Hybrid Benign Peripheral Nerve Sheath Tumors: A Comprehensive Literature Review with Emphasis on Their Clinical, Morphological and Genetic Features

Hybrid Peripheral Nerve Sheath Tumors (HPNSTs) are rare benign neoplasms that exhibit a combination of histological features from multiple types of benign peripheral nerve sheath tumors, including schwannomas, neurofibromas, and perineuriomas. These tumors present a diagnostic challenge due to their...

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Main Authors: Serena Salzano, Rosario Caltabiano, Magda Zanelli, Andrea Palicelli, Maurizio Zizzo, Nektarios Koufopoulos, Ioannis Boutas, Gaetano Magro, Valeria Barresi, Giuseppe Broggi
Format: Article
Language:English
Published: MDPI AG 2025-03-01
Series:Diagnostics
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Online Access:https://www.mdpi.com/2075-4418/15/7/855
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Summary:Hybrid Peripheral Nerve Sheath Tumors (HPNSTs) are rare benign neoplasms that exhibit a combination of histological features from multiple types of benign peripheral nerve sheath tumors, including schwannomas, neurofibromas, and perineuriomas. These tumors present a diagnostic challenge due to their morphological and histological variability. In this article, we aim to summarize the key morphological, histological, and molecular characteristics of HPNSTs, providing insights into their diagnostic approaches. We review the different hybrid subtypes, including schwannoma–perineurioma, schwannoma–neurofibroma, and perineurioma–neurofibroma, emphasizing their clinical features, genetic associations, and the role of surgical excision as the gold-standard treatment.
ISSN:2075-4418