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Background <p>Advances in the management of NF2-related schwannomatosis (NF2-SWN) have improved survival, but long-term complications such as increased intracranial pressure (IICP) remain insufficiently characterized. This study investigated the mechanisms, clinical presentation, and prognostic impact of IICP in NF2-SWN.</p> Methods <p>We conducted a bicentric retrospective study including 551 NF2-SWN patients followed between 1994 and 2026 in two national reference centers. IICP was defined using combined clinical, ophthalmological, and radiological criteria, including papilledema. Mechanisms were categorized into hydrocephalus, tumor-related mass effect, and venous outflow obstruction. Volumetric analyses were performed using 3D segmentation to calculate tumor volumes and annual growth rates. Clinical manifestations of IICP were also compared according to history of posterior fossa vestibular schwannoma surgery.</p> Results <p>Thirty-three patients (6.0%) developed confirmed IICP, predominantly with moderate-to-severe genetic phenotypes (83%). Mechanisms included hydrocephalus (45.5%), venous outflow obstruction (36.4%), and tumor-related mass effect (15.2%). Outcomes were poor, with visual impairment in 30%, blindness in 6%, and death in 12% of patients. Annual tumor growth rates were significantly higher in patients with IICP than in controls (median 0.41 vs 0.04 year⁻¹, p = 0.011), whereas absolute tumor volume was not significantly different. Prior vestibular schwannoma surgery was independently associated with a lower frequency of headache (OR 0.06, p = 0.0019).</p> Conclusions <p>IICP in NF2-SWN is an uncommon but potentially severe complication associated with major visual morbidity and mortality. Because classical symptoms may be absent, systematic ophthalmological and radiological surveillance appears essential for early detection and management.</p>

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Increased intracranial pressure in NF2‑related schwannomatosis: an underestimated danger with serious consequences

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年5月21日

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  • Antoine Keraudy,
  • Christophe Vincent,
  • Michael Risoud,
  • Apolline Monfilliette,
  • Riyad Hanafi,
  • Matthieu Peyre,
  • Nicolas Reyns,
  • Michel Kalamarides,
  • Rabih Aboukais

摘要

Background

Advances in the management of NF2-related schwannomatosis (NF2-SWN) have improved survival, but long-term complications such as increased intracranial pressure (IICP) remain insufficiently characterized. This study investigated the mechanisms, clinical presentation, and prognostic impact of IICP in NF2-SWN.

Methods

We conducted a bicentric retrospective study including 551 NF2-SWN patients followed between 1994 and 2026 in two national reference centers. IICP was defined using combined clinical, ophthalmological, and radiological criteria, including papilledema. Mechanisms were categorized into hydrocephalus, tumor-related mass effect, and venous outflow obstruction. Volumetric analyses were performed using 3D segmentation to calculate tumor volumes and annual growth rates. Clinical manifestations of IICP were also compared according to history of posterior fossa vestibular schwannoma surgery.

Results

Thirty-three patients (6.0%) developed confirmed IICP, predominantly with moderate-to-severe genetic phenotypes (83%). Mechanisms included hydrocephalus (45.5%), venous outflow obstruction (36.4%), and tumor-related mass effect (15.2%). Outcomes were poor, with visual impairment in 30%, blindness in 6%, and death in 12% of patients. Annual tumor growth rates were significantly higher in patients with IICP than in controls (median 0.41 vs 0.04 year⁻¹, p = 0.011), whereas absolute tumor volume was not significantly different. Prior vestibular schwannoma surgery was independently associated with a lower frequency of headache (OR 0.06, p = 0.0019).

Conclusions

IICP in NF2-SWN is an uncommon but potentially severe complication associated with major visual morbidity and mortality. Because classical symptoms may be absent, systematic ophthalmological and radiological surveillance appears essential for early detection and management.