TY - JOUR
T1 - Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors
AU - Lafemina, Jennifer
AU - Qin, Li Xuan
AU - Moraco, Nicole H.
AU - Antonescu, Cristina R.
AU - Fields, Ryan C.
AU - Crago, Aimee M.
AU - Brennan, Murray F.
AU - Singer, Samuel
N1 - Funding Information:
ACKNOWLEDGMENT This work was supported by Soft Tissue Sarcoma Program Project grant P01 CA 047179 (SS), SPORE in Soft Tissue Sarcoma P50 CA 14014 (SS), and the Dr. Murray F. Brennan/ Gorin Fellowship Endowment Fund (JL).
PY - 2013/1
Y1 - 2013/1
N2 - Background: 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. Methods: 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. Results: 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; P < 0.001) and positive margin (HR 3.30; 95 % CI 1.74-6.28; P < 0.001). 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). Conclusions: 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.
AB - Background: 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. Methods: 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. Results: 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; P < 0.001) and positive margin (HR 3.30; 95 % CI 1.74-6.28; P < 0.001). 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). Conclusions: 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.
UR - http://www.scopus.com/inward/record.url?scp=84871764535&partnerID=8YFLogxK
U2 - 10.1245/s10434-012-2573-2
DO - 10.1245/s10434-012-2573-2
M3 - Article
C2 - 22878618
AN - SCOPUS:84871764535
SN - 1068-9265
VL - 20
SP - 66
EP - 72
JO - Annals of Surgical Oncology
JF - Annals of Surgical Oncology
IS - 1
ER -