Review
Oncology
Ayesha Hassan, Roberto Carmagnani Pestana, Amanda Parkes
Summary: Malignant peripheral nerve sheath tumors (MPNSTs) are rare mesenchymal neoplasms that pose a therapeutic challenge due to lack of effective targeted therapy. Although standard chemotherapy regimens exist, there is a particular need for novel therapeutic strategies, especially for neurofibromatosis type 1 (NF1)-associated MPNST.
CURRENT TREATMENT OPTIONS IN ONCOLOGY
(2021)
Article
Otorhinolaryngology
Raven Dunn, Matthew J. Marget, Suhael Momin, Laura Garcia-Rodriguez
Summary: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare and aggressive subtype of sarcomas with neural origin. Head and neck manifestations are uncommon, presenting challenges in diagnosis and management. However, achieving local control with good aesthetic outcome can be possible through excision and radiation therapy.
AMERICAN JOURNAL OF OTOLARYNGOLOGY
(2021)
Article
Health Care Sciences & Services
Garrett Alewine, Jerrica Knight, Adithya Ghantae, Christina Mamrega, Bashnona Attiah, Robert A. Coover, Cale D. Fahrenholtz
Summary: Neurofibromatosis type 1 (NF1) is a common neurogenic disorder characterized by loss of function mutations in the neurofibromin gene. Recent research has found that nanomedicine, specifically silver nanoparticles (AgNPs), shows promise in the treatment of NF1-associated malignant peripheral nerve sheath tumors (MPNSTs). AgNPs exhibit selective cytotoxicity towards NF1-associated MPNSTs and this sensitivity is correlated with the expression levels of functional neurofibromin. The use of AgNPs in co-culture with Schwann cells can selectively eradicate NF1-associated MPNSTs at doses tolerable to normal cells.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Review
Biochemistry & Molecular Biology
Bandarigoda N. Somatilaka, Ali Sadek, Renee M. McKay, Lu Q. Le
Summary: This article provides an overview of the biology and genetics of malignant peripheral nerve sheath tumors (MPNSTs), discusses research findings regarding their developmental origin, summarizes the various model systems used to study MPNSTs, and discusses current management strategies and recent developments in translating basic research findings into potential therapies.
Article
Oncology
Nicolas Bachmann, Dominic Leiser, Alessia Pica, Barbara Bachtiary, Damien C. Weber
Summary: This study analyzed the outcomes of (m)PNST patients treated with proton therapy and PBS delivery paradigm. The results showed that PBSPT resulted in favorable oncologic outcomes and low toxicity rates in patients with (m)PNST.
FRONTIERS IN ONCOLOGY
(2022)
Article
Oncology
Yihui Gu, Wei Wang, Yuehua Li, Haibo Li, Zizhen Guo, Chengjiang Wei, Manmei Long, Manhon Chung, Rehanguli Aimaier, Qingfeng Li, Zhichao Wang
Summary: Malignant peripheral nerve sheath tumors (MPNSTs) are rare and difficult to treat soft-tissue sarcomas. This study evaluated different MEK inhibitors for the treatment of MPNSTs, and found that p-ERK can serve as a biomarker for predicting prognosis and an effective therapeutic target. Trametinib was identified as the most potent MEK inhibitor for the treatment of MPNSTs.
FRONTIERS IN ONCOLOGY
(2022)
Article
Surgery
Yan Zhang, Hongfei Cai, Guangchao Lv, Yang Li
Summary: The article reports a case of a female patient with neurofibroma on body surfaces and MPNST in the posterior mediastinum. Following surgical resection, there was no tumor relapse in the lungs, indicating the importance of timely surgery and detailed examination in improving tumor diagnostic accuracy.
Article
Multidisciplinary Sciences
Miriam Magallon-Lorenz, Ernest Terribas, Sara Ortega-Bertran, Edgar Creus-Bachiller, Marco Fernandez, Gerard Requena, Inma Rosas, Helena Mazuelas, Itziar Uriarte-Arrazola, Alex Negro, Tereza Lausova, Elisabeth Castellanos, Ignacio Blanco, George DeVries, Hiroyuki Kawashima, Eric Legius, Hilde Brems, Viktor Mautner, Lan Kluwe, Nancy Ratner, Margaret Wallace, Juana Fernandez-Rodriguez, Conxi Lazaro, Jonathan A. Fletcher, David Reuss, Meritxell Carrio, Bernat Gel, Eduard Serra
Summary: This article presents the genomic characterization of 9 widely used human MPNST cell lines, providing valuable resources for translational research. NF1-related cell lines exhibited characteristics similar to primary MPNSTs, while sporadic cell lines showed different inactivation patterns of tumor suppressor genes and mutations. The re-classification of cell lines as melanomas and other sarcomas demonstrated different responses to drug treatment. Deep genomic analysis, methylome-based classification, and cell-identity marker expression challenged the identity of common MPNST cell lines, highlighting the need for revisions in MPNST diagnosis.
Article
Medicine, General & Internal
Naim Abuzarifa, Muath Mamdouh Mahmod Al-Chalabi, Wan Azman Wan Sulaiman
Summary: Malignant peripheral nerve sheath tumors are rare, highly aggressive soft tissue sarcomas that arise from peripheral nerves or nerve sheath-related cells. This article reports a case of a Malay female patient with neurofibromatosis type I who presented with a massive, rapidly growing malignant peripheral nerve sheath tumor on the lower back.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2021)
Article
Oncology
Inka Ristow, Frederic Madesta, Lennart Well, Farzad Y. Shenas, Felicia Wright, Isabel Molwitz, Said Farschtschi, Peter Bannas, Gerhard Adam, Victor F. Mautner, Rene Werner, Johannes Salamon
Summary: This study demonstrates that machine-learning classification using MRI-based radiomics features allows sensitive and specific differentiation of benign peripheral nerve sheath tumors (BPNST) and malignant peripheral nerve sheath tumors (MPNST) in patients with neurofibromatosis type 1 (NF1). The feature expression of premalignant atypical tumors (ANF) falls between that of benign and malignant tumors, suggesting the biological plausibility of these radiomics features.
Review
Oncology
Jordan Jones, Sarah Cain, Jonathan Pesic-Smith, Peter F. M. Choong, Andrew P. Morokoff, Kate J. Drummond, Gabriel Dabscheck
Summary: The main cause of early death in NF1 patients is MPNST. Current methods for diagnosis and monitoring are inadequate, but ctDNA shows promise in improving patient care.
JOURNAL OF NEURO-ONCOLOGY
(2021)
Review
Oncology
Jun Liu, Jing-Ning Huang, Ming-Han Wang, Zhen-Yang Ni, Wei-Hao Jiang, Manhon Chung, Cheng-Jiang Wei, Zhi-Chao Wang
Summary: This review summarizes current studies on different imaging methods for differentiating benign and malignant tumors in NF1. Pathological evaluation is the gold standard for diagnosis, but the invasive nature restricts its application. Non-invasive methods like CT and MRI are commonly used for tumor evaluation in NF1 patients. However, there is no consensus on screening the malignant transformation of benign tumors, and novel technologies like radiogenomics and PET-MRI have not been fully adopted.
FRONTIERS IN ONCOLOGY
(2022)
Article
Pediatrics
Samantha W. E. Knight, Tristan E. Knight, Teresa Santiago, Andrew J. Murphy, Abdelhafeez H. Abdelhafeez
Summary: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with nerve sheath differentiation. They have a tendency to metastasize and are relatively common in individuals with neurofibromatosis type 1. The staging of MPNSTs is complex and requires multi-disciplinary collaboration. Surgical management is the primary approach, with non-surgical modalities playing a supportive role. Advances in molecular characterization have allowed the integration of targeted inhibitors into MPNST management.
Article
Oncology
Sumanth Nagabushan, Loretta M. S. Lau, Paulette Barahona, Marie Wong, Alexandra Sherstyuk, Glenn M. Marshall, Vanessa Tyrrell, Eva A. Wegner, Paul G. Ekert, Mark J. Cowley, Chelsea Mayoh, Toby N. Trahair, Philip Crowe, Antoinette Anazodo, David S. Ziegler
Summary: The prognosis of recurrent malignant peripheral nerve sheath tumors (MPNST) is poor and surgical resection remains the main treatment option. Chemoradiation approaches have not significantly improved outcomes, while therapies targeting genomic drivers of MPNST have been unsuccessful. MEK inhibitors show promise in preclinical studies but clinical efficacy remains unknown.
NPJ PRECISION ONCOLOGY
(2021)
Article
Oncology
Mohit R. Sharma, Ketul Sureshbhai Puj, Abhijeet A. Salunke, Shashank J. Pandya, Jahnavi S. Gandhi, Ankita R. Parikh
Summary: The study analyzed prognostic factors and treatment outcomes of 92 patients with MPNST, finding that the presence of NF1, tumor grade, and margin status were closely related to patient survival. For patients receiving curative-intent treatment, tumor grade and margin status significantly affected disease-free survival, while the presence of NF1 and tumor location had a significant impact on overall survival.
JOURNAL OF CANCER RESEARCH AND THERAPEUTICS
(2021)