Clinical Signs of Neurofibromatosis Impact on the Outcome of Malignant Peripheral Nerve Sheath Tumors.
| Autor: | Lamm, W., Schur, S., Köstler, W.J., Funovics, P., Windhager, R., Amann, G., Panotopoulos, J., Pokrajac, B., Brodowicz, T. |
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| Předmět: |
ACADEMIC medical centers
CONFIDENCE intervals MEDICAL records METASTASIS NERVOUS system tumors SARCOMA SURVIVAL TUMOR classification RETROSPECTIVE studies SEVERITY of illness index DATA analysis software DESCRIPTIVE statistics KAPLAN-Meier estimator DISEASE complications NEUROFIBROMATOSIS 1 SYMPTOMS DIAGNOSIS |
| Zdroj: | Oncology; Feb2014, Vol. 86 Issue 2, p122-126, 5p, 3 Charts |
| Abstrakt: | 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. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR] |
| Databáze: | Complementary Index |
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