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Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience
- Source :
- Surgical Oncology. 35:276-284
- Publication Year :
- 2020
- Publisher :
- Elsevier BV, 2020.
-
Abstract
- Introduction Malignant peripheral nerve sheath tumor (MPNST) accounts for about 5% of soft tissue sarcomas. It can occur as sporadic diseases or can be associated with type 1 neurofibromatosis. MPNST is usually associated with poor prognosis, mostly due to their aggressive behavior, high metastatic potential, and resistance to chemotherapy. Our study aimed to determine treatment outcomes and associated prognostic factors in a large cohort of patients with MPNSTs treated at the reference sarcoma center. Methods 239 consecutive patients (114 women and 125 men) diagnosed with MPNST between March 1998 and March 2018 who were treated with surgery with curative intent in the reference sarcoma center were included in the retrospective analysis. Results The mean age at diagnosis was 51 years (range 15–86). 28 (11.7%) patients had neurofibromatosis type 1 associated tumors (NF1 positive). Median OS was 126.5 months and 5-year survival rate was 61.9% in the group treated with curative intent. Median DFS, LRFS and DMFS were 91.6, 126.5 and 126.5 months, respectively. We identified tumor size, high tumor grade and positive surgical margins as independent negative predictors of DFS, LRFS, DMFS and OS. Conclusions High-quality surgery remains a gold standard of MPNST treatment. High grade, size and quality of surgery are significant independent prognostic factors for overall survival. There is an unmet need for improvement, especially regarding the perioperative treatment and treatment of metastatic disease. Future studies on the biology of MPNST would lead to the development of novel treatment options and improvement of treatment outcomes.
- Subjects :
- Adult
Male
0301 basic medicine
Oncology
medicine.medical_specialty
Adolescent
medicine.medical_treatment
Malignant peripheral nerve sheath tumor
Disease
Disease-Free Survival
Nerve Sheath Neoplasms
Cohort Studies
Young Adult
03 medical and health sciences
0302 clinical medicine
Internal medicine
medicine
Humans
Neurofibromatosis
Survival rate
Aged
Aged, 80 and over
Chemotherapy
business.industry
Sarcoma
Perioperative
Middle Aged
Prognosis
medicine.disease
Survival Rate
Treatment Outcome
030104 developmental biology
030220 oncology & carcinogenesis
Female
Surgery
Poland
Positive Surgical Margin
business
Subjects
Details
- ISSN :
- 09607404
- Volume :
- 35
- Database :
- OpenAIRE
- Journal :
- Surgical Oncology
- Accession number :
- edsair.doi.dedup.....ce1f9b225a026f774a550062b9b8b08b