Histopathology and clinical outcome of NF1-associated vs. sporadic malignant peripheral nerve sheath tumors

Journal of Neuro-Oncology - Tập 82 - Trang 187-192 - 2006
Christian Hagel1, Ulrich Zils2, Matthias Peiper3, Lan Kluwe4, Stefan Gotthard5, Reinhard E. Friedrich5, David Zurakowski6, Andreas von Deimling2, Victor Felix Mautner4
1Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
2Institute of Neuropathology, Charité-Universitätsmedizin, Berlin, Germany
3Klinik für Allgemein- und Viszeralchirurgie, Universitätsklinikum Düsseldorf, Düsseldorf, Germany
4Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
5Department of Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
6Department of Biostatistics, Children’s Hospital, Boston, USA

Tóm tắt

The differences in the clinical course and histopathology of sporadic and neurofibromatosis type 1 (NF1)-associated malignant peripheral nerve sheath tumors (MPNST) were investigated retrospectively. The collective comprised 38 NF1 patients and 14 sporadic patients. NF1 patients were significantly younger at diagnosis (p < 0.001) and had a significantly shorter survival time than sporadic patients (median survival 17 months vs. 42 months, Breslow p < 0.05). The time interval to local recurrence and metastatic spread was also significantly shorter in NF1 patients (9.4 months vs. 30.0 months, p < 0.01; 9.1 months vs. 33.2 months, p < 0.001, respectively). In patients with the original histopathological data available (22 NF1 patients, 14 sporadic cases), NF1-associated MPNST showed a significantly higher cellularity compared to sporadic tumors (p < 0.001) whereas sporadic MPNST featured a significantly higher pleomorphism (p< 0.01). Most importantly, while histopathological variables correlated with French Fédération Nationale des Centres de Lutte Contre le Cancer grading in sporadic MPNST, this was not the case for NF1-associated tumors. The differences between NF1-associated and sporadic MPNST in regard to the clinical course and histopathology may reflect some fundamental differences in biology and pathomechanism of the two tumor groups. Our findings indicate the necessity for a separate grading scheme which takes into account the genetic background in NF1 patients.

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Thông tin xuất bản

Journal of Neuro-Oncology

Tập 82

187-192

Thông tin tác giả