Original article
Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis

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Malignant peripheral nerve sheath tumors (MPNST) are rare. They account for 10% of all soft tissue sarcomas. The incidence of MPNST in patients with neurofibromatosis type 1 (NF-1) is 4%. A retrospective study was undertaken to evaluate the prognosis of patients with MPNST and NF-1 vs patients with MPNST alone.

Twenty-two patients with MPNST were diagnosed. The MPNST was diagnosed with NF-1 in 11 patients (50%). Treatment was defined as curative in 20 patients (90%), non-curative in one patient (5%), and one patient received no treatment.

The median disease-free survival (DFS) was 14 months (range 0–153 months). The median overall survival 24 months (range 1–153 months). There were three patients with isolated local failures (14%), four patients with local and distant failures (18%), and seven patients with isolated distant failures (32%). There was no statistical difference between patients with and without NF-1. Five patients with NF-1 developed a second MPNST (45%), none of the patients without NF-1 did (P = 0.018). A statistically worse survival was found when the duration of the first symptoms had been longer than six months.

Patients with MPNST have a poor prognosis with a high risk for local and distant failures (>50%). There is no statistical difference in the final outcome for MPNST with or without NF-1, but patients with NF-1 have a high risk for developing a second MPNST.

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    There have been only 2 cases of synchronous MPNST reported in the literature to date, and neither patient was reported as having NF-1.2,3 Multifocal presentation over time may be more common, with series ranging from 3.2%–45% of NF-1 patients with MPNST developing a second primary tumor over the long term.1,7 We did not identify any asynchronous, multifocal cases of MPNST in our cohort.

  • Malignant peripheral nerve sheath tumors of the head and neck: Demographics, clinicopathologic features, management, and treatment outcomes

    2015, Oral Oncology
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    These tumors can arise either sporadically or in association with neurofibromatosis (NF). Neurofibromatosis type I (NF1), also known as von Recklinghausen’s disease, accounts for 40–50% of MPNST cases, with an average age of onset 10–15 years younger than in sporadic cases and a predilection for central anatomical locations [4–6]. While the development of MPNST in this population has been related to genetic insults involving p53 and p16, NF1 gene activity is believed to predispose patients to spontaneous development or transformation of Schwann cells from a preexisting benign neurofibroma [7–9].

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The results of this study were presented at the Joint Meeting of the European Society of Surgical Oncology. British Association of Surgical Oncology, Chirurgische Arbeitsgemeinschaft Onkologie der Deutschen Gesellschaft für Chirurgie, Heidelberg, 8–11 June 1994.

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