Sacral Chordoma: Report of a Rare Malignant Neoplasm

• Autor(a) principal: Zimmer , Rafael Gonçalves
• Data de Publicação: 2023
• Outros Autores: Stábille, Isadora Lyrio, Potros, Francine Ribeiro, Martins, Adriana Batista Alves
• Tipo de documento: Artigo
• Idioma: por; eng; spa
• Título da fonte: Revista Brasileira de Cancerologia (Online)
• Texto Completo: https://rbc.inca.gov.br/index.php/revista/article/view/3519
• topic: neoplasias ósseas; cordoma; região sacrococcígea; relatos de casos; bone neoplasms; chordoma; sacrococcygeal region; case reports; neoplasias óseas; cordoma; región sacrococcígea; informes de casos

Introduction: Chordoma is a type of sarcoma, a primary bone malignancy that originates from the notochord and is located on the spinal axis between the clivus and the sacrum. The first description of this pathology occurred in 1857. Patients between 40 and 60 years old are the most affected according to the disease’s epidemiology, the main site involved is the sacral/coccygeal region. The clinical condition is variable depending on the site affected, with generally nonspecific symptoms, delaying the diagnosis made by biopsy. Among the treatment options, surgical resection with margins is currently the main method, and may be associated with radiotherapy or radiosurgery when necessary; the most common metastatic sites are lungs, bones, liver and local lymph nodes. Case report: A 62-year-old female patient had pain in the coccygeal region, worsening while sitting and the appearance of a nodular lesion with progressive growth, diagnosed as a chordoma three years later, after biopsy of the lesion. Due to the extensive lesion, initially she was submitted to chemotherapy and radiotherapy for cytoreduction, but because of the poor response, she was successfully submitted to sacralectomy, however, dehiscence of the surgical wound was detected, and the patient underwent a new approach; since then, no recurrence in the clinical follow-up. Conclusion: Apparently, it is clear the necessity for further investigations on chordoma, a rare tumor with poor response to non-surgical treatments, in order to improve the chemotherapy for this potentially deforming neoplasm.