A case report on a rare primary bone tumour: sacral chordoma presenting as sacral swelling with sphincter dysfunction
DOI:
https://doi.org/10.18203/2349-2902.isj20243994Keywords:
Sacral chordoma, Nerve root preservation, Surgical management, Functional outcomesAbstract
Chordoma is a rare, locally aggressive malignant tumour arising from remnants of the notochord, primarily affecting the axial skeleton, seen twice often in males above 40 years. This case report presents a 50-year-old male with primary sacral chordoma, emphasizing the need for early evaluation, treatment modalities and focusing on the importance of sacral nerve preservation during surgery for better functional outcome. Purpose of the study was to demonstrate the significance of early evaluation and diagnosis, emphasizing the importance of at least unilateral sacral nerve root preservation for optimizing postoperative bowel and bladder function. A 50-year-old male presented with sacral swelling, saddle anaesthesia, lower backache, urinary incontinence, and constipation. Examination revealed a firm, immobile sacral mass with reduced anal sphincter tone. Magnetic resonance imaging (MRI) suggested the presence of a sacral chordoma, leading to the planning of surgical resection. Intraoperatively, frozen section analysis confirmed the diagnosis of chordoma. Intraoperatively, the lesion was found to extend predominantly toward the left side, necessitating careful dissection and preservation of the sacral nerve roots on the right side. Post-operatively, the patient experienced improved bowel and bladder function, along with significant pain relief. The patient showed substantial postoperative improvement, with resolution of urinary and bowel dysfunction. Follow-up imaging revealed no tumour recurrence. Preservation of sacral nerve roots was crucial to the patient’s recovery, improving overall quality of life. This case highlights the need to consider chordoma in the differential diagnosis of sacral masses and emphasizes the importance of sacral nerve root preservation during resection to optimize functional outcomes in patients with this rare tumour.
Metrics
References
Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000;88(9):2122-34. DOI: https://doi.org/10.1002/(SICI)1097-0142(20000501)88:9<2122::AID-CNCR19>3.0.CO;2-1
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):1-11. DOI: https://doi.org/10.1023/A:1008947301735
Fletcher CDM, Unni KK, Mertens F. Pathology and genetics of tumours of soft tissue and bone. Lyon: IARC Press. 2002.
Chambers PW, Schwinn CP. Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol. 1979;72(5):765-76. DOI: https://doi.org/10.1093/ajcp/72.5.765
Di Maio S, Temel Y. The management of sacral chordomas. Neurologist. 2013;19(1):51-4.
Zileli M, Borkar SA, Sani S. Surgical management of chordomas of the skull base and vertebrae. World Neurosurg. 2020;139:285-306.
Stacchiotti S, Sommer J. Chordoma: a review of clinical management. J Surg Oncol. 2015;115(6):687-96.
Fisher CG, Mankin HJ. Chordoma of the spine: A review. Spine (Phila Pa 1976). 2004;29(5):579-83.
York JE, Kaczaraj A, Abi-Said D, Fuller GN, Skibber JM, Janjan NA, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 1999;44(1):74-9. DOI: https://doi.org/10.1097/00006123-199901000-00041
Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012;13(2).
George B, Bresson D, Herman P, Froelich S. Chordomas: A review. Neurosurg Clin N Am. 2006;17(1):31-44.
Mankin HJ, Hornicek FJ. Diagnosis, classification, and management of chordoma. J Surg Oncol. 2006;94(8):978-83.
Furlong MA, Tuvia J, Fanburg-Smith JC. Chordoma: Clinicopathologic features, prognostic factors, and long-term outcomes in a series of 29 cases. Arch Pathol Lab Med. 2004;128(2):166-70.
Boriani S, Bandiera S, Biagini R, Bacchini P, Boriani L, Cappuccio M, et al. Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976). 2006;31(4):493-503. DOI: https://doi.org/10.1097/01.brs.0000200038.30869.27
Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012;13(2):69-76. DOI: https://doi.org/10.1016/S1470-2045(11)70337-0