Chordoma: study of five cases

Abstract

Background: Chordoma is a rare slow growing but malignant midline bone tumor arising from the notochordal remnant and recapitulates the notochord at various stages of differentiation. They involve the axial skeleton. The aim of this study was to find the frequency of this tumor and to analyze the clinical, radiological and pathological findings.Materials and Methods: Cases of chordoma diagnosed at B & B Hospital from January 1999 to November 2011 were studied for clinicopathologic features and immunohistochemical profile.Results: There were 5 cases of chordoma during the study period. Females were affected more with male to female ratio of 1:1.5. Sixty percent of the tumors involved the sacrum, while 40% were intracranial and located at the base of the skull. Median age of diagnosis was 44.8 years. Four cases (80%) were conventional chordoma and 1 case (20%) was a chondroid variant. Two cases evaluated were immunoreactive for S-100 protein, pan-cytokeratin and EMA.Conclusion: Though a low grade tumor with prolonged clinical course, chordomas can cause extensive local destruction with significant morbidity and mortality. Hence a prompt diagnosis with adequate excision is important to save the patient’s life.Journal of Pathology of Nepal (2012) Vol. 2, 260-264DOI: http://dx.doi.org/10.3126/jpn.v2i4.6874

Sacrococcygeal Chordoma Diagnosed by Fnac: A Rare Case Report

“…About one half of patients with chordomas have a palpable sacral mass. 7 On gross, 4 it is gelatinous and soft with areas of hemorrhage. On histopathology, 4 It resembles the normal notochordal tissue in different stages of development.…”

“…3 This is confirmed by the recent evidence that brachyury, a transcription factor involved in notochordal development, is detectable in the disease. 7 Chordoma is a slow-growing neoplasm with little metastatic, but considerable local destructive potential, typically occurring in the axial skeleton at either ends of the spinal column.…”