title: Patterns of recurrence and survival in sporadic, neurofibromatosis Type 1-associated, and radiation-associated malignant peripheral nerve sheath tumors.
abstract: OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are an aggressive group of soft tissue sarcomas that can arise sporadically, in the context of neurofibromatosis Type 1 (NF1) or at a site of prior irradiation. Large series profiling the features and outcomes of sporadic, NF1-associated, and radiation-associated MPNSTs are limited. The goal of this study was to elucidate differences between MPNST etiologies in a large single-institution retrospective study. METHODS Patients (n = 317) were identified through the tumor registry of The University of Texas MD Anderson Cancer Center. Clinicopathological features were retrospectively collected. Features were compared among MPNST subtypes for patients who had sufficient clinical history (n = 289), and clinicopathological features were used to identify adverse predictors of recurrence and survival outcomes. RESULTS Five-year local recurrence-free survival (LRFS), distant recurrence-free survival (DRFS), and disease-specific survival (DSS) estimates were 56.6%, 49.6%, and 53.6%, respectively, for the high-grade MPNST cohort. Five-year DSS was lower in NF1-associated and radiation-associated MPNST than in sporadic MPNST (52%, 47%, and 67%, respectively, p = 0.140). Patients with radiation-associated MPNST had worse 5-year LRFS than those with the sporadic and NF1-associated subtypes (RT-associated vs sporadic, p = 0.010; RT-associated vs NF1-associated, p = 0.232). Truncally located tumors, positive surgical margins, local recurrence, and metastasis were predictors of adverse DSS in multivariate analysis. CONCLUSIONS Radiation-associated MPNSTs are associated with poorer local recurrence-free and disease-specific survival than sporadic and NF1-associated tumors. NF1-associated MPNSTs may have worse survival outcomes owing to large tumor size, compromising truncal location, and lower rate of negative resection margins compared with sporadic tumors.
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Kelsey L
authors.initials: KL
authors.lastname: Watson
authors.affiliation: Pathology.
authors.forename: Ghadah A
authors.initials: GA
authors.lastname: Al Sannaa
authors.affiliation: Departments of 1 Surgical Oncology.|The University of Texas Health Science Center at Houston Graduate School of Biomedical Sciences, Houston, Texas.
authors.forename: Christine M
authors.initials: CM
authors.lastname: Kivlin
authors.affiliation: Pathology.
authors.forename: Davis R
authors.initials: DR
authors.lastname: Ingram
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Sharon M
authors.initials: SM
authors.lastname: Landers
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Christina L
authors.initials: CL
authors.lastname: Roland
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Janice N
authors.initials: JN
authors.lastname: Cormier
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Kelly K
authors.initials: KK
authors.lastname: Hunt
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Barry W
authors.initials: BW
authors.lastname: Feig
authors.affiliation: Radiation Oncology.
authors.forename: B
authors.initials: B
authors.lastname: Ashleigh Guadagnolo
authors.affiliation: Radiation Oncology.
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Bishop
authors.affiliation: Pathology.
authors.forename: Wei-Lien
authors.initials: WL
authors.lastname: Wang
authors.affiliation: Neuro-Oncology, and.
authors.forename: John M
authors.initials: JM
authors.lastname: Slopis
authors.affiliation: Neurosurgery, The University of Texas MD Anderson Cancer Center; and.
authors.forename: Ian E
authors.initials: IE
authors.lastname: McCutcheon
authors.affiliation: The University of Texas Health Science Center at Houston Graduate School of Biomedical Sciences, Houston, Texas.
authors.forename: Alexandar J
authors.initials: AJ
authors.lastname: Lazar
authors.affiliation: Departments of 1 Surgical Oncology.
authors.forename: Keila E
authors.initials: KE
authors.lastname: Torres
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D009381:Neoplasms, Radiation-Induced / Q000453:epidemiology* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy; D018319:Neurofibrosarcoma / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology / Q000628:therapy; D012189:Retrospective Studies; D016019:Survival Analysis; D055815:Young Adult
keywords: DRFS = distant recurrence-free survival; DSS = disease-specific survival; HR = hazard ratio; LRFS = local recurrence-free survival; MPNST; MPNST = malignant peripheral nerve sheath tumor; NF1 = neurofibromatosis Type 1; malignant peripheral nerve sheath tumor; neurofibromatosis Type 1; radiation-induced sarcoma