Lumbosacral Chordomas: The Enigmatic and Dreaded Entity

Author(s):

Mir Younis Ali, Mohsin Fayaz* and Abdul Rashid Bhat

Introduction: Primary tumours of the spine are rare, accounting for less than 5% of all osseous neoplasms and less than 0.2% of all cancers. Among all primary tumours of the spine, the incidence of primary malignant tumours varies between 32% and 71%. The most common primary malignant tumours of the spine are chordoma and sacral sarcomas like chondrosarcoma, osteosarcoma and Ewing sarcoma. Chordoma is a slow-growing, locally aggressive neoplasm of bone which arises from embryonic remnants of the notochord, with an overall incidence of 0.08 per 100,000 individuals accounting for 40% of all primary sacral tumours. The tumour has a delicate pseudo capsule, in which satellite lesions and occult extension are frequent. Hence, local recurrence is expected due to failure to achieve negative surgical margins. Chordomas are insensitive to chemotherapy and radiotherapy. Therefore, complete surgical excision is the mainstay for long-term survival.

Materials and Methods: The present study was undertaken in the Department of Neurosurgery, Sher-i-Kashmir Institute of Medical Sciences. Our study was a prospective and retrospective study which included all patients who underwent surgical procedures for lumbosacral spinal chordomas, retrospectively from January 2008-August 2016 and prospectively from September 2016 to August 2018.

Results: Shows the age distribution of patients in study.Mean age was 53 years. Youngest patient was 18 years and eldest was 72 years. Maximum number of patients (33.33%) belonged to 50-60 age group, followed by 27.77% patients in age group of 60-70. Out of 18 patients 12 (66.6%) were males and 6 (33.3%) were females with a male to female ratio of 2:1. The symptomatology of the patients and the most common symptom was pain in 16 (89%) patients followed by weakness in 11 (61%) patients, followed by swelling in 11 (44%). Normal Power (grade V) power was present in 7 (38.8%) patients whereas powerwas decreased (grade III/IV) in 11 (61.11%) patients. The duration of symptoms is usually long due to vague symptomatology of the spinal chordomas. Patients often are seen in general surgery and urology specialities due to rectal dysfunction or urinary tract symptoms.In our study mean duration of symptoms before the patient was diagnosed was 24.88 months. Location of tumour in our study and tumour was sacral/ sacrococcygealin 13 (72%) and lumbar/lumbosacral in 5 (28%) of patients. Size of tumour ranged from 6 to 13 cm and mean greatest diameter was 9.8 cm. In our study, 55.5% of the patients had tumour size less than 10 cm whereas in 44.5% partients tumour size was greater than 10 cm. Out of 18 patients 16.6% patients had postoperative stay in hospital for 0-5 days, 27.8% patients had hospital stay for 5-10 days, 33.4% patients had stay in hospital for 10-15 days, 11.2% had stay in hospital for 15-20 days, 5.5% had stay in hospital for 25-30 days and 5.5% had hospital stay for 40-45 days and Range was between 5-41 days and mean duration of hospital stay was 13 days. Adjuvant therapy in the form of radiotherapy was given in 6 (33%) out of 18 patients.

Conclusions: Local recurrence of the disease was found in 7 (39%) out of 18 patients after a period of one year after surgery and within 3 years of follow up. Two patients had recurrence in 2^nd year post surgery and five patients had recurrence in 3^rd year after surgery. Metastasis was present in 3 (17%) of patients which included lung and bone metastasis.