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This case demonstrates the complex surgical approach for pheochromocytoma in a patient with neurofibromatosis type 1, emphasizing unique challenges and implications.
A 41-year-old woman who was diagnosed, with neurofibromatosis type 1 (NF1) in childhood, experienced significant fluctuation in blood pressure during cervical kyphosis surgery. Postoperative examination revealed a right pheochromocytoma, which was a large hypervascular tumor with a maximum diameter of 18 cm. The patient had a short stature and thoracic deformity associated with NF1, which posed a challenge in securing the surgical field in the upper abdomen during the resection procedure. Additionally, it was necessary to consider the vascular and bone fragility associated with NF1. A positioning simulation was performed in collaboration with other medical departments the day before surgery. On the day of the surgery, the patient was positioned in a magic bed, to allow for safe rotation. A wide surgical field was successfully secured by performing a thoracoabdominal opening with a midline upper abdominal incision and a diagonal incision at the 9th intercostal space. The intraoperative hemodynamics were stable, and the surgery was completed safely. The patient has been recurrence-free for approximately 2 years since the surgery. Patients with NF1 have various lesions that differ greatly from one individual to another, and each case requires an appropriate surgical approach.
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Hirano et al. (2025) studied this question.
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