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      Primary Chordoma of the Nasopharynx: A Rare Case Report and Review of the Literatures


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          Primary chordoma of the nasopharynx is an extremely rare malignant tumor of notochordal origin in the extra-osseous axial skeleton. It presents as a soft tissue mass without involvement of the skull base bone (clivus) and may mimic other lesions of the nasopharynx. A 26-year-old male patient is presented with nasal obstruction and congestion for the last 3 years. Physical and radiological examination revealed a mass in the naso-oropharyngeal region. It was suspected to be a cystic mass or abscess on radiological imaging. However, histopathological examination revealed a chordoma. We review all 20 cases of primary nasopharyngeal chordoma reported previously in the literature. Nasopharyngeal chordoma should be considered in the differential diagnosis of nasopharyngeal mass due to its unspecific appearance on clinical and radiology examination.

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          Most cited references25

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          Chordoma: the nonsarcoma primary bone tumor.

          Chordomas are rare, slowly growing, locally aggressive neoplasms of bone that arise from embryonic remnants of the notochord. These tumors typically occur in the axial skeleton and have a proclivity for the spheno-occipital region of the skull base and sacral regions. In adults, 50% of chordomas involve the sacrococcygeal region, 35% occur at the base of the skull near the spheno-occipital area, and 15% are found in the vertebral column. Craniocervical chordomas most often involve the dorsum sella, clivus, and nasopharynx. Chordomas are divided into conventional, chondroid, and dedifferentiated types. Conventional chordomas are the most common. They are characterized by the absence of cartilaginous or additional mesenchymal components. Chondroid chordomas contain both chordomatous and chondromatous features, and have a predilection for the spheno-occipital region of the skull base. This variant accounts for 5%-15% of all chordomas and up to 33% of cranial chordomas. Dedifferentiation or sarcomatous transformation occurs in 2%-8% of chordomas. This can develop at the onset of the disease or later. Aggressive initial therapy improves overall outcome. Patients who relapse locally have a poor prognosis but both radiation and surgery can be used as salvage therapy. Subtotal resection can result in a stable or improved status in as many as 50% of patients who relapse after primary therapy. Radiation therapy may also salvage some patients with local recurrence. One series reported a 2-year actuarial local control rate of 33% for patients treated with proton beam irradiation.
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            Update on nasopharyngeal carcinoma.

            R Thompson (2007)
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              Brachyury: A Diagnostic Marker for the Differential Diagnosis of Chordoma and Hemangioblastoma versus Neoplastic Histological Mimickers

              Brachyury is a transcription factor which is required for posterior mesoderm formation and differentiation as well as for notochord development during embryogenesis. Due to its expression in the neoplastic cells of chordoma, a malignant tumour deriving from notochordal remnants, but not in tumors showing a similar histology, brachyury has been proposed as a diagnostic marker of this neoplasia. Though commonly considered a hallmark of chordoma, the expression of brachyury has been also documented in the stromal cells of hemangioblastoma (HBL), a slow growing tumor which may involve the central nervous system (CNS) and, rarely, the kidney. Herein we review the role of brachyury immunohistochemical detection in the identification and differential diagnosis of chordoma and HBL towards histological mimickers and suggest that brachyury is added to the panel of immunohistochemical markers for the recognition of HBL in routinary practice, principally in unusual sites.

                Author and article information

                Case Rep Pathol
                Case Rep Pathol
                Case Reports in Pathology
                23 September 2019
                : 2019
                : 3826521
                1Department of Anatomical Pathology, Faculty of Medicine Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia
                2Department of Anatomical Pathology, Faculty of Medicine, Universitas Sebelas Maret, Solo, Indonesia
                3Department of Pathology and Laboratory Medicine, Istishari Hospital, Amman, Jordan
                Author notes

                Academic Editor: Piero Tosi

                Author information
                Copyright © 2019 Ery Kus Dwianingsih et al.

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                : 22 April 2019
                : 6 August 2019
                Case Report


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