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Laryngeal malignant peripheral nerve sheath tumor mixed with high- and low-grade malignancies. | LitMetric

AI Article Synopsis

  • Malignant peripheral nerve sheath tumors (MPNSTs) vary in severity and can be mistaken for other nerve tumors based on their cellular appearance in histological analysis.
  • A 57-year-old male patient presented with a recurrent tumor in the larynx, leading to a surgical evaluation that revealed both high-grade and low-grade MPNST lesions in the affected tissue.
  • The study highlights the importance of detailed immunohistochemical examination in accurately diagnosing and treating these tumors to improve patient outcomes, especially in preserving vocal function.

Article Abstract

Malignant peripheral nerve sheath tumors (MPNSTs), as defined by immunohistochemical evaluation, are identified along a spectrum ranging from atypical neurofibroma to high-grade MPNST because these tumors are similar in terms of cell shape and tissue components on hematoxylin-eosin (HE) staining. The patient was a 57-year-old male referred to our hospital, with a recurrent red tumor at the anterior commissure of the larynx and submucosal swelling of the right vocal fold. A surgical specimen from a right horizontal partial laryngectomy was evaluated immunohistochemically. A high-grade MPNST lesion was included in the submucosal white tumor, whereas a low-grade MPNST lesion was encountered around the high-grade MPNST lesion. This tumor may involve different malignancies even when it is small. Although intra-tumor heterogeneity in cancers has been reported recently, careful immunohistochemical examination can be important and beneficial for eradicating the tumor while preserving vocal function.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8407030PMC
http://dx.doi.org/10.1093/jscr/rjab373DOI Listing

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Article Synopsis
  • The study investigated the expression of VEGFRs, PDGFRs, and c-Kit in pediatric patients with high-grade non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) to understand their impact on growth, invasion, and treatment outcomes.
  • Out of 113 pediatric patients analyzed, about one-third showed high expression of PDGFRα, while other markers had lower expression rates; high PDGFRα and VEGFR2 were notably associated with poorer survival rates.
  • The results suggest that these tyrosine kinase receptors are upregulated in NRSTS, indicating a potential for targeted therapies to improve outcomes in affected patients.
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With no more than two dozen cases reported in the literature, malignant peripheral nerve sheath tumor (MPNST) is a rare primary mesenchymal neoplasm arising in the female genital tract. Most cases occurred in middle-aged adults with high grade histology, unfavorable clinical outcome, and no history of neurofibromatosis type 1. Its extreme rarity in this site no doubt poses a diagnostic challenge during routine clinical practice.

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A Rare Case Report of Malignant Peripheral Nerve Sheath Tumor in the Infratemporal Fossa.

Ear Nose Throat J

October 2024

Department of Otolaryngology-Head and Neck Surgery, Al-Mouwasat University Hospital, Faculty of Medicine, Damascus University, Damascus, Syria.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors that develop from peripheral nerve sheath cells and they account for approximately 5% to 10% of all soft-tissue sarcomas. MPNSTs in the head and neck region represent approximately 2% to 6% of all head and neck sarcomas and account for 12% to 19% of all MPNSTs, and the infratemporal fossa is a rare site for MPNSTs. MPNSTs originating from the trigeminal nerve are extremely rare.

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Objective: To investigate the clinical features, treatment methods, and prognostic influence factors of patients with malignant peripheral nerve sheath tumor (MPNST).

Methods: A retrospective analysis was conducted on 96 MPNST patients treated between January 1, 2015 and December 31, 2021. There were 46 males and 50 females, aged between 15 and 87 years (mean, 48.

View Article and Find Full Text PDF

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