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      Intraosseous Nerve Sheath Tumors in the Jaws

      case-report

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          Abstract

          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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          Bone cancers.

          H D Dorfman, B Czerniak (1995)
          Frequency distribution data for primary bone sarcomas have long been used to provide clues to the diagnosis of bone cancers after their identification in radiographs. Age and skeletal site are often helpful, in addition to specific radiographic features, in narrowing down the probable histologic categories of bone neoplasms before biopsy. Data on 2627 histologically confirmed primary malignant tumors of bone, as collected by the SEER program during the period 1973-1987, were analyzed by age, sex, race, and anatomical site. Osteosarcoma was the most frequently diagnosed primary sarcoma of bone (35.1%), followed by chondrosarcoma (25.8%), Ewing's sarcoma (16.0%), chordoma (8.4%), and malignant fibrous histiocytoma, including fibrosarcoma (5.6%). The most frequently diagnosed sarcoma of bone in persons younger than age 20 was osteosarcoma, followed by Ewing's sarcoma. Chondrosarcoma was the most frequently diagnosed bone tumor in the population older than age 50. The overall 5-year relative survival rates were as follows: osteosarcoma, 41.0%; chondrosarcoma, 72.7%; Ewing's sarcoma, 41.2%; chordoma, 63.8%; and malignant fibrous histiocytoma, 42.9%. There was an improvement in the survival rates during the period 1973-1987 for patients with chordoma and for white female patients with Ewing's sarcoma. Ewing's sarcoma and chordoma occurred almost exclusively in the white population. SEER data provide a unique opportunity to evaluate the incidence and survival rates of bone sarcomas, which are uncommon but highly lethal tumors. The findings from this analysis provide information useful in the diagnosis of these cancers.
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            Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors.

            Katherine M Enzinger, J Langloss, Thomas S. Weiss (1983)
            Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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              A textbook of oral pathology

              WG Shafer, MK Hine, BM Levy … (1983)
              Article 0 comments Cited 31 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Yonsei Med J
                Journal ID (publisher-id): YMJ
                Title: Yonsei Medical Journal
                Publisher: Yonsei University College of Medicine
                ISSN (Print): 0513-5796
                ISSN (Electronic): 1976-2437
                Publication date (Print): 30 April 2006
                Publication date (Electronic): 30 April 2006
                Volume: 47
                Issue: 2
                Pages: 264-270
                Affiliations
                [1 ]Department of Oral and Maxillofacial Surgery, College of Dentistry, Yonsei University, Seoul, Korea.
                [2 ]Department of Oral Pathology, College of Dentistry, Yonsei University, Seoul, Korea.
                [3 ]Oral Science Research Center, College of Dentistry, Yonsei University, Seoul, Korea.
                [4 ]Oral Cancer Research Institute, College of Dentistry, Yonsei University, Seoul, Korea.
                [5 ]Medical Research Center, College of Dentistry, Yonsei University, Seoul, Korea.
                Author notes
                Reprint address: requests to Dr. Sang-Hwy Lee, Department of Oral and Maxillofacial Surgery, College of Dentistry, Yonsei University, Seoul, Korea. Tel: 82-2-2228-8750, Fax: 82-2-364-0992, sanghwy@ 123456yumc.yonsei.ac.kr
                Article
                DOI: 10.3349/ymj.2006.47.2.264
                PMC ID: 2687639
                PubMed ID: 16642559
                SO-VID: dad44176-4944-4dee-9ecf-71d8933562e2
                Copyright © Copyright © 2006 The Yonsei University College of Medicine
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 25 May 2004
                Date accepted : 01 June 2005
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Medicine
                Keywords: nerve sheath tumor,malignant peripheral nerve sheath tumor (mpnst),jaw,neurofibromatosis,neurofibroma
                Data availability:
                ScienceOpen disciplines: Medicine
                Keywords: nerve sheath tumor, malignant peripheral nerve sheath tumor (mpnst), jaw, neurofibromatosis, neurofibroma

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