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
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
Sarah Attia, Mina Guirguis, Lu Q. Le, Avneesh Chhabra
Summary: This study suggests that there is no predisposition for pNFs and dNFs to develop into MPNSTs in NF1 patients. Therefore, these lesions may not require special surveillance compared to discrete peripheral nerve sheath tumors.
SKELETAL RADIOLOGY
(2023)
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
Clinical Neurology
Hiroshi Koike, Yoshihiro Nishida, Shinji Ito, Yoshie Shimoyama, Kunihiro Ikuta, Hiroshi Urakawa, Tomohisa Sakai, Koki Shimizu, Kan Ito, Shiro Imagama
Summary: There are significant differences between MPNST and pNF in terms of tumor size, peripheral enhancement pattern, perilesional edema-like zone, and intratumoral cystic change. The ADC values were significantly lower in MPNST compared to pNF, and setting a cutoff value of mean ADC at 1.85 x 10(-)(3) mm(2)/s provided sensitivity of 80% and specificity of 74%. Adding ADC evaluation to standard MRI evaluation improved the diagnostic accuracy.
WORLD NEUROSURGERY
(2022)
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
Oncology
Michael Zhang, Elizabeth Tong, Sam Wong, Forrest Hamrick, Maryam Mohammadzadeh, Vaishnavi Rao, Courtney Pendleton, Brandon W. Smith, Nicholas F. Hug, Sandip Biswal, Jayne Seekins, Sandy Napel, Robert J. Spinner, Mark A. Mahan, Kristen W. Yeom, Thomas J. Wilson
Summary: Machine learning approaches using radiomics features extracted from MRI images, in combination with clinical features, successfully differentiated peripheral schwannomas from neurofibromas. The radiomics-based classifiers outperformed human expert evaluators in terms of accuracy and AUC on the ROC curve.
Article
Medicine, General & Internal
Matthias Holzbauer, Kathrin Aufschnaiter-Hiessboeck, Maximilian Zaussinger, Oskar C. Aszmann, Manfred Schmidt
Summary: The study identified four common morphological patterns of bPNST relative to nerve fascicles, reflecting the complexity of tumor removal and providing assistance for preoperative diagnostics and surgical planning.
JOURNAL OF CLINICAL MEDICINE
(2022)
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
Behavioral Sciences
Luiz Guilherme Darrigo Junior, Victor Evangelista de Faria Ferraz, Marina Candido Visontai Cormedi, Luissa Hikari Hayashi Araujo, Mariana Prado Silva Magalhaes, Rafaella Curis Carneiro, Luis Henrique Nunes Sales, Mendel Suchmacher, Karin Soares Cunha, Aguinaldo Bonalumi Filho, David Rubem Azulay, Mauro Geller
Summary: Neurofibromatosis type 1 (NF1) is a common genetic and sporadic disease, and plexiform neurofibroma (PN) is a significant complication. This study retrospectively analyzed 491 NF1 patients in two reference centers in Brazil, and found that approximately 38% of patients had PN, mainly affecting the head and neck and extremities. The study also found that PN can significantly reduce patients' life quality and some patients may develop malignant peripheral nerve sheath tumors.
BRAIN AND BEHAVIOR
(2022)
Review
Oncology
Alessia Pellerino, Robert M. Verdijk, Lucia Nichelli, Nicolaus H. Andratschke, Ahmed Idbaih, Roland Goldbrunner
Summary: The 2021 WHO classification of the CNS Tumors recognizes Peripheral nerve sheath tumors (PNST) as a distinct group with specific characteristics and recommends diagnostic and therapeutic approaches based on limited evidence due to their rarity. Diagnosis is mainly determined by histological analysis and immunohistochemistry, with genetic analysis required for mosaic forms of NF1 and SPS. MRI is the preferred method for assessing tumor extent. Complete resection is the preferred treatment, balancing nerve preservation. Radiotherapy can be omitted in benign and NF-related tumors. Systemic therapy is recommended for incompletely resected plexiform neurofibromas/MPNSTs, with MEK inhibitors and anthracycline-based treatment being potential options. Clinical trials on MEK1-2 inhibitors and mTOR inhibitors are underway for plexiform neurofibromas and MPNST, respectively.
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)
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
Medicine, General & Internal
Min Dai, Ting Wang, Jun-Ming Wang, Li-Ping Fang, Ying Zhao, Asmitananda Thakur, Dong Wang
Summary: Different pathological types of orbital peripheral nerve sheath tumors have unique imaging characteristics. Comprehensive consideration of the patient's clinical and imaging manifestations is of great value in the diagnosis of orbital peripheral nerve sheath tumors.
WORLD JOURNAL OF CLINICAL CASES
(2022)
Review
Oncology
Omar Rabab'h, Abeer Gharaibeh, Ali Al-Ramadan, Manar Ismail, Jawad Shah
Summary: Neurofibromatosis type 1 is a common cancer predisposition genetic disease that affects the nervous system pathways and is associated with significant morbidity and mortality. Despite advancements in targeted pharmacological interventions, the underlying pathogenesis of NF1 is still not fully understood, and treatment options remain limited.
