Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis

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...

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Bibliographic Details
Published in:European journal of surgical oncology 1995-02, Vol.21 (1), p.78-82
Main Authors: Doorn, P.F., Molenaar, W.M., Buter, J., Hoekstra, H.J.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aged
Biological and medical sciences
Child
Combined Modality Therapy
Female
Humans
Male
malignant schwannoma
Medical sciences
Middle Aged
Nerve Sheath Neoplasms - complications
Nerve Sheath Neoplasms - therapy
neurofibroma
neurofibromatosis
Neurofibromatosis 1 - complications
neurofibrosarcoma
Neurology
Peripheral Nervous System Neoplasms - complications
Peripheral Nervous System Neoplasms - therapy
Prognosis
Retrospective Studies
Risk Factors
sarcoma
Treatment Outcome
Tumors of the nervous system. Phacomatoses
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Summary: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.
ISSN:0748-7983
1532-2157
DOI:10.1016/S0748-7983(05)80073-3