scholarly journals A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
José Alcides Arruda ◽  
Pamella Álvares ◽  
Luciano Silva ◽  
Alexandrino Pereira dos Santos Neto ◽  
Cleomar Donizeth Rodrigues ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Malignant Neoplasm ◽  
Surgical Approaches ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Malignant Soft Tissue Tumor

Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed.

scholarly journals Peranan Radiologi dan Radioterapi pada Penatalaksanaan Malignant Peripheral Nerve Sheath Tumor

2019 ◽  
Vol 3 (2) ◽  
pp. 66-73
Author(s):  
Wigati Dhamiyati ◽  
Sri Retna Dwidanarti ◽  
Nurmalia Nurmalia
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Histopathological Examination ◽  
Soft Tissue Sarcomas ◽  
Surgical Excision ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Complete Surgical Excision ◽  
Imaging Characteristics

Malignant peripheral nerve sheath tumor (MPNST) is biologically an aggressive tumor of soft tissue sarcoma. MPNSTs represent 5–10% of all soft-tissue sarcomas. They correspond to malignant forms of neurofibromas and schwannomas. The main clinical symptoms of MPNST are increasing size of tumors, local or radicular pain, paraparesis, and paresthesia and/or weakness of extremities.Radiological imaging is important to determine the site and extension of the tumor, especially before surgery. Magnetic resonance imaging (MRI) is the imaging modality of choice. To some extent, MPNSTs share basic imaging characteristics with benign peripheral nerve sheath tumor, although there are some evidence of malignant transformation. Diagnosis may be challenging because there are no specific immunohistochemical or molecular markers. However, Histopathological examination is needed for definitive diagnosisThe mainstay of treatment is surgical resection. The goal of the operation is to achieve complete surgical excision of the tumor with negative (wide) margins. Together with wide surgical excision, radiation therapy offers the best outcome of local and overall survival rates.

scholarly journals From Genes to -Omics: The Evolving Molecular Landscape of Malignant Peripheral Nerve Sheath Tumor

Genes ◽  
2020 ◽  
Vol 11 (6) ◽  
pp. 691
Author(s):  
Kathryn M. Lemberg ◽  
Jiawan Wang ◽  
Christine A. Pratilas
Keyword(s):  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Genetic Changes ◽  
Genetic Syndrome ◽  
Genetic Sequencing ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Molecular Landscape

Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive soft tissue sarcomas that occur with significantly increased incidence in people with the neuro-genetic syndrome neurofibromatosis type I (NF1). These complex karyotype sarcomas are often difficult to resect completely due to the involvement of neurovascular bundles, and are relatively chemotherapy- and radiation-insensitive. The lifetime risk of developing MPNST in the NF1 population has led to great efforts to characterize the genetic changes that drive the development of these tumors and identify mutations that may be used for diagnostic or therapeutic purposes. Advancements in genetic sequencing and genomic technologies have greatly enhanced researchers’ abilities to broadly and deeply investigate aberrations in human MPNST genomes. Here, we review genetic sequencing efforts in human MPNST samples over the past three decades. Particularly for NF1-associated MPNST, these overall sequencing efforts have converged on a set of four common genetic changes that occur in most MPNST, including mutations in neurofibromin 1 (NF1), CDKN2A, TP53, and members of the polycomb repressor complex 2 (PRC2). However, broader genomic studies have also identified recurrent but less prevalent genetic variants in human MPNST that also contribute to the molecular landscape of MPNST and may inform further research. Future studies to further define the molecular landscape of human MPNST should focus on collaborative efforts across multiple institutions in order to maximize information gathered from large numbers of well-annotated MPNST patient samples, both in the NF1 and the sporadic MPNST populations.

scholarly journals Case report of malignant peripheral nerve sheath tumor of parotid in a patient with neurofibromatosis type 1

2021 ◽  
Vol 7 (12) ◽  
pp. 1927
Author(s):  
Anuradha P. ◽  
Bathi Reddy ◽  
Suhail Gaur
Keyword(s):  
Parotid Gland ◽  
Peripheral Nerve ◽  
Protein A ◽  
Soft Tissue Sarcomas ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Parotid Swelling ◽  
The Right

<p>Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, accounts for &lt;5% of all soft tissue sarcomas, and carries a poor prognosis. Diagnosis of MPNSTs is usually based on histopathology aided by IHC, which reflects the Schwann cell differentiation in this neoplasm. Approximately 50-90% of MPNSTs are positive for S-100 protein. A 56-year-old male presented with right parotid swelling for 3 months and incomplete right eye closure. The patient was diagnosed with neurofibromatosis 1 (NF-1) and pulmonary tuberculosis in the past. On Examination, a hard, tender swelling measuring 5×4 cm involving the right parotid gland and right grade 2 LMN facial palsy was noted. Histopathology was suggestive of a MPNST.</p><p><strong> </strong></p>

Sign in / Sign up

Export Citation Format

Share Document