

  • 0.18% of pediatric cancer patients (24/13,615) presented with PP within 60 days.
  • Common primary diagnoses included brain tumors (12), adrenocortical carcinoma (5), and hepatoblastoma (4).
  • PP preceded neoplasm diagnosis by 0-48 months, with 17 peripheral and 7 central PP cases.

Conclusions:

  • Neoplasm-related PP is a rare paraneoplastic syndrome due to hormone production or altered gonadotropin regulation.
  • Early consideration of neoplastic causes is vital to prevent delayed cancer diagnosis.
  • Primary malignancy treatment resolved or improved PP in patients with intact hypothalamic-pituitary-gonadal axis.