Peripheral Nerve Considerations in the Management of Extremity Soft Tissue Sarcomas

2009 ◽  
pp. 243-256 ◽  
Author(s):  
Peter C. Ferguson ◽  
Anna A. Kulidjian ◽  
Kevin B. Jones ◽  
Benjamin M. Deheshi ◽  
Jay S. Wunder
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Soft Tissue Sarcomas

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.

Malignant Peripheral Nerve Sheath Tumors

2018 ◽  
Author(s):  
Marie-Noëlle Hébert-Blouin
Keyword(s):  
Decision Making ◽  
Soft Tissue ◽  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Adjuvant Therapies ◽  
Nerve Sheath ◽  
Schwann Cell Differentiation

Malignant peripheral nerve sheath tumors (PNSTs) are soft tissue sarcomas arising from a peripheral nerve or a pre-existing benign nerve sheath tumor or are sarcomas with features of Schwann-cell differentiation. Differentiating between benign and malignant PNSTs can be challenging. The chapter begins with a case example and then discusses assessment, investigations (including imaging), and diagnosis of malignant PNSTs, as well as the steps involved in decision-making about management of a malignant PNST. The surgical principles and goals for resection of a malignant PNST, the adjuvant therapies used in treatment, and the complications and outcomes of treatment are presented.

scholarly journals Surgical management of malignant peripheral nerve sheath tumors

2007 ◽  
Vol 22 (6) ◽  
pp. 1-8 ◽  
Author(s):  
John Gachiani ◽  
Daniel Kim ◽  
Adriane Nelson ◽  
David Kline
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Surgical Techniques ◽  
Soft Tissue Sarcomas ◽  
Neurofibromatosis Type ◽  
Surgical Removal ◽  
Complete Excision ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Object The aim of this study was to describe the presentation of patients harboring soft tissue sarcomas involving the nerves, most of which were malignant peripheral nerve sheath tumors (MPNSTs), and provide an algorithm for their treatment. Methods The authors retrospectively analyzed data on 43 surgically treated soft tissue sarcomas involving the nerves, 34 (79%) of which were MPNSTs. Tumor classifications are presented, together with patient numbers, locations of MPNSTs, surgical techniques, and adjunctive treatments. Results The 34 MPNSTs were surgically treated during a period of 40 years. Most of these lesions (19 MPNSTs [56%]) were located in the brachial plexus, whereas the rest were located on other major nerves. Neurofibromatosis Type 1–associated tumors (12 lesions) represented 35% of the total number of MPNSTs. Although the main goal of surgery was complete excision, it was successful in only 16 patients (47%). Of the available records, 18 patients (53%) died due to disease progression. Conclusions Malignant peripheral nerve sheath tumors are rare. Surgical removal with margins when possible coupled by adjuvant radiotherapy offers the best chance of survival. The role of chemotherapy is still being defined.

p53 and Ki-67 Proliferating Cell Nuclear Antigen in Benign and Malignant Peripheral Nerve Sheath Tumors in Children

1999 ◽  
Vol 2 (4) ◽  
pp. 377-384 ◽  
Author(s):  
Helen Liapis ◽  
Edith F. Marley ◽  
Yuan Lin ◽  
Louis P. Dehner
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Plexiform Neurofibroma ◽  
Soft Tissue Sarcomas ◽  
Nuclear Antigen ◽  
Ki 67 ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Pathologic Features

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon soft tissue tumors. In children with neurofibromatosis 1 (NF1), a MPNST often arises in a preexisting neurofibroma, or may represent an initial manifestation without other obvious stigmata of the disease. The development of MPNSTs may be associated with instability of the p53 tumor suppressor gene since it is the most frequent genetic abnormality in soft tissue sarcomas. To assess the presence of p53 accumulation in MPNSTs and its correlation with clinical and pathologic features, we studied 12 neurofibromas (NFs), including 4 tumors with cellular features (one congenital) and 10 MPNSTs. Six MPNSTs were associated with NF1, all of which developed within a plexiform neurofibroma. Cell proliferation evaluated with an antibody to Ki-67 and nuclear p53 staining were both detected by immunohistochemistry We found p53 positivity in 60% of MPNSTs. All NFs except the congenital tumor were p53 immunonegative ( P < 0.01). Rare p53-positive nuclei were detected in the transitional zone in two of six MPNSTs arising in plexiform NFs. Ki-67 distinguished the NFs from MPNSTs ( P < 0.005). Half of the NF1 patients with p53-positive MPNSTs developed recurrence or metastases or developed a second malignancy within 2 years of diagnosis, whereas patients with p53-positive sporadic MPNSTs were free of disease 1 to 7 years later. We found p53 accumulation more frequently in NF1-associated MPNSTs. p53 mutations may be an additional biologic factor to account for the poor prognosis in these tumors.

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.

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