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KMID : 0613320030090020131
Journal of the Korean Bone and Joint Tumor Soceity
2003 Volume.9 No. 2 p.131 ~ p.138
Treatment and Survial Rate of Malignant Peripheral Nerve Sheath Tumors
Lee Jong-Seok

Jeon Dae-Geun
Cho Wan-Hyeong
Lee Soo-Yong
Oh Jung-Moon
Kim Jin-Wook
Abstract
Purpose: We analyzed our malignant peripheral nerve sheath tumor (MPNST) cases to find out their oncologic results following by each treatment modalities.

Materials and Methods: Thirty four patients with MPNST were registered in Korea Cancer Center Hospital from Feb. 1986 to Nov. 1996. Seventeen cases were male and 17, female. Average age was 41 years (range 18 to 74). Location of the tumor was as follows; 17 in lower extremity, 11 upper extremity, 4 trunk, and 2 retroperitoneum. Following the AJC classification, stage IA were 2 cases, stage IIA 2, stage IIB 6, stage III 16 and stage IV 8. Twenty six patients took operations and adjuvant chemotherapy and/or radiation therapy, 3 operation only and 3 adjuvant chemotherapy or radiation therapy. Average follow up period was 33.5 months (5.6 to 146.1). Kaplan-Meiyer method was done for survival curve, and log rank test for comparison analysis.

Results: Fourteen cases were continuous disease free, 2 no evidence of disease, 2 alive with disease and 14 dead of disease states at final follow up. Actual 5-year and 10-year survival rates were 53.5%, 35.7%. Local recurrence rate after operation was 24.1%. 5-year survival rates of stage I/II/III were 100/85.7/55.9% and 2-year survival rate of stage IV was 14.3% (p=0.04). In 21 cases operated with stage II-III, wide margin (15cases) had 76.0% 5-year survival rate, and marginal or intralesional marigin (6cases) had 40.0%. The actual 5-year survival rate of the group which were done 4 or more cycles chemotherapy (8cases) was 71.4% and the actual 3-year survival rate less than 4cycles chemotherapy (6cases) was 83.3% (p=0.96). In 19 cases operated with stage II-III and which had no radiotherapy, marginal or intralesional margin (5cases) had 3 cases of local recurrences (60.0%), though wide margin (14cases) had 4 cases recurrences (28.6%). There was no local recurrence in 8cases which had pre- or post-operative radiotherapy.

Conclusions: Surgical margin is an important factor in local recurrence. Resection margin has a tendency to influence the survival despite insufficient statistical significance. Conventional chemotherapy has no defnite statistical sigficance in the effect on local control and survival. Preoperative and postoperative radiotherapy has some positive effect on local control.
KEYWORD
Malignant peripheral nerve sheath tumor, Survival, Treatment
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