Article
Clinical Neurology
Megan C. Everson, Courtney Pendleton, Megan M. Jack, Brandon W. Smith, Jodi M. Carter, Robert J. Spinner
Summary: Malignant perineurioma is a rare subset of MPNST with distinct clinical features. Patients in the study had uncomplicated clinical courses post-diagnosis, but further research is needed to fully understand the clinical course of these rare tumors.
WORLD NEUROSURGERY
(2021)
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
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)
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
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
Multidisciplinary Sciences
Jody Fromm Longo, Stephanie N. Brosius, Iya Znoyko, Victoria A. Alers, Dorea P. Jenkins, Robert C. Wilson, Andrew J. Carroll, Daynna J. Wolff, Kevin A. Roth, Steven L. Carro
Summary: This article introduces a new sporadic MPNST cell line, 2XSB, with molecular and genomic features similar to the parent tumor, providing a useful tool for investigating the biology and potential treatment regimens for sporadic MPNSTs. The cells display invasive capabilities and form solid tumors when xenografted into immunodeficient mice, and genomic analyses reveal mutations in key genes implicated in MPNST pathogenesis.
SCIENTIFIC REPORTS
(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)
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
Medicine, General & Internal
Gaetano Magro, Giuseppe Broggi, Giuseppe Angelico, Lidia Puzzo, Giada Maria Vecchio, Valentina Virzi, Lucia Salvatorelli, Martino Ruggieri
Summary: Peripheral nerve sheath tumors include a range of lesions with different biological behavior, such as neurofibromas, schwannomas, and perineuriomas. These tumors can occur as isolated lesions or in association with other neurofibromatosis or schwannomatosis. Malignant peripheral nerve sheath tumors are soft tissue sarcomas that may arise from a peripheral nerve or pre-existing neurofibromas.
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
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.
Article
Orthopedics
Patrick Debs, Rodrigo Luna, Laura M. Fayad, Shivani Ahlawat
Summary: This study compared the MRI features of sporadic and neurofibromatosis syndrome-related localized schwannomas and neurofibromas. The results showed that signal heterogeneity and the presence of multiple lesions along a nerve are differentiating characteristics of syndrome-related tumors.
SKELETAL RADIOLOGY
(2023)
Article
Oncology
Yoshihiro Nishida, Hiroshi Urakawa, Robert Nakayama, Eisuke Kobayashi, Toshifumi Ozaki, Keisuke Ae, Yoshihiro Matsumoto, Hiroyuki Tsuchiya, Takahiro Goto, Hiroaki Hiraga, Norifumi Naka, Shunji Takahashi, Yuichi Ando, Masahiko Ando, Yachiyo Kuwatsuka, Shunsuke Hamada, Takafumi Ueda, Akira Kawai
Summary: The study examined the efficacy of pazopanib against MPNST, demonstrating favorable clinical benefit, progression-free survival, and overall survival rates. Patients with higher FNCLCC grades showed better outcomes with pazopanib treatment.
INTERNATIONAL JOURNAL OF CANCER
(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
Acoustics
Zhenzhen Jin, Kaiping Zhao, Wen Guo, Dandan Wang, Yukun Deng, Tao Chen
Summary: This study aimed to differentiate malignant peripheral nerve sheath tumors (MPNSTs) from benign peripheral nerve sheath tumors (BPNSTs) using sonography. Key features were identified, and a scoring system was established. The results demonstrated the effectiveness of sonography in differentiating between MPNSTs and BPNSTs.
