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

To report the first case of an intracranial ganglion cell tumor in a patient with ROHHAD syndrome and discuss its classification based on the findings.

Key Findings:
  • The patient exhibited rapid weight gain and hypoventilation, consistent with ROHHAD syndrome.
  • An intracranial ganglion cell tumor was found, which is rare in ROHHAD syndrome cases.
  • Pathological analysis confirmed the tumor as a low-grade ganglion cell tumor.
Interpretation:

The presence of an intracranial tumor in this patient raises questions about the classification of tumors associated with ROHHAD syndrome, as discussed in the source.

Limitations:
  • The case is a single report and may not represent broader trends as noted in the source.
  • Further studies are needed to understand the implications of intracranial tumors in ROHHAD syndrome, as suggested in the source.
Conclusion:

This case highlights the need for awareness of both extra- and intracranial neural crest tumors in patients with rapid-onset obesity, as indicated in the source.

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