Clinical Signs of Neurofibromatosis Impact on the Outcome of Malignant Peripheral Nerve Sheath Tumors

• Published in: Oncology Vol. 86; no. 2; pp. 122 - 126
• Main Authors: Lamm, W., Schur, S., Köstler, W.J., Funovics, P., Windhager, R., Amann, G., Panotopoulos, J., Pokrajac, B., Brodowicz, T.
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01.01.2014
• Subjects: Adult; Aged; Aged, 80 and over; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Clinical outcomes; Disease-Free Survival; Female; Humans; Male; Middle Aged; Nerve Sheath Neoplasms - drug therapy; Nerve Sheath Neoplasms - mortality; Nerve Sheath Neoplasms - pathology; Neurofibromatosis 1 - drug therapy; Neurofibromatosis 1 - mortality; Neurofibromatosis 1 - pathology; Oncology; Retrospective Studies; Short Communication; Tumors; Young Adult; Overall survival; Neurofibromatosis I; Malignant peripheral nerve sheath tumors
• ISSN: 0030-2414; 1423-0232
• DOI: 10.1159/000357137

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.