TY - JOUR
T1 - Malignant peripheral nerve sheath tumor; A report of three cases
AU - Watanabe, Satoko
AU - Komatsu, Seiji
AU - Mae, Okiharu
AU - Ao, Masakazu
AU - Nose, Soichiro
PY - 2008/2/1
Y1 - 2008/2/1
N2 - We report three cases of malignant peripheral nerve sheath tumor (MPNST). One case was associated with neurofibromatosis type 1 (NF-1), while the other two were sporadic. All three patients who had undergone previous surgery at another unit developed local recurrence and underwent wide resection and reconstruction. The defects after tumor ablation were covered with a skin graft, superficial cervical artery perforator flap and latissimus dorsi myocutaneous flap, respectively. One case with NF-1 underwent adjuvant radiation therapy. All cases had no evidence of local recurrence after wide resection, while one with NF-I had lung metastases eight months after the surgery. Poor prognostic indicators for MPNST are as follows: 1) tumor size larger than 5 cm, 2) presence of NF-1, 3) insufficient resection due to small safety margin. Neither chemotherapy nor radiation therapy was reported to affect the survival rate. Therefore, it is essential to perform wide resection at the initial operation to improve the survival rate when a tumor grows and is suspected of being MPNST, especially in the case of NF-1 patients.
AB - We report three cases of malignant peripheral nerve sheath tumor (MPNST). One case was associated with neurofibromatosis type 1 (NF-1), while the other two were sporadic. All three patients who had undergone previous surgery at another unit developed local recurrence and underwent wide resection and reconstruction. The defects after tumor ablation were covered with a skin graft, superficial cervical artery perforator flap and latissimus dorsi myocutaneous flap, respectively. One case with NF-1 underwent adjuvant radiation therapy. All cases had no evidence of local recurrence after wide resection, while one with NF-I had lung metastases eight months after the surgery. Poor prognostic indicators for MPNST are as follows: 1) tumor size larger than 5 cm, 2) presence of NF-1, 3) insufficient resection due to small safety margin. Neither chemotherapy nor radiation therapy was reported to affect the survival rate. Therefore, it is essential to perform wide resection at the initial operation to improve the survival rate when a tumor grows and is suspected of being MPNST, especially in the case of NF-1 patients.
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M3 - Article
AN - SCOPUS:41549117940
VL - 51
SP - 217
EP - 224
JO - Japanese Journal of Plastic Surgery
JF - Japanese Journal of Plastic Surgery
SN - 0021-5228
IS - 2
ER -