Table 1 Patient and tumor characteristics for 21 patients with neurofibromatosis 1 and a diagnosis of malignant peripheral nerve-sheath tumors between 1993 and 2003
Age, years | |
|---|---|
Median (range) | 31 (18–79) |
Tumor size, cm | |
Median (range) | 13 (3–28) |
Gender, n (%) | |
Male | 12 (57) |
Female | 9 (43) |
Metastases at diagnosis, n (%) | |
Absent | 18 (86) |
Present | 3 (14) |
Type of surgery, n (%) | |
R0, complete resection | 1 (5) |
R1, incomplete resection (microscopically) | 3 (14) |
R2, incomplete resection (macroscopically) | 7 (33) |
No surgery | 10 (48) |
Histological grade (FNCLCC), n (%) | |
1 | 1 (5) |
2 | 8 (38) |
3 | 12 (57) |
Pathological staging, n (%) | |
T1 | 3 (14) |
T2 | 18 (86) |
Tumor site, n (%) | |
Head and neck | 3 (14) |
Trunk | 4 (19) |
Extremity | 8 (38) |
Abdomen or pelvis | 6 (29) |
Chemotherapy, n (%) | |
Single agent | 6 (29) |
Combination | 15 (71) |
Type of chemotherapy, n (%) | |
Doxorubicin | 5 (24) |
Ifosfamide | 1 (5) |
Ifosfamide + anthracycline | 11 (52) |
Ifosfamide + anthracycline + platinum salt | 4 (19) |