JOURNAL OF ULTRASOUND IN MEDICINE
(2022)
Article
Pathology
Narasimhan P. Agaram, Yun-Shao Sung, Lei Zhang, Chun-Liang Chen, Hsiao-Wei Chen, Samuel Singer, Mark A. Dickson, Michael F. Berger, Cristina R. Antonescu
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
(2015)
Article
Oncology
Murray Brennan
ANNALS OF SURGICAL ONCOLOGY
(2015)
Article
Oncology
Kaitlyn J. Kelly, Luke Selby, Joanne F. Chou, Katerina Dukleska, Marinela Capanu, Daniel G. Coit, Murray F. Brennan, Vivian E. Strong
ANNALS OF SURGICAL ONCOLOGY
(2015)
Meeting Abstract
Oncology
R. Sullivan, I. A. Olusegun, B. O. Anderson, R. Audisio, P. Autier, A. Aggarwal, C. Balch, M. Brennan, A. Dare, A. D'Cruz, A. Eggermont, K. Fleming, L. Hagander, C. A. Herrera, A. M. Ilbawi, J. F. Ji, T. P. Kingham, J. Liberman, A. J. M. Leather, J. G. Meara, S. S. Murthy, S. Omar, G. P. Parham, C. S. Pramesh, R. Riviello, D. Rodin, L. Santini, S. V. Shrikhande, M. Shrime, R. Thomas, A. T. Tsunoda, D. Watters, S. Wang, Y. L. Wu, F. Van de Velde, U. Veronesi, M. Zeiton, A. Purushotham
EUROPEAN JOURNAL OF CANCER
(2015)
Article
Oncology
Aimee M. Crago, Juliann Chmielecki, Mara Rosenberg, Rachael O'Connor, Caitlin Byrne, Fatima G. Wilder, Katherine Thorn, Phaedra Agius, Deborah Kuk, Nicholas D. Socci, Li-Xuan Qin, Matthew Meyerson, Meera Hameed, Samuel Singer
GENES CHROMOSOMES & CANCER
(2015)
Article
Oncology
Narasimhan P. Agaram, Lei Zhang, Francois LeLoarer, Tarik Silk, Yun-Shao Sung, Sasinya N. Scott, Deborah Kuk, Li-Xuan Qin, Michael F. Berger, Cristina R. Antonescu, Samuel Singer
GENES CHROMOSOMES & CANCER
(2016)
Article
Oncology
Rodrigo R. Munhoz, Sandra P. D'Angelo, Mrinal M. Gounder, Mary L. Keohan, Ping Chi, Richard D. Carvajal, Samuel Singer, Aimee M. Crago, Jonathan Landa, John H. Healey, Li-Xuan Qin, Meera Hameed, Marietta O. Ezeoke, Arun S. Singh, Mark Agulnik, Bartosz Chmielowski, Jason J. Luke, Brian A. Van Tine, Gary K. Schwartz, William D. Tap, Mark A. Dickson
Article
Pathology
Shih-Chiang Huang, Lei Zhang, Yun-Shao Sung, Chun-Liang Chen, Yu-Chien Kao, Narasimhan P. Agaram, Samuel Singer, William D. Tap, Sandra D'Angelo, Cristina R. Antonescu
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
(2016)
Article
Surgery
Sam C. Wang, Joanne F. Chou, Vivian E. Strong, Murray F. Brennan, Marinela Capanu, Daniel G. Coit
Article
Surgery
Marcus C. B. Tan, Murray F. Brennan, Deborah Kuk, Narasimhan P. Agaram, Cristina R. Antonescu, Li-Xuan Qin, Nicole Moraco, Aimee M. Crago, Samuel Singer
Article
Oncology
Ann Y. Lee, Narasimhan P. Agaram, Li-Xuan Qin, Deborah Kuk, Christina Curtin, Murray F. Brennan, Samuel Singer
ANNALS OF SURGICAL ONCOLOGY
(2016)
Article
Multidisciplinary Sciences
Li-Xuan Qin, Thomas Tuschl, Samuel Singer
SCIENTIFIC REPORTS
(2016)
Meeting Abstract
Oncology
L. Selby, G. C. Schnorr, S. B. Solomon, M. A. Schattner, M. F. Brennan, D. G. Coit, P. B. Bach, E. B. Elkin, V. E. Strong
ANNALS OF SURGICAL ONCOLOGY
(2015)