A thirty years old male patient presented with low back ache for one year and gradually progressive weakness of both lower limbs for last two months. He underwent Lumbar spine Plain film radiography, CT, MRI and CT guided FNAB. He was diagnosed as a case of lumbar spine chordoma.
A 30 years old male patient presented with low back ache for one year, partially responsive to NSAIDS. He also had gradually progressive weakness of both lower limbs for last two months, more marked on right side. There was no history of any trauma, fever or bladder/ bowel involvement. On examination power was 3/5 in right limb and 4/5 in left limb, with no associated sensory impairment. Routine haematological and biochemical profiles were within normal parameters. CNS, CVS, GIT and respiratory system examinations were within normal limits. Plain X-ray revealed osteosclerosis of L4 vertebra (ivory vertebra) with preserved bony contour and intervertebral disc spaces(Fig 1,2). CT scan lumber spine revealed osteosclerotic involvement of body of L4 vertebra with a large soft tissue mass centered posterolaterally, more on right side, with areas of low attenuation and extension into the spinal canal. Small Specks of amorphous calcification was also seen within the soft tissue mass(Fig 3). On MRI imaging the involved vertebra was markedly hypointense on T1 weighted imaging, with heterogenous, predominantly hypointense signal on T2W images.Intervertebral disc spaces were preserved .The Lobulated hyperintense soft tissue mass had craniocaudal extension of one vertebral length(Fig 4,5). The mass was seen displacing the paraspinal and psoas muscles and extending into the spinal canal with prominent septations posterolaterally(Fig 6). A differential diagnosis of Chondrosarcoma, chordoma and lymphoma was made.CT guided FNAB was done and the final diagnosis of chordoma was made. The patient underwent near total removal of the mass with postoperative radiotherapy.
Chordoma is the second most common primary malignant tumor of the spine in adults after lymphoproliferative neoplasms [1]. It has been observed in an autopsy series that in upto 2% of cadavers, non neoplastic notochordal vestiges are seen, most frequently in the spheno-occipital syndrosis and the sacrococcygeal region [1]. The location of chordomas parallel this distribution and this notochordal origin explains the fact that the majority of cases present with central vertebral body destruction with a large soft tissue mass usually placed anteriorly or laterally [2]. Pathologically, the soft tissue component of the chordoma is commonly contained within a fibrous pseudocapsule. Cytological findings are characterized by abundant myxoid ground substance and large physaliphorous cells with abundant pale, vacuolated, bubbly cytoplasm and well defined cell borders [3,4]. There is abundant mucin, both intracellularly and extracellularly and this accounts for the zones of low attenuation within the soft tissues mass on CT scans [1,2]. Chordomas are slow growing, locally invasive malignant tumours. The most striking radiographic feature of chordoma is central vertebral body destruction associated with a large soft tissue mass. There may be osseous expansion and osteosclerosis, especially of the vertebral end plates. In some cases osteosclerosis may predominate, resulting in an “Ivory vertebra” appearance [1, 5]. The incidence of osteosclerosis has been reported to be found in 64% of the cases [6]. The differential diagnosis of ivory vertebra include lymphoma, metastasis especially from prostate and breast, Pagets disease, tuberculous spondylitis, plasmacytoma and POEMS syndrome. Intervertebral disc involvement has been described in 11-14% of chordomas leading to adjacent vertebral involvement [1, 5]. On CT scan there may be solitary or multiple areas of low attenuation within the soft tissue mass, which usually has sharply defined margins. Amorphous calcifications especially at the periphery of the mass is another important supportive finding found in approximately 30% of spinal chordomas above the level of sacrum [1]. MRI has a special role to play in the imaging of chordoma due to its multiplanar imaging characterstics and excellent contrast resolution. On T1 weighted images chordomas have low to intermediate signal intensity and very high signal intensity on T2 weighted imaging [1,7]. On both CT scan and MRI, there is contrast enhancement. Chordoma may also metastasize systemically to liver, lungs, regional lymph nodes, peritoneum, skin etc. and has been reported in 10-43% of patients [1,2,6]. The prognosis of chordoma depends on its resectability, which to a large extent depends on its location and its size at presentation. The death is usually due to local recurrence and invasion rather than metastasis. CT and MR imaging helps in delineating the exact extent of the tumour, local invasion and any systemic metastases and thus helps in planning the surgery as well as defining the exact port for the adjuvant radiotherapy. In conclusion we would like to reemphasize the fact that even though chordoma of lumbar spine is a rare entity, it should be included in the differential diagnosis of solitary ‘ivory vertebra’.
LUMBAR SPINE (L 4) CHORDOMA
This 30-year-old male patient has had persistent lower back pain for one year and has experienced progressively worsening bilateral lower limb weakness over the past two months. After undergoing X-ray, CT, and MRI examinations, the main imaging findings can be summarized as follows:
Based on the imaging findings and the patient’s clinical presentation, the following should be considered as potential diagnoses or differentials:
Taking into account the patient’s history (chronic low back pain and progressively worsening neurological symptoms), imaging features (lumbar “ivory vertebra,” central destruction with soft tissue mass formation, high T2 signal on MRI), and CT-guided fine needle aspiration biopsy (FNAB) pathology (showing typical chordoma cytological features), the most likely diagnosis in this case is:
Chordoma of the Lumbar Spine.
Based on the patient’s age, tumor location, local invasiveness, and neurological deficits, the main therapeutic strategies include:
Following spinal tumor surgery and radiotherapy, particular attention should be paid to bone fragility and the stability of any surgical implants. Rehabilitation must be conducted under guidance from qualified therapists or physicians to avoid excessive flexion, twisting, or heavy loads that could lead to further spinal injury.
This analysis report is provided solely as a reference based on current imaging and medical history, and it does not replace an in-person consultation or professional medical advice. If you have any questions or changes in symptoms, please seek help from a specialized medical institution or a professional doctor.
LUMBAR SPINE (L 4) CHORDOMA
