Clinical signs of neurofibromatosis impact on the outcome of malignant peripheral nerve sheath tumors.
OBJECTIVE
Malignant peripheral nerve sheath tumors (MPNST) are a rare subtype of sarcoma, with a poor outcome. MPNST are regarded as being sporadic or associated with neurofibromatosis type 1 (NF1). Few comparative overall-survival (OS) data in these 2 subsets of MPNST patients exist. The aim of this retrospective study was to assess OS in sporadic and NF1-associated MPNST patients.


METHODS
Fourteen consecutive patients with initial localized as well as initial metastatic MPNST were diagnosed and treated in our department. Patients with sporadic MPNST were assigned to group A and those with NF1-associated MPNST to group B.


RESULTS
Eight versus 6 patients were allocated to groups A and B. Primary tumors were located on the extremities in all but 1 patient. Two patients in group A and 4 patients in group B experienced a relapse. Four patients died in each of the 2 groups. Median follow-up was 66.2 and 57.2 months in group A and group B, respectively. Median OS in group A was 46.9 months versus 12.7 months in group B.


CONCLUSIONS
In this small, single-center study, sporadic-MPNST patients had a longer median OS than those with NF1-associated MPNST.
Clinical Division of Oncology, Department of Medicine I, Medical University of Vienna, Vienna, Austria.
W
W
Lamm
S
S
Schur
W J
WJ
Köstler
P
P
Funovics
R
R
Windhager
G
G
Amann
J
J
Panotopoulos
B
B
Pokrajac
T
T
Brodowicz
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D012189:Retrospective Studies; D055815:Young Adult