Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience
| Autor: | Tomasz Świtaj, Tadeusz Morysiński, Piotr Rutkowski, Hanna Koseła-Paterczyk, Anna M. Czarnecka, Paweł Sobczuk, Marcin Zdzienicki, Tomasz Goryń, Paweł Teterycz, Katarzyna Kozak, Sławomir Falkowski |
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| Rok vydání: | 2020 |
| Předmět: |
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 |
| Zdroj: | Surgical Oncology. 35:276-284 |
| ISSN: | 0960-7404 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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