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Background <p>Patients with neurofibromatosis type 1 (NF1) have a 0.1–5.7% incidence of pheochromocytomas. Blood pressure control is difficult in cases of undiagnosed pheochromocytomas without preoperative treatment.</p> Case presentation <p>A 39-year-old woman with NF1 and severe cervical deformity presented with progressive quadriplegia and underwent urgent halo-vest placement. A large adrenal mass was incidentally detected on preoperative computed tomography (CT) scans; however, no further examination was performed because the patient had no symptoms suggestive of pheochromocytoma and her cervical spinal condition was considered urgent. When hemodynamic instability occurred during surgery, pheochromocytoma was suspected, and the diagnosis was confirmed postoperatively. Fourteen months later, the tumor was safely resected using strict preoperative management with metyrosine and alpha-blockers and a careful surgical plan.</p> Conclusions <p>This case highlights the importance of thorough preoperative screening and the role of anesthesiologists in detecting pheochromocytomas in asymptomatic patients with NF1 who present with intraoperative hemodynamic instability.</p>

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Intraoperative hemodynamic collapse revealing undiagnosed pheochromocytoma in a patient with neurofibromatosis type 1 and severe cervical deformity: a case report on perioperative management

  • 期刊论文

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  • 发表日期：

    2026年5月11日

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  • Riko Ideyama,
  • Chikashi Takeda,
  • Yohei Chiwata,
  • Yui Takei,
  • Figwang Fang,
  • Akiko Hirotsu,
  • Moritoki Egi

摘要

Background

Patients with neurofibromatosis type 1 (NF1) have a 0.1–5.7% incidence of pheochromocytomas. Blood pressure control is difficult in cases of undiagnosed pheochromocytomas without preoperative treatment.

Case presentation

A 39-year-old woman with NF1 and severe cervical deformity presented with progressive quadriplegia and underwent urgent halo-vest placement. A large adrenal mass was incidentally detected on preoperative computed tomography (CT) scans; however, no further examination was performed because the patient had no symptoms suggestive of pheochromocytoma and her cervical spinal condition was considered urgent. When hemodynamic instability occurred during surgery, pheochromocytoma was suspected, and the diagnosis was confirmed postoperatively. Fourteen months later, the tumor was safely resected using strict preoperative management with metyrosine and alpha-blockers and a careful surgical plan.

Conclusions

This case highlights the importance of thorough preoperative screening and the role of anesthesiologists in detecting pheochromocytomas in asymptomatic patients with NF1 who present with intraoperative hemodynamic instability.