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Article Abstract
Background: Individuals with neurofibromatosis type 1 (NF1) are confronted with a 10% lifetime susceptibility to malignant peripheral nerve sheath tumors (MPNST). The majority of NF1-associated MPNST arise from pre-existing plexiform neurofibromas (PNF). Nevertheless, the precise subtypes of PNF susceptible to malignant transformation remain unclear.
Methods: This retrospective study included patients with head and neck PNF who underwent surgical treatment at Shanghai Ninth People's Hospital from June 2012 to July 2023. Logistic regression analyses were conducted to assess risk factors for malignant transformation. Cox proportional hazards regression evaluated the potential effects of variables on the time to malignant transformation.
Results: 19 (4%) of the 470 PNF underwent malignant transformation. Deep/proximal PNF involving cranial nerve ganglions are significantly associated with an increased risk of malignant transformation (adjusted OR, 3.10 [1.07 to 9.00]) while the associations with tumor morphology, relationship to adjacent tissues, and demographic parameters was not significant. Ganglion involvement significantly accelerated the time to malignant transformation by 36% (HR, 7.20 [2.33 to 22.28]).
Discussion: The involvement of cranial nerve ganglion is an independent risk factor for the malignant transformation of head and neck PNF. Consequently, patients with this high-risk profile require closer surveillance for malignancies and proactive management of suspicious lesions.
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