Case Report: First description of an intracranial “NET” in ROHHAD syndrome; time to rename to the ROHHAD-NeCT syndrome? - Scorecard - MDSpire

Case Report: First description of an intracranial “NET” in ROHHAD syndrome; time to rename to the ROHHAD-NeCT syndrome?

  • By

  • Nathalie J. Doelman-Oldenburger

  • Antoinette Y. N. Schouten-van Meeteren

  • Mariette E. G. Kranendonk

  • Kim Boshuisen

  • Michiel A. G. E. Bannier

  • Hanneke M. van Santen

  • May 29, 2026

  • 0 min

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Clinical Scorecard: First Report of an Intracranial Neuroendocrine Tumor in ROHHAD Syndrome: Should We Consider Renaming It to ROHHAD-NeCT Syndrome?

At a Glance

CategoryDetail
Condition
Key MechanismsHypothalamic dysfunction leading to hyperphagia and decreased energy expenditure; associated with ganglioneuroblastoma, neuroblastoma, or ganglioneuroma.
Target Population
Care Setting

Key Highlights

  • 40-50% of ROHHAD patients may have associated neuro-endocrine tumors, as reported in the source.

Guideline-Based Recommendations

Diagnosis

    Management

    • Management may include monitoring for respiratory failure and potential tumor treatment, as indicated in the source.

    Monitoring & Follow-up

      Risks

        Patient & Prescribing Data

        Prednisone treatment was initiated for suspected lymphoma, reflecting the diagnostic process.

        Clinical Best Practices

        • Consider ROHHAD syndrome in children with rapid weight gain and respiratory issues, as per clinical criteria.

        Related Resources & Content

        Original Source(s)

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