Ann Thorac Surg 2010;89:302-303. doi:10.1016/j.athoracsur.2009.05.043
© 2010 The Society of Thoracic Surgeons
Case Reports
Primary Chordoma of the Lung
Salvatore Strano, MD, PhDa,
Lamia Ouafi, MDb,
Mariette Baud, MDc,
Marco Alifano, MD, PhDa,*
a Department of Thoracic Surgery, Hotel-Dieu University Hospital, Paris, France
b Department of Pathology, Hotel-Dieu University Hospital, Paris, France
c Department of Pneumology, Saint-Antoine University Hospital, Paris, France
Accepted for publication May 13, 2009.
* Address correspondence to Dr Alifano, Unité de Chirurgie Thoracique, Hotel-Dieu, 1, Pace du Parvis Notre-Dame, Paris, 75004, France (Email: marcoalifano{at}yahoo.com).
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Abstract
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We report the case of a 79-year-old woman referred to our institution for persistent cough and right-sided chest pain. A computed tomographic scan revealed a 2-cm round nodule in the right lower lobe. A wedge resection of the lesion was achieved by video-assisted thoracic surgery. Pathologic examination was consistent with the diagnosis of chordoma. Magnetic resonance imaging of the whole spine and skull basis was normal. Therefore, a diagnosis of primary lung chordoma, an exceptional condition, could be established.
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Introduction
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Chordoma is a rare neoplasm that arises from nothocordal remnants [1]. In the United States, approximately 25 new cases are diagnosed each year [1], and these cases account for approximately 1% to 4% of all primary malignant bone tumors [2].
Chordoma is more common in men and is extremely rare among blacks and patients aged less than 40 years. Almost all the chordomas arise at the skull basis or sacrococcygeal level, with approximately 5% of cases originating at the midline thoracolumbar level [3].
We report herein a case of primary chordoma of the lung in a woman, which is a very exceptional condition.
A 79-year-old woman, who is a non-smoker, was referred to our hospital for persistent cough and minimal right-sided chest pain. Her past clinical history was unremarkable. A clinical examination of the patient showed no abnormalities. A chest roentgenogram found a round opacity in the right lung. A computed tomographic chest scan (Fig 1) confirmed a 2-cm round nodule in the right lower lobe. Peripheral calcifications were observed within the nodule. There was not any nodal, hepatic, adrenal, or cerebral abnormalities. Her fiberoptic bronchoscopy results were normal. A computed tomographic-coupled positron emission tomographic scan showed a minimal uptake of the radiotracer (standard uptake value of 1, 2). The spirometry and arterial blood gas analyses were normal.
At video-assisted thoracic surgery, a wedge resection including the nodule with wide margins was performed. No other relevant abnormalities were observed. Postoperative course was uneventful.
A pathologic examination showed tumoral tissue surrounded by a fibrous capsule. Tumor cells had a trabecular organization and a vacuolated cytoplasm (so-called physaliphorous cells). The nuclei were spheric, hyperchromatic, and atypia was rare. Immunohistochemistry showed positivity for protein S100, epithelial membrane antigen, vimentin, and pancytokeratin (Fig 2). These characteristics were consistent with the diagnosis of chordoma. On the basis of these results, a magnetic resonance image of the whole spine and base of the skull was performed and no abnormalities were discovered. The patient is well with no evidence of disease recurrence at 24-months follow-up.
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Fig 2. (A) Histopathologic examination of the resected specimen (Hematoxylin and eosin stain, original magnification x100). (B) Immunohistochemistry for epithelial membrane antigen counterstained with hematoxylin, original magnification x100. (C) Immunohistochemistry for vimentin counterstained with hematoxylin, original magnification x100. (D) Immunohistochemistry for protein S100 counterstained with hematoxylin, original magnification x40.
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Comment
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Chordomas originate from embryonic remnants of notochord [1]. In the embryo of 11 mm, notochord attains complete maturation before involution and fragmentation. Fragments are displaced cranially and caudally from the original central position. The hypothesis that notochord is at the origin of mature nucleus pulposus of intervertebral disks is controversial, but recent histologic studies are against this earlier theory. Considering the aforementioned embryologic findings, the more frequent occurrence of chordoma at skull basis (25% to 35% of cases) and sacrum (50% to 60% of cases) is therefore explained. Less then 10% of chordomas occur in cervical vertebrae and less than 5% in the thoracolumbar spine [4].
At the thoracic level, few cases of posterior mediastinal chordomas (independent from spine) have been described [5]. At the extrathoracic level, extraosseous chordomas have been reported in the sella turcica, paranasal sinuses, larynx, maxilla and nasopharynx.
We believe that a single case of primary lung chordoma has been reported so far [6]. No definitive explanations for this condition can be provided. The tumor may originate from either multipotent cells in the lung parenchyma, or from notochordal cells whose migration from the midline would have been completely aberrant.
Chordomas are often relatively slow growing, low-grade malignancies, but their potentially aggressive local behavior is well known. The exact rate of distant metastasis is unknown, as figures ranging from 3% to 60% have been reported. The most common sites of distant spreading are lungs, bones, soft tissues, lymph nodes, liver, and skin. In any case, it should be kept in mind that this information is derived from midline chordomas [3, 4].
Chemotherapy is considered to be ineffective. In spite of relative radio resistance, radiotherapy is often administered after radical resection or debulking of midline lesions. It is believed to possibly increase the disease-free survival or even the time to progression in tumors whose margins are usually close to resection limits [3, 4].
Neither chemotherapy nor radiotherapy was prescribed to our patient because of the size of the lesion, the existence of unviolated capsula, and the wide surgical margins free of disease. Therefore, a decision of close follow-up was adopted.
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Acknowledgments
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The authors are grateful to Dr Stephan Moffett for help in the editing the manuscript.
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References
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- Heaton JM, Turner DR. Reflection on notochordal differentiation arising from a study of chordomas Histopathology 1985;9:543-550.[Medline]
- Jemal A, Siegel R, Ward E, Murray T, Xu J, Thun MJ. Cancer statistics, 2007 CA Cancer J Clin 2007;57:43-66.[Medline]
- McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence, and survival patterns in the United States, 1973–1995 Cancer Causes Control 2001;12:1-11.[Medline]
- Mirra J, Nelson S, Della Rocca C. ChordomaIn: Fletcher CD, Unni K, Mertens F, editors. Pathology and genetics of tumors of soft tissue and bone. Lyon, France: IARC Press; 2002. pp. 316-317.
- Huang SM, Chen CC, Chiu PC, Lai PH, Ho JT, Tseng HH. Unusual presentation of posterior mediastinal chordoma in a 2-year-old boy J Pediatr Hematol Oncol 2003;25:743-746.[Medline]
- Park SY, Kim SR, Choe YH, et al. Extra-axial chordoma presenting as a lung mass Respiration 2009;77:219-223.[Medline]
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Abstract
Introduction
