ABSTRACT
Chordoma is a rare tumor. It has unique clinical, pathological and immunohistochemical characteristics. Accurate diagnosis is essential as the tumor shows an aggressive clinical course and requires a multimodal therapeutic approach. A case with wide spread distant metastatic disease that was initially thought to represent metastatic thyroid carcinoma is presented. Appropriate clincopathologic correlation and the histologic findings raised the possibility of poorly differentiated chordoma. The diagnosis was confirmed by immunohistochemistry for INI-1 and Brachyury. The approach to the diagnosis emphasizing the clinical and pathologic findings of this case is discussed and reviewed in the context of the published literature.
Subject(s)
Humans , Male , Adult , Chordoma/diagnosis , Chordoma/pathology , Upper Extremity , SMARCB1 Protein/therapeutic use , Neoplasm Metastasis , Notochord/injuriesABSTRACT
El cordoma es un tumor osteocartilaginoso raro, de lento crecimiento, con una tasa de incidencia global de 8,4 casos por cada 10 millones de habitantes. Comúnmente aparece en la quinta y sexta década de la vida, predomina en el sexo masculino. Se presenta un paciente masculino de 47 años de edad, con cervicobraquialgia, cuadriparesia y masa tumoral palpable en región anterolateral del cuello. El estudio de resonancia magnética demostró la presencia de una lesión retrofraríngea con destrucción vertebral y compresión extradural. Se decidió resección quirúrgica de la lesión. El diagnóstico histopatológico por inmunohistoquímica arrojó como resultado, un cordoma(AU)
Chordoma is a rare, slow-growing osteocartilaginous tumor with an overall incidence rate of 8.4 cases per 10 million inhabitants. Commonly appears in the fifth and sixth decade of life, predominates in the male sex. We present a 47-year-old male patient with cervicobrachialgia, quadriparesis and palpable tumor mass in the anterolateral region of the neck. The magnetic resonance study showed the presence of a retropharyngeal lesion with vertebral destruction and extradural compression. Surgical resection of the lesion was decided. The histopathological diagnosis by immunohistochemistry resulted in a chordoma(AU)