Local Recurrence in Malignant Peripheral Nerve Sheath Tumors: A Multicenter Cohort Study
Christianne Y.M.N. Jansma, BSc1,2, Ibtissam Acem, BSc2, J. Henk Coert, MD PhD1, Walter Taal, MD, PhD3, Dirk J Grunhagen, MD, PhD4, Cornelis Verhoef, MD, PhD3 and Enrico Martin, MD, PhD5, 1University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, 2Erasmus MC Cancer Institute, Rotterdam, Zuid-Holland, Netherlands, 3Erasmus Medical Center, Rotterdam, Zuid-Holland, Netherlands, 4Erasmus Medical Center, Rotterdam, Netherlands, 5UMC Utrecht, Utrecht, Utrecht, Netherlands
Introduction: Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare and aggressive malignant soft-tissue sarcomas (STS), compromising 5-10% of all STS cases. Approximately 40% of MPNST cases are associated with neurofibromatosis type 1 (NF1). High-grade MPNSTs have a high rate of local recurrence (LR), making them the most recurrent tumors among all STS types. The development of recurrences in MPNST patients is associated with a morbid event that decreases functional outcomes, particularly since many patients have already undergone surgery and radiotherapy as part of multimodality treatment. Therefore, it is crucial to prevent LRs as much as possible. Due to the rarity of MPNSTs, risk factors, optimal LR treatment, and its impact on overall survival (OS) vary in current literature.
Materials and Methods: Surgically treated primary MPNSTs between 1988 to 2019 in the MONACO multicenter cohort were included. Multivariate Cox regression analyses were performed to identify factors associated for development of LR and OS after LR. Treatment of LR was evaluated.
Results: A total of 507 patients were included, of which 142 developed a first LR (28%). High-grade (HR 2.63; 95% CI, 1.15-5.99), microscopically positive margins (R1) (HR 2.19; 95% CI, 1.51-3.16), and large tumor size (HR 2.14; 95% CI, 1.21-3.78) were independent risk factors for the development of a LR. Radiotherapy (HR 0.62; 95% CI, 0.43-0.89) on the other hand, reduced the risk. Patients with a LR had significantly worse OS. In patients with a LR, synchronous metastasis was associated with poor OS (HR 1.79; 95% CI 1.02-3.14) while surgically treated LRs had a better OS (HR 0.38; 95% CI 0.22-0.64). Most LRs (64.9%) were treated surgically without additional therapy. Radiotherapy combined with surgery was administered in 11.3% of patients with a LR.
Conclusions: Large, high-grade MPNSTs with R1 resections are at increased risk of developing a LR, while the use of radiotherapy decreases this risk. Surgical treatment of LR is associated with improved OS.
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