Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1
Matthias Kolberg,Maren Høland,Trude H. Ågesen,Helge R. Brekke,Knut Liestøl,Kirsten Sundby Hall,Fredrik Mertens,Piero Picci,Sigbjørn Smeland,Ragnhild A. Lothe +9 more
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It is suggested that the choice of treatment for MPNST should be independent of NF1 status, as in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis.Abstract:
There are conflicting reports as to whether malignant peripheral nerve sheath tumor (MPNST) patients with neurofibromatosis type 1 (NF1) have worse prognosis than non-NF1 MPNST patients. Large clinical studies to address this problem are lacking due to the rareness of MPNST. We have performed meta-analyses testing the effect of NF1 status on MPNST survival based on publications from the last 50 years, including only nonoverlapping patients reported from each institution. In addition, we analyzed survival characteristics for 179 MPNST patients from 3 European sarcoma centers. The meta-analyses including data from a total of 48 studies and >1800 patients revealed a significantly higher odds ratio for overall survival (OR(OS)) and disease-specific survival (OR(DSS)) in the non-NF1 group (OR(OS) = 1.75, 95% confidence interval [CI] = 1.28-2.39, and OR(DSS) = 1.68, 95% CI = 1.18-2.40). However, in studies published in the last decade, survival in the 2 patient groups has been converging, as especially the NF1 group has shown improved prognosis. For our own MPNST patients, NF1 status had no effect on overall or disease-specific survival. The compiled literature from 1963 to the present indicates a significantly worse outcome of MPNST in patients with NF1 syndrome compared with non-NF1 patients. However, survival for the NF1 patients has improved in the last decade, and the survival difference is diminishing. These observations support the hypothesis that MPNSTs arising in NF1 and non-NF1 patients are not different per se. Consequently, we suggest that the choice of treatment for MPNST should be independent of NF1 status.read more
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A RASopathy gene commonly mutated in cancer: the neurofibromatosis type 1 tumour suppressor
Nancy Ratner,Shyra J. Miller +1 more
TL;DR: Current knowledge of NF1 disease, the NF1 gene and neurof fibromin, neurofibromin signalling pathways and recent developments in NF1 therapeutics are summarized.
Journal ArticleDOI
Malignant Peripheral Nerve Sheath Tumors
Mohamad Farid,Elizabeth G. Demicco,Roberto A. Garcia,Linda Ahn,Pamela R. Merola,Angela Cioffi,Robert G. Maki +6 more
TL;DR: The current understanding of MPNST biology, current best practices of management, and recent research developments in this disease are reviewed, with a view to informing future advancements in patient care.
Journal ArticleDOI
Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1-a consensus overview.
Markku Miettinen,Cristina R. Antonescu,Christopher D.M. Fletcher,AeRang Kim,Alexander J. Lazar,Martha Quezado,Karlyne M. Reilly,Anat Stemmer-Rachamimov,Douglas R. Stewart,David Viskochil,Brigitte C. Widemann,Arie Perry +11 more
TL;DR: The term "atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP)" is proposed for lesions displaying at least 2 of the histopathologic features involved in the malignant transformation of neurof fibromas, and should prompt additional sampling, clinical correlation, and possibly, expert pathology consultation.
Journal ArticleDOI
Gene for von Recklinghausen neurofibromatosis is in the pericentromeric region of chromosome 17
Journal ArticleDOI
Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas.
Melike Pekmezci,David E. Reuss,Angela C. Hirbe,Sonika Dahiya,David H. Gutmann,Andreas von Deimling,Andrew E. Horvai,Arie Perry +7 more
TL;DR: Combinations of these histopathological and immunohistochemical features provide useful criteria to distinguish between malignant peripheral nerve sheath tumor and cellular schwannoma with high sensitivity and specificity.
References
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Book
Enzinger and Weiss's Soft Tissue Tumors
Sharon W. Weiss,John R. Goldblum +1 more
TL;DR: The Enzinger and Weiss's soft tissue tumors are described, a large number of which consist of "hard-to-reach granuloma-like deposits".
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TL;DR: The fifth volume in the new WHO series on histological and genetic typing of human tumours is "Pathology and Genetics of Tumours of Soft Tissue and Bone" as discussed by the authors.
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Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases.
Barbara S. Ducatman,Bernd W. Scheithauer,David G. Piepgras,Herbert M. Reiman,Duane M. Ilstrup +4 more
TL;DR: The MPNST is an aggressive uncommon neoplasm, and large tumor size, the presence of neurofibromatosis, and total resection are the most important prognostic indicators.
Journal ArticleDOI
Malignant peripheral nerve sheath tumours in neurofibromatosis 1
TL;DR: The lifetime risk of MPNST in NF1 is much higher than previously estimated and warrants careful surveillance and a low threshold for investigation.
Journal ArticleDOI
Enzinger and Weiss’s Soft Tissue Tumors. 4th ed.
TL;DR: This unique tome on soft-tissue tumors is the fourth edition of Enzinger and Weiss’s original text and includes contributions by ten additional pathologists and clinicians.