Oncologic Outcomes of Sporadic, Neurofibromatosis-Associated, and Radiation-Induced Malignant Peripheral Nerve Sheath Tumors
| العنوان: | Oncologic Outcomes of Sporadic, Neurofibromatosis-Associated, and Radiation-Induced Malignant Peripheral Nerve Sheath Tumors |
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| المؤلفون: | Nicole Moraco, Samuel Singer, Li-Xuan Qin, Cristina R. Antonescu, Murray F. Brennan, Jennifer LaFemina, Ryan C. Fields, Aimee M. Crago |
| المصدر: | Annals of Surgical Oncology. 20:66-72 |
| بيانات النشر: | Springer Science and Business Media LLC, 2012. |
| سنة النشر: | 2012 |
| مصطلحات موضوعية: | Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, Pathology, medicine.medical_specialty, Neoplasm, Residual, Neoplasms, Radiation-Induced, Neurofibromatosis 1, Adolescent, medicine.medical_treatment, Malignant peripheral nerve sheath tumor, Kaplan-Meier Estimate, Article, Nerve Sheath Neoplasms, Statistics, Nonparametric, Young Adult, Risk Factors, Surgical oncology, Confidence Intervals, Humans, Medicine, Neurofibroma, Neoplasm Metastasis, Neurofibromatosis, neoplasms, Prior Radiation Therapy, Aged, Proportional Hazards Models, Retrospective Studies, Aged, 80 and over, business.industry, Soft tissue sarcoma, Middle Aged, medicine.disease, Neoadjuvant Therapy, eye diseases, nervous system diseases, Survival Rate, Radiation therapy, Oncology, Chemotherapy, Adjuvant, Multivariate Analysis, Female, Radiotherapy, Adjuvant, Surgery, Neoplasm Recurrence, Local, business, Nerve sheath neoplasm, Follow-Up Studies |
| الوصف: | Malignant peripheral nerve sheath tumors (MPNSTs) occur sporadically, after prior radiation therapy (RT), or in association with neurofibromatosis type 1 (NF1). It is controversial whether patients with NF1-associated MPNST have worse outcomes. We investigated the prognostic significance of sporadic, NF1-associated, and RT-induced MPNST.Patients with primary high-grade MPNST from 1982 to 2011 were identified from a prospectively maintained database. Patients with sporadic MPNST were included only if the MPNST was not associated with NF1 or a neurofibroma or if it was immunohistochemically S100-positive.We studied 105 patients; 42 had NF1-associated tumors, 49 sporadic, and 14 RT-induced. Median age at diagnosis was 38 years. Median follow-up for surviving patients was 4 years. Mean tumor diameter was 5.5 cm for RT-induced tumors and 9.7 cm for NF1-associated and sporadic tumors (P=0.004). In multivariate analysis, factors associated with worse disease-specific survival (DSS) were larger size (HR 1.08; 95% CI 1.04-1.13; P0.001) and positive margin (HR 3.30; 95% CI 1.74-6.28; P0.001). Age, gender, site of disease, and S100 staining were not associated with DSS. The 3-year and median DSS were similar for NF1 and sporadic cases; combined 3-year DSS was 64% and median DSS was 8.0 years. For RT-induced tumors, 3-year DSS was 49% and median DSS was 2.4 years. The relationship between RT association and DSS approached statistical significance (HR 2.29; 95% CI 0.93-5.67; P=0.072).Margin status and size remain the most important predictors of DSS in patients with MPNST. NF1-associated and sporadic MPNSTs may be associated with improved DSS compared with RT-induced tumors. |
| تدمد: | 1534-4681 1068-9265 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ed6fc27cbbd5755c112059896315025dTest https://doi.org/10.1245/s10434-012-2573-2Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....ed6fc27cbbd5755c112059896315025d |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15344681 10689265 |
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