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      Tumor maligno de la vaina nerviosa periférica mandibular. Caso clínico y revisión de la literatura Translated title: Malignant peripheral nerve sheath tumour of mandible. Case report and bibliographic review

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          Abstract

          RESUMEN El tumor maligno de la vaina nerviosa periférica (TMVNP) es un tumor mesenquimal raro que surge de las células de la vaina del nervio periférico. Tiene un comportamiento agresivo. Generalmente se presenta en extremidades y tronco, siendo su presencia en cabeza y cuello muy poco frecuente. Reportamos un caso raro de TMVNP del nervio dentario inferior en su trayecto intraóseo mandibular, no asociado a neurofibromatosis.

          Translated abstract

          ABSTRACT Malignant peripheral nerve sheath tumour (MPNST) is a rare mesenchymal tumor that arises from peripheral nerve sheath cells. It has aggressive behavior. It usually occurs in extremities and trunk, being its presence in head and neck very infrequent. We report a rare case of TMVNP of the inferior dental nerve in its mandibular intraosseous pathway, not associated with neurofibromatosis.

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          Malignant peripheral nerve sheath tumors of the head and neck: analysis of prognostic factors.

          Malignant peripheral nerve sheath tumors of the head and neck region are uncommon and may be associated with neurofibromatosis or occur in a sporadic manner. This is a retrospective review of 17 patients with these tumors who were treated at a single institution. Analysis of clinical and pathologic factors that influenced outcome was performed. There were 9 men and 8 women. Seven patients had a history of neurofibromatosis. Radiotherapy was implicated as a possible etiologic factor in 4 patients. The neck was the most frequently involved site. Overall survival at 5 years was 52%. Survival was improved for women and for patients with low-grade tumors. Age, tumor site, and size had no impact on survival. Survival was worse for patients with neurofibromatosis than for those with the sporadic form of the disease (P = 0.02). Survival was calculated by the method of Kaplan and Meier. The significance of such results was based on results of the log rank test. Local recurrence correlated with tumor size and resection margin status. No local recurrences occurred in those patients who had negative margins of resection and received adjuvant radiotherapy. Tumor grade was predictive of the development of distant metastases. Negative margins of resection are essential for obtaining local control, and the addition of adjuvant radiotherapy may be beneficial in this group. Salvage surgery for local recurrence is possible in some patients.
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            Postirradiation sarcoma of the head and neck.

            With improvement in survival after cancer treatment, it is becoming increasingly important to study treatment-related morbidity and mortality. Sarcoma can develop in the irradiated field after radiation therapy. The authors performed a study to estimate the risk, and compared the risk of sarcoma after radiation therapy with that of other treatment modalities used against cancer. Between 1955 and 1988, 229 patients with sarcoma of the head and neck were seen at the University of California, Los Angeles (UCLA), Medical Center. Of these, 13 (6%) had a previous history of radiation therapy to the head and neck. Radiation doses were known in 10 of 13 patients and ranged from 30 to 124.4 Gy. The latency time from radiation therapy to the development of postirradiation sarcoma (PIS) ranged from 3 months to 50 years, with a median of 12 years. More than 2000 patients have received radiation therapy to the head and neck for various conditions at the UCLA Medical Center since 1955. The authors conclude that most head and neck sarcomas are not radiation related and that the risk of PIS after head and neck irradiation for other diseases is low. From a review of the literature comparing mortality risks of chemotherapy, general surgery, and anesthesia, the risk of PIS appears no worse. Given the large number of patients who can be cured or receive palliation with radiation therapy, concern about PIS should not be a major factor influencing treatment decisions in patients with cancer.
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              Intraosseous Nerve Sheath Tumors in the Jaws

              Although the head and neck region is recognized as the most common location for peripheral nerve sheath tumors, central involvement, particularly in the jaw bones, is quite unusual. Neurofibroma is one of the most common nerve sheath tumors occurring in the soft tissue and generally appears in neurofibromatosis 1 (NF1 or von Recklinghausen's disease). Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in the soft tissue. Here, we report four cases of intraosseous peripheral nerve sheath tumors occurring in the jaw bones and compare the clinical, radiologic, and pathologic findings in order to make a differential diagnosis.
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                Author and article information

                Journal
                maxi
                Revista Española de Cirugía Oral y Maxilofacial
                Rev Esp Cirug Oral y Maxilofac
                Sociedad Española de Cirugía Oral y Maxilofacial y de Cabeza y Cuello (Madrid, Madrid, Spain )
                1130-0558
                2173-9161
                September 2020
                : 42
                : 3
                : 132-135
                Affiliations
                [2] Madrid orgnameHospital Universitario 12 de Octubre orgdiv1Servicio de Anatomía Patológica España
                [1] Madrid orgnameHospital Universitario 12 de Octubre orgdiv1Servicio de Cirugía Oral y Maxilofacial España
                Article
                S1130-05582020000300006 S1130-0558(20)04200300006
                10.20986/recom.2020.1106/2019
                d3439d0c-77c7-4f94-a600-40ac99ee9b1c

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 15 April 2020
                : 09 December 2019
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 9, Pages: 4
                Product

                SciELO Spain

                Categories
                Casos Clínicos

                mandíbula,neurofibromatosis,Schwannoma,Schwannoma neurofibromatosis,Malignant peripheral nerve sheath tumor,inmunohistoquímica,Tumor maligno de vainas de nervio periférico,jaw,immunohistochemistry

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