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Chondroid chordoma

Case report

W. Craig Clark, Jon H. Robertson, and Rafael Lara

C hordoma is a rare tumor appearing in close relation to the axial skeleton and believed to arise from notochordal remnants. Although they are histologically benign and slow-growing, chordomas may be considered as possessing malignant potential due to their occurrence in critical locations, their locally aggressive behavior, and their high recurrence rates. 14 The histological resemblance of chondroma and chondroid variants of other neoplasms has been appreciated since Virchow's study 15 and has contributed to the difficulty in differentiating the two

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Narayan Sundaresan, Joseph H. Galicich, Florence C. H. Chu, and Andrew G. Huvos

notochord. Although the centrum of the nucleus pulposus is believed to be the only derivative of this structure, remnants of chordal tissue can persist in the adult from the clivus to the coccyx. The supposition that chordomas arise from notochordal rests is based primarily on the fact that these neoplasms occur almost exclusively in the axial skeleton and histologically resemble embryonic notochord by both light and electron microscopy. 31 Furthermore, Ribbert 29 and subsequently, Congdon 10 demonstrated experimentally in rabbits that proliferating tissue following

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Laurence E. Becker, Allan J. Yates, Harold J. Hoffman, and Margaret G. Norman

C lival chordomas are uncommon at any age. By 1967 less than a dozen cases had been reported in children who had not yet reached puberty. 8 We believe the case presented here is in the youngest patient reported to have this problem. Case Report A previously healthy, 10-month-old boy developed a right sixth nerve paresis. By 16 months he also had developed left sixth and third nerve pareses, and at 21 months he stopped walking and became unduly irritable. We first saw him when he was 23 months old. Examination The patient had an enlarged head, with

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Francis H. Tomlinson, Bernd W. Scheithauer, Gary M. Miller, and Burton M. Onofrio

C hordomas , tumors presumably derived from notochordal rests, are rare. Most are located in the sacrococcygeal and clival regions. Only 15% of all chordomas arise within true vertebrae, more than one-half of these being encountered in the lumbar region. 18 Extraosseous processes that histologically resemble a chordoma include ecchordosis physaliphora 14, 17, 22, 25 and intradural chordoma. 1, 4, 8, 13, 16, 23 The latter tumors are rare and are confined to the cranial region. The term “ecchordosis physaliphora” describes midline intracranial lesions of

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Intradural chordoma without bone involvement 

Case report and review of the literature

Joyce Steenberghs, Carlotte Kiekens, Johan Menten, and Jan Monstrey

nature and is generally associated with extradural extension and bone destruction. If left to grow for long periods, however, it may invade the dura mater, extending intra- and extradurally. 13 The incidence of metastasis, which has been reported to be as high as 25%, is greater than previously believed and may be related to its long clinical history. In order of most to least frequent, the common sites of distant metastases are the lungs, liver, and bone. 2 We describe the rare case of a patient with a primary intradural chordoma, located in the dorsolumbal and

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Kenan I. Arnautović and Ossama Al-Mefty

C hordomas are rare, aggressive, slow-growing, invasive, and locally destructive tumors. Their propensity for local recurrence, direct extension from the primary site, and systemic and cerebrospinal fluid metastasis are well known. Distal metastasis most often occurs in young patients, those with sacrococcygeal or vertebral tumors, and those with atypical chordoma histological features. Predictably, a poor prognosis is demonstrated in patients with metastasis. 1–12, 15–20, 22–50 Radical resection followed by high-dose radiotherapy “sterilizes” the field and

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Rui M. Vaz, Josué C. Pereira, Umbelina Ramos, and Celso R. Cruz

C hordomas are rare tumors of the central nervous system that are found predominantly in the sacrococcygeal area (50%) and in the basisphenoidal region (30% to 40%). Most of the remainder are related to vertebral bodies. 5, 7, 17, 18 Chordomas involving the vertebral column are more frequently located in the lumbar region and typically cause destruction of vertebral bodies and secondary invasion of the pedicle, lamina, and spinal process. 5, 17 This localization of the axial skeleton is related to the presumed embryonic origin of chordomas, that is, the

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M. Akif Bayar, Yavuz Erdem, Omur Tanyel, Koray Ozturk, and Zeki Buharali

C hordomas are slow-growing, locally aggressive neoplasms of the axial skeleton and account for 1 to 4% of all malignant bone tumors. 11 Approximately 50% of chordomas arise in the sacrococcygeal region, 35% at the clivus, and 15% in the true vertebral column. 11, 12 To date, unusual extraosseous locations such as tentorium cerebelli, primary intradural pontocerebellar, intradural foramen magnum, intradural cervical, intradural thoracic, and epidural lumbar regions have been reported in association with chordomas. 4, 5, 8, 9, 14, 15, 17, 18, 21 To our

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Ronald E. Warnick, Jack Raisanen, Theodore Kaczmar Jr., Richard L. Davis, and Michael D. Prados

autopsy cases. 10 These “ecchordosis physaliphora” are located ventral to the brain stem and are usually contiguous with the clivus through a small opening in the dura. This notochordal vestige is thought to represent a benign developmental lesion. 11 There have been seven reported cases of completely intradural chordoma. 11 All previous cases had a ventral location similar to the distribution of ecchordosis physaliphora. Despite this anatomical correlation, there is considerable disagreement regarding the origin of intradural chordomas from these intradural

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Ronald Sahyouni, Khodayar Goshtasbi, Amin Mahmoodi, and Jefferson W. Chen

C hordoma is a rare tumor that occurs in bones of the skull (mainly of clival origin), the centrum of the mobile spine, and the bones in the sacrococcygeal region. Chordoma has an age-adjusted incidence of 8 cases per million persons per year, as calculated by McMaster et al. using the Surveillance, Epidemiology, and End Results (SEER) program of the National Cancer Institute for the period from 1973 to 1995. 37 Furthermore, it accounts for approximately 1%–4% of all bone malignancies. 17 , 53 These highly recurrent low-grade neoplasms can affect patients of