A clinical case of sacrum and coccyx chordoma having a massive intrapelvic component (surgical treatment with a brief review of the literature)

Abstract

BACKGROUND: Chordoma is a rare malignant tumor that develops from the remnants of the notochord and is located in the axial skeleton in the absolute majority of cases. It is most often localized in the sacrum, coccyx and pelvis, and is characterized by an initially asymptomatic long-time course, making early diagnosis difficult. Radical surgical treatment is the leading factor allowing to prolong the recurrence-free and overall survival of patients with chordoma, but it is often difficult due to complex anatomical localization of the tumor, as well as delayed medical treatment, frequently accompanied with the subsequent development of neurological complications, while in elderly patients with high comorbidity it is not always feasible. CLINICAL CASE DESCRIPTION: We present a clinical case of radical surgical treatment of a patient with a S4-5 vertebral and coccyx chordoma showing a massive intrapelvic component. Clinical manifestations of the disease in the form of pain syndrome and pelvic organ dysfunction developed only when the tumor reached a larger size, forming a massive intrapelvic component up to 20 cm in size. The examination, including computer and magnetic resonance tomography, trepan biopsy with pathomorphologic examination, allowed to establish the diagnosis. Taking into account the size and localization of the tumor, the multidisciplinary team performed radical surgical intervention including sacral resection at the S3 level, coccygectomy with tumor removal. Morphological study of the removed tumor confirmed the diagnosis. In the early postoperative period, the wound healed by primary tension, the development of pelvic organs dysfunction was noted, which partially regressed by discharge. The article presents a brief review of the current problems of diagnosis and treatment of patients with chordoma. CONCLUSION: Diagnosis and treatment of sacral chordoma is one of the most difficult problems of orthopedic oncology. A full preoperative examination and a multidisciplinary approach in this case made it possible to perform radical surgical intervention, reduce the risks of tumor progression, intra- and postoperative complications, and preserve the patient’s quality of life as much as possible